460 results on '"angiofibromas"'
Search Results
2. Clinical and histopathological evaluation of cutaneous angiofibromas
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Shayan Dasdar, Maryam Nasimi, Nika Kianfar, Kambiz Kamyab, and Arezou Moradi
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Adult ,Male ,Pathology ,medicine.medical_specialty ,Skin Neoplasms ,Histology ,Adolescent ,Acanthosis ,Dermatology ,Angiofibroma ,Pathology and Forensic Medicine ,Young Adult ,030207 dermatology & venereal diseases ,03 medical and health sciences ,0302 clinical medicine ,Humans ,Medicine ,Child ,Nose ,Histiocyte ,Aged ,Retrospective Studies ,Aged, 80 and over ,business.industry ,Mean age ,Middle Aged ,medicine.disease ,Angiofibromas ,medicine.anatomical_structure ,Child, Preschool ,030220 oncology & carcinogenesis ,Female ,Histopathology ,Fibrous Papule ,business - Abstract
BACKGROUND Angiofibromas are common benign skin lesions that are diagnosed clinically and histopathologically. Fibrous papule (FP) is one of the clinical types of angiofibromas located primarily on the nose. METHODS The cases with the confirmed diagnosis of angiofibroma were enrolled. Demographic features of the patients and clinical characteristics of the lesions were extracted. Moreover, histopathological features of the available slides were microscopically reviewed. RESULTS From 83 cases of cutaneous angiofibromas, 57 (69%) had lesions consistent with FP. The mean age at diagnosis was 37.7 ± 16.3 years and the most common site of involvement was the nose (53% of cases). Regarding the histopathological evaluation of 71 slides, most cases represented vascular changes (97%) and dermal fibroplasia (87%). Melanin incontinence (41%), acanthosis (31%), and lichenoid features (25%) were other common histopathological findings. Moreover, a sparse inflammatory cell infiltrate was present in 68% of cases. The most prevalent inflammatory cells were lymphocytes (61%) and histiocytes (34%). CONCLUSION This study presented the histopathological findings of a series of cutaneous angiofibromas. Dermatopathologists should be aware of these features, especially those that are less common, to better diagnose and differentiate them from malignant neoplasms. more...
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- 2021
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3. Case report on tuberous sclerosis
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Nadezhda V. Krasnova, Geliya G. Gimalieva, and Larisa G. Sinitsyna
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Skin manifestations ,medicine.medical_specialty ,business.industry ,Visual examination ,tuberous sclerosis ,Dermatology ,Disease ,neurocutaneous disease ,medicine.disease ,Angiofibromas ,Shagreen ,030207 dermatology & venereal diseases ,03 medical and health sciences ,Tuberous sclerosis ,0302 clinical medicine ,Infectious Diseases ,RL1-803 ,030220 oncology & carcinogenesis ,Concomitant ,Medicine ,orphan disease ,medicine.symptom ,business ,Hypopigmentation - Abstract
Patient M., 23 years old, consulted a dermatologist with complaints of rashes on the face, which had bothered since childhood. On objective examination, skin lesions were widespread. A visual examination revealed spots of hypopigmentation, angiofibromas of the face, shagreen fate of the skin, periungual fibromas. She was diagnosed with tuberous sclerosis. Further examination revealed a neoplasm in the brain and right kidney, damage to the lungs, tubular bones, lymphadenopathy. The patient continues to be monitored by a neurologist and therapist. Based on the results of CT scan of the chest organs, an oncologist's consultation was scheduled to conduct an oncology search. Thus, with skin manifestations characteristic of this disease, it is necessary to conduct a comprehensive examination to identify concomitant pathology and early diagnosis of complications. more...
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- 2021
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4. A case of a rare pelvic neoplasm - unusually large angiofibromas
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I. D. Pokladov, A. V. Vazhenin, O. N. Chernova, and R. E. Shtentsel
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medicine.medical_specialty ,Average size ,Similarity (network science) ,business.industry ,Medicine ,Radiology ,Angiofibroma ,Differential diagnosis ,business ,medicine.disease ,Angiofibromas - Abstract
Pelvic tumors are quite common. Of these, angiofibromas are very rare. It is causes difficulties in differential diagnosis. But getting the right diagnosis is important, given the great similarity with other, often malignant tumors. According to the literature, the average size of angiofibromas does not exceed 12 cm. We present the case of a patient with an unusually large angiofibroma. more...
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- 2021
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5. Combined Treatment with Electrocauterization, Carbon Dioxide Laser, and Microneedle Fractional Radiofrequency for Facial Angiofibromas in Tuberous Sclerosis Complex: A Case Report and Literature Review
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Jing Mi, Jiying Dong, Huyan Lin, Min Yao, Ying Zeng, Yiqiu Zhang, Min Yan, and Shen Wang
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medicine.medical_specialty ,Facial angiofibromas ,business.industry ,medicine.medical_treatment ,Carbon dioxide laser ,Cheek ,medicine.disease ,Dermatology ,Angiofibromas ,Chin ,Tuberous sclerosis ,medicine.anatomical_structure ,Combined treatment ,medicine ,Electrocauterization ,business - Abstract
Tuberous sclerosis complex is a type of genetic multisystem disease that causes hamartomas in various organs. Facial angiofibromas commonly occur in 80% of patients and are prominently distributed over the cheek, chin, and nasolabial folds with severe disfigurement and emotional distress. Recently, photoelectric devices have been identified for the treatment of angiofibromas with great efficacy and fewer side effects. We report a case of a 42-year-old man with facial angiofibromas, who was treated with a combination of high-frequency electrocauterization, Ultrapulse CO2 laser, and microneedle fractional radiofrequency with 7 sessions and a 6-month follow-up. The patient showed great improvement in relation to the elevated lesions and nodules. A low recurrence rate was observed. This is the first study to investigate the efficacy of high-frequency electrocauterization and microneedle fractional radiofrequency in angiofibromas. It may provide an optimal approach for clinicians wherein a combined treatment of various lasers and electric devices is effective for complicated, protuberant, and firm angiofibromas of specific patients. more...
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- 2021
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6. Endoscopic resection of juvenile nasopharyngeal angiofibromas
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Christopher Pool and Meghan N. Wilson
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medicine.medical_specialty ,business.industry ,medicine.disease ,Tumor control ,Angiofibromas ,humanities ,Benign tumor ,03 medical and health sciences ,0302 clinical medicine ,Otorhinolaryngology ,Blood loss ,030220 oncology & carcinogenesis ,medicine ,Juvenile ,Surgery ,Endoscopic resection ,Radiology ,030223 otorhinolaryngology ,business - Abstract
Juvenile nasopharyngeal angiofibromas (JNAs) are a rare, highly vascular benign tumor affecting adolescent males. Recent advances in endoscopic visualization and technique have allowed for complete extirpation of these tumors endoscopically. Proponents of this approach find benefit in the lack of facial incisions, decreased intraoperative blood loss, and excellent tumor control. This chapter describes the endoscopic approach to JNAs. more...
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- 2021
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7. Open resection of juvenile nasopharyngeal angiofibroma
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Nicole Ruszkay and Meghan N. Wilson
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medicine.medical_specialty ,business.industry ,Juvenile nasopharyngeal angiofibroma ,Head and neck tumors ,Angiofibromas ,Resection ,Vascular Tumors ,Otorhinolaryngology ,Open Resection ,Medicine ,Juvenile ,Surgery ,Radiology ,business ,Young male - Abstract
Juvenile nasopharyngeal angiofibromas are rare, benign vascular tumors of the nasopharynx that account for less than 0.5% of all head and neck tumors. These lesions occur almost exclusively in young males. There are various approaches that allow for successful resection of nasopharyngeal angiofibromas, including open, endoscopic, and combined procedures. With the advancement of endoscopic technologies, the endoscopic approach has become the favored technique for smaller, less extensive lesions. more...
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- 2021
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8. Extranasopharyngeal Angiofibroma of the Natural Ostium of Maxillary Sinus
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Seong Kook Park, Chanhyeon Park, Eung Rok Yeon, and Soo Jin Jeong
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Adult ,Male ,medicine.medical_specialty ,Maxillary sinus ,Nose Neoplasms ,Nasal congestion ,Angiofibroma ,otorhinolaryngologic diseases ,medicine ,Nasal septum ,Humans ,Nasal Septum ,business.industry ,General Medicine ,Maxillary Sinus ,medicine.disease ,Nasal meatus ,Angiofibromas ,Ostium ,medicine.anatomical_structure ,Otorhinolaryngology ,Surgery ,Radiology ,Nasal Cavity ,Differential diagnosis ,medicine.symptom ,business - Abstract
Extranasopharyngeal angiofibroma which occurs in all head and neck regions is extremely rare. Unlike most angiofibromas which show nasal congestion and recurrent epistaxis as their symptoms, extranasopharyngeal angiofibromas (ENAF) may lead to various symptoms depending on their location. Nasal septum is the most frequent site of origin of ENAF. No study of ENAF originating in natural ostium of maxillary sinus has been reported. We present a case of 27-year-old male who has extranasopharyngeal angiofibroma arising from the natural ostium of maxillary sinus in an adult patient whose symptom was right sided nasal obstruction. With this study, although uncommon, angiofibroma should be considered as a differential diagnosis in patient with mass lesion in the middle nasal meatus. more...
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- 2021
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9. Bannayan‐Riley‐Ruvalcaba syndrome with gingival hyperpigmentation and facial papules
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Alexander Hicks, Sharon E. Albers, Eden M. Lyons, Jeffrey P. Zwerner, and Eva B. Niklinska
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Male ,Gingival hyperpigmentation ,congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,Adolescent ,Genotype ,business.industry ,PTEN Phosphohydrolase ,Context (language use) ,Dermatology ,Cowden syndrome ,medicine.disease ,Angiofibromas ,Phenotype ,Bannayan–Riley–Ruvalcaba syndrome ,Hyperpigmentation ,PTEN HAMARTOMA TUMOR SYNDROME ,Pediatrics, Perinatology and Child Health ,medicine ,Humans ,Hamartoma Syndrome, Multiple ,Facial papules ,business - Abstract
One of the distinctive cutaneous manifestations of Bannayan-Riley-Ruvalcaba syndrome (BRRS), a PTEN hamartoma tumor syndrome, is penile pigmented macules. We present a 13-year-old boy with gingival hyperpigmentation along with facial and ear angiofibromas in the context of a BRRS-concordant phenotype and PTEN hamartoma tumor syndrome genotype. To our knowledge, these two findings have not been previously reported with BRRS and may expand the known phenotype of this disorder. more...
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- 2021
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10. Colagenomas: manifestación cutánea de neoplasia endocrina múltiple tipo 1 (MEN 1)
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Natalia Giraldo, Laura Pulgarín, Simón Gallo, Carlos Montealegre, and Alejandro Roman-González
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Pathology ,medicine.medical_specialty ,business.industry ,Collagenomas ,General Medicine ,medicine.disease ,Angiofibromas ,Cushing Disease ,medicine.anatomical_structure ,Anterior pituitary ,Female patient ,Medicine ,Endocrine system ,business ,Multiple endocrine neoplasia ,Primary hyperparathyroidism - Abstract
La neoplasia endocrina múltiple tipo 1 (MEN 1) es un síndrome hereditario raro caracterizado por la aparición de tumores de paratiroides, páncreas e hipófisis anterior, que en algunos casos se acompaña de neoplasias cutáneas benignas, como angiofibromas y colagenomas, cuya aparición puede preceder a las manifestaciones endocrinológicas. Se presenta el caso de una paciente de 23 años, en quien se diagnosticó hiperparatiroidismo primario, enfermedad de Cushing, tumor neuroendocrino de páncreas y múltiples colagenomas, que aparecieron antes de las manifestaciones hormonales. more...
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- 2020
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11. Facial Papules in Birt-Hogg-Dubé Syndrome: A Growing Spectrum of Pathologic Findings
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Lauren G. Yi, Richard H. Flowers, Ricardo Guerra, and Sam B. Wu
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Skin manifestations ,medicine.medical_specialty ,business.industry ,Genodermatosis ,Histology ,medicine.disease ,Dermatology ,Angiofibromas ,Birt–Hogg–Dubé syndrome ,medicine ,Folliculin Gene ,Facial papules ,business ,Novel mutation - Abstract
Birt-Hogg-Dubé syndrome is rare genodermatosis caused by a mutation in the folliculin gene. It is characterized by pulmonary cysts, renal tumors, and a variety of skin manifestations including trichodiscomas, fibrofolliculomas, less commonly reported angiofibromas, and perifollicular fibromas. These cutaneous lesions have overlapping histopathologic features. Here we report a case of a man presenting with facial papules and nonspecific histology found to have Birt-Hogg-Dubé syndrome and a novel mutation in the folliculin gene. Our aim is to raise awareness of the spectrum of associated cutaneous and pathologic findings in Birt-Hogg-Dubé syndrome, which are not all included in the diagnostic criteria. more...
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- 2020
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12. A Novel Case of Mammary-Type Myofibroblastoma With Sarcomatous Features
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Laura J. Tafe, Konstantinos Linos, Alexander M. Strait, and Kristen E. Muller
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Male ,0301 basic medicine ,Pathology ,medicine.medical_specialty ,DNA Copy Number Variations ,Microarray ,Breast Neoplasms, Male ,Pathology and Forensic Medicine ,Diagnosis, Differential ,Neoplasms, Muscle Tissue ,03 medical and health sciences ,0302 clinical medicine ,Biomarkers, Tumor ,medicine ,Humans ,Breast ,Mammary-Type Myofibroblastoma ,Mitosis ,Aged ,Chromosomes, Human, Pair 13 ,biology ,Retinoblastoma protein ,medicine.disease ,Neoplasms, Complex and Mixed ,Angiofibromas ,030104 developmental biology ,030220 oncology & carcinogenesis ,Spindle cell lipoma ,biology.protein ,Surgery ,Sarcoma ,Anatomy ,Myofibroblastoma - Abstract
Mammary-type myofibroblastoma (MFB) is a benign spindle cell tumor of the breast and soft tissue characterized by 13q14 alterations leading to loss of Rb-1 protein expression, a feature shared among spindle cell lipoma and cellular angiofibroma. In this article, we present a novel case of MFB arising in the left breast of a 70-year old man that microscopically showed an abrupt transition from classic MFB morphology to an area with cytologic atypia and mitotic activity, akin to sarcomatous transformation described in cellular angiofibromas. A thorough workup of the molecular underpinnings of both components using chromosomal microarray and next-generation sequencing platforms supported a clonal relationship. Nearly identical copy number changes, including a single copy loss of 13q14, were found in both components; in addition, the sarcomatous component harbored biallelic TP53 alterations. It is important for pathologists to recognize that sarcomatous features can occur in mammary-type MFB to arrive at the correct diagnosis. more...
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- 2020
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13. Mandibular Intraosseous Angiofibroma—A Rare Clinical Entity
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R. John Tannyhill, Ami Amini-Salari, David Y. Ahn, and Krzysztof Glomski
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medicine.medical_specialty ,business.industry ,Nasopharyngeal neoplasm ,Mandible ,Angiofibroma ,medicine.disease ,Angiofibromas ,Otorhinolaryngology ,Male patient ,Rare case ,medicine ,Surgery ,Radiology ,Oral Surgery ,business - Abstract
Angiofibromas located in the maxillofacial region are rare and almost exclusively occur in adolescent males. These benign tumors are highly vascular, locally invasive, and commonly found in the nasopharyngeal space. In the present report, we describe a very rare case of an intraosseous mandibular angiofibroma in a 23-year-old male patient with histomorphologic and molecular confirmation. This type of tumor occurring in the mandible has been reported previously only once, to the best of our knowledge. more...
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- 2020
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14. Cellular Angiofibroma: A Rare Vulvar Tumor Case Report
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Cátia Sofia Guilherme Ferreira Pais, Marta Andrea Ferreira Fernandes, Nuno Jorge Lopes Dias, Ana Fachada, and António Manuel Leitão Loureiro Pipa
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Vulvovaginal Region ,Pathology ,medicine.medical_specialty ,Vulvar Neoplasms ,business.industry ,Mesenchymal Tumor ,Obstetrics and Gynecology ,Middle Aged ,Angiofibroma ,Angiofibromas ,Vulva ,Diagnosis, Differential ,medicine.anatomical_structure ,Bartholin Cyst ,Cellular angiofibroma ,Vulvar Tumor ,Humans ,Medicine ,Immunohistochemistry ,Female ,business - Abstract
Cellular angiofibroma (CA)is a rare benign mesenchymal tumor. In women, it occurs mainly in the vulvovaginal region, with vulvar location in 70% of the cases. Its clinical presentation is nonspecific and similar to several other vulvar tumors of different cellular origins. Thus, its histological and immunohistochemical features allow distinction from other tumors. Cellular angiofibromas have good prognosis, despite some risk of relapse. The authors present the case of a 49-year-old woman with a bulky right vulvar lesion, for which the preoperative diagnosis was a Bartholin cyst, but the histological and immunohistochemical evaluation yielded a CA.O angiofibroma celular é um tumor mesenquimatoso benigno e raro. Nas mulheres, surge principalmente na região vulvo-vaginal, com localização vulvar em 70% dos casos. A sua apresentação clínica é inespecífica e semelhante a vários outros tumores vulvares de diferentes origens celulares. Assim, são as suas características histológicas e imunohistoquímicas que permitem a diferenciação entre eles. Os angiofibromas celulares têm bom prognóstico, embora apresentem algum risco de recidiva. Os autores apresentam o caso de uma mulher de 49 anos de idade com uma lesão vulvar direita volumosa, cujo diagnóstico pré-operatório era de quisto da glândula de Bartholin, mas cujo exame histológico e imunohistoquímico revelaram tratar-se de um angiofibroma celular. more...
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- 2020
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15. Safety and Efficacy of the Sirolimus Gel for TSC Patients With Facial Skin Lesions in a Long-Term, Open-Label, Extension, Uncontrolled Clinical Trial
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Hironori Niizeki, Chiharu Tateishi, Kazuyoshi Fukai, Shigeki Shimasaki, Yasuyuki Fujita, Izumi Hamada, Tatsuro Takahata, Akihiko Asahina, Yuuki Ohno, Hiroyuki Murota, Hiroo Yokozeki, Masaaki Ogai, Kenji Shimizu, Mari Wataya-Kaneda, Yuichi Yoshida, Kazue Yoshida, and Hiroshi Nagai more...
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medicine.medical_specialty ,Erythema ,Dermatology ,Hypomelanotic macules ,030207 dermatology & venereal diseases ,03 medical and health sciences ,Tuberous sclerosis ,Long-term administration ,0302 clinical medicine ,Dry skin ,medicine ,Clinical endpoint ,Angiofibromas ,Adverse effect ,Acne ,Original Research ,Topical sirolimus ,business.industry ,Skin lesions ,Hypomelanotic macule ,medicine.disease ,Discontinuation ,Cephalic plaques ,Clinical trial ,Sirolimus gel ,Tuberous sclerosis complex ,RL1-803 ,030220 oncology & carcinogenesis ,medicine.symptom ,business - Abstract
Introduction Our previous clinical studies have demonstrated the short-term efficacy and safety of the sirolimus gel for patients with tuberous sclerosis complex (TSC). However, long-term clinical evidence is lacking. Our objective was to assess the safety and efficacy of long-term treatment with the sirolimus gel for the skin lesions of TSC patients. Methods We conducted a multicenter, open-label, uncontrolled clinical trial in 94 Japanese patients with TSC. Patients applied the 0.2% sirolimus gel on their face or head twice daily for > 52 weeks (maximum 136 weeks for safety). The safety endpoints were the rate of adverse event (AE)-caused discontinuation (primary endpoint) and the incidence of AEs. The efficacy endpoint was the response rate of angiofibromas, cephalic plaques, and hypomelanotic macules. Results Among 94 enrolled patients (mean age, 21 years; range 3–53 years), the rate of AE-caused discontinuation was 2.1% (2/94 patients). Although application site irritation and dry skin occurred relatively frequently, none of the drug-related AEs were serious; most of the drug-related AEs resolved rapidly. The major drug-related AEs (≥ 5% in incidence) were application site irritation (30.9%), dry skin (27.7%), acne (20.2%), eye irritation (8.5%), pruritus (8.5%), erythema (7.4%), dermatitis acneiform (6.4%), and dermatitis contact (5.3%). The response rates of angiofibromas, cephalic plaques, and hypomelanotic macules were 78.2% [95% confidence interval (CI) 68.0–86.3%], 66.7% (95% CI 51.1–80.0%), and 72.2% (95% CI 46.5–90.3%), respectively. Conclusions The gel was well tolerated for a long time by patients with TSC involving facial skin lesions and continued to be effective. Trial Registration ClinicalTrials.gov identifier: NCT02634931. more...
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- 2020
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16. Scleromyxedema histopathologically mimicking hypercellular fibrous papules (angiofibomas): Case report of an unusual histopathological presentation
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Viktoryia Kazlouskaya, Edward Heilman, and Neelam Khan
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medicine.medical_specialty ,Pathology ,Histology ,business.industry ,CD68 ,Mucin ,Dermatology ,Angiofibroma ,medicine.disease ,Angiofibromas ,Colloidal iron ,Pathology and Forensic Medicine ,030207 dermatology & venereal diseases ,03 medical and health sciences ,0302 clinical medicine ,030220 oncology & carcinogenesis ,Scleromyxedema ,medicine ,Histopathology ,Factor XIIIa ,business - Abstract
Scleromyxedema (SMX) is an inflammatory condition of unknown etiology strongly associated with monoclonal gammopathy. Classical histopathology of SMX is characterized with the triad of diffuse mucin deposits, increased amount of collagen, and presence of stellate fibroblasts. Herein, we report an unusual histopathological variant of SMX in a 41-year-old female with lesions of the nose histopathologically mimicking cellular angiofibromas. The dome-shaped papules were characterized by increased collagen bundles and fascicles of spindle cells. Widened vessels were seen at the periphery of the proliferation. Cells expressed CD68. Factor XIIIa was expressed only by dendritic cells. The mucin was highlighted with colloidal iron. In sum, we draw attention to this unusual variant of SMX, which should be suspected in a setting of multiple "angiofibromas/fibrous papules" on the face with presence of mucin. more...
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- 2020
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17. Cutaneous angiofibroma: A clinching evidence for diagnostic workup of tuberous sclerosis complex
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Ashwini Kulkarni, Bhushan M. Warpe, Shweta Joshi-Warpe, and Bhagyashree P Mundhe
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Cortical tubers ,Pathology ,medicine.medical_specialty ,congenital, hereditary, and neonatal diseases and abnormalities ,medicine.diagnostic_test ,business.industry ,fungi ,face ,food and beverages ,Magnetic resonance imaging ,General Medicine ,Angiofibroma ,tuberous sclerosis complex ,lcsh:RL1-803 ,medicine.disease ,Angiofibromas ,White matter ,Tuberous sclerosis ,medicine.anatomical_structure ,angiofibroma ,Cortex (anatomy) ,Subependymal nodules ,medicine ,lcsh:Dermatology ,business - Abstract
Tuberous sclerosis complex (TSC) or Bourneville's disease is a genetic multisystem disorder of multisite hamartomas. Majority of TSC cases are sporadic. TSC typically presents in the first decade of life and has a reported incidence of 1:6000–12,000, with intracranial involvement in the form of cortical tubers or subependymal nodules. Tubers (potato-like nodules) are triangular-shaped lesions centered at the cortex/juxtacortical, with apex oriented “inward” toward the ventricles, on magnetic resonance imaging (MRI) of the brain. Tubers represent cortical glioneuronal hamartomas and consist of focal distortions in the cellular organization and morphology, which extend into the underlying (subcortical) white matter. Skin lesions with a history of seizures can be an eye-opener to diagnose angiofibromas and its variant, which can lead to further effective workup such as ophthalmoscopy and MRI of the brain to diagnose TSC, just like in our case. more...
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- 2020
18. Birthmark: Journey from aesthetic to unalluring
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Vikram Singh, Sakshi Sharma, Mamta Singh, and Pratiksha Hada
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lcsh:Medical physics. Medical radiology. Nuclear medicine ,medicine.medical_specialty ,Port wine ,genetic structures ,sws ,lcsh:R895-920 ,Sturge–Weber syndrome ,Glaucoma ,Epilepsy ,Age groups ,medicine ,Radiology, Nuclear Medicine and imaging ,encephalotrigeminal angiomatosis ,Birthmark ,General Dentistry ,angiofibromas ,portwine stain ,neurocutaneous ,business.industry ,Angiomatosis ,medicine.disease ,Dermatology ,tram- track calcifications ,lcsh:RK1-715 ,Otorhinolaryngology ,lcsh:Dentistry ,sense organs ,business ,psychological phenomena and processes - Abstract
Encephalotrigeminal Angiomatosis, also known as Sturge Weber Syndrome (SWS), Dimitri disease, Sturge Kalischer Weber Syndrome is specifically non familial, congenital rare disorder consisting of hamartomatous malformations that may affect eye, skin, and CNS. Failure of proper vascular development is believed to be the most likely cause of the condition. The malformed blood vessels may lead to port wine stains, epilepsy, and glaucoma depending on its location. We are presenting here a triad of case reports on Sturge Weber Syndrome representing individuals belonging to varied age groups. more...
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- 2020
19. A Rare Case of Angiofibroma Mimicking Fibrous Epulis in Posterior Gingival Mucosa
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Annete Nabila, Ni Putu Mira Sumarta, and Okky Prasetio
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Pathology ,medicine.medical_specialty ,medicine.diagnostic_test ,Epulis ,business.industry ,Soft tissue ,General Medicine ,Angiofibroma ,medicine.disease ,Angiofibromas ,Gingival Angiofibroma ,Biopsy ,Gingival mucosa ,medicine ,Fibrous epulis ,business - Abstract
Angiofibromas are highly vascular, locally aggressive but histologically benign tumors. A 40-year-old woman complaint of pedunculated mass in the right gingiva of the posterior region since 7 months earlier. Surgical excisional biopsy under general anesthesia was performed. Histopathological examination showed an angiofibroma. Angiofibromas are localized in the superficial soft tissue and the gingiva could have a similar clinical appearance as an epulis. Definitive diagnosis was determined through histopathological analysis. more...
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- 2021
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20. Juvenile Nasopharyngeal Angiofibromas: Clinico-Radiological Evaluation
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Shweta Sharma, Mir Rizwan Aziz, Bharat Bhushan Sharma, Shashi Sharma, and Akriti Sharma
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medicine.medical_specialty ,medicine.diagnostic_test ,Juvenile nasopharyngeal angiofibroma ,business.industry ,Computed tomography ,Case presentation ,medicine.disease ,Angiofibromas ,Radiological weapon ,medicine ,Vascular tumor ,Radiology ,Young adult ,Fibroma ,business - Abstract
Background: Juvenile nasopharyngeal angiofibroma (JNA) is also known as bleeding fibroma since it is highly vascular tumour.. This is a benign tumour that is locally aggressive and bleeds in prepubescent and young adults. The tumour expands due to the potential space available in the nasopharyngeal area without much of the resistance JNA requires early and accurate diagnosis by cross sectional modalities with the background setting of clinical symptomatology. This will helpful in early management and can avoid various future complications. Case Presentation: We present two cases of JNAs where the role of contrast enhanced computed tomography (CECT) is highlighted. These two examples of adolescent nasopharyngeal angiofibromas (JNAs) were diagnosed at at differing stages with the help of CECT reformatted reconstructed 3D images.. Conclusion: We lay a high value on the clinico-radiological evaluation in concluding the diagnosis. The adequate pre-operative radiological staging is required for the surgical management more...
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- 2021
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21. Frontoethmoidal Extranasopharyngeal Angiofibroma With Orbital Pyocele
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Ramesh Babu Telugu, Regi Kurien, Lalee Varghese, Raghav Mehan, Meera Thomas, and Vedantam Rupa
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medicine.medical_specialty ,Extranasopharyngeal angiofibroma ,business.industry ,Nose Neoplasms ,Nasopharyngeal Neoplasms ,Angiofibroma ,Angiofibromas ,Respiratory Tract Neoplasms ,Diagnosis, Differential ,Vascular Tumors ,Otorhinolaryngology ,Humans ,Medicine ,Radiology ,business - Abstract
Significance Statement Extranasopharyngeal angiofibromas (ENA) are rare vascular tumors that do not conform to the clinical characteristics of typical nasopharyngeal angiofibromas. We present the management of an angiofibroma in a rare site, within the frontal sinus with a concomitant orbital pyocele, which was completely excised via an endoscopic approach. ENAs should be considered as a differential diagnosis in patients with sinonasal mass and epistaxis. Awareness of this rare entity will avoid radical surgery thus decreasing postoperative morbidity. more...
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- 2020
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22. Spontaneous Involution of Juvenile Nasopharyngeal Angiofibromas: Report of a Case
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Janice T Chua, Dillon Cheung, Joseph A I Choy, Edward C. Kuan, Ronald Sahyouni, Naveen D. Bhandarkar, and Jack Birkenbeuel
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Male ,Surgical resection ,medicine.medical_specialty ,Neoplasm, Residual ,Adolescent ,Juvenile nasopharyngeal angiofibroma ,Angiofibroma ,Nasopharynx ,Humans ,Juvenile ,Medicine ,Involution (medicine) ,Tumor growth ,business.industry ,Standard treatment ,Endoscopy ,Nasopharyngeal Neoplasms ,Magnetic Resonance Imaging ,Angiofibromas ,Surgery ,Natural history ,Treatment Outcome ,Otorhinolaryngology ,Tomography, X-Ray Computed ,business ,Follow-Up Studies - Abstract
Juvenile nasopharyngeal angiofibroma (JNA) is a locally aggressive tumor that predominantly affects adolescent males. Surgical resection is generally considered the standard treatment for both primary and recurrent tumors, regardless of staging. The natural history of these tumors, particularly when untreated or in the setting of residual tumor, is not well characterized. In this article, we report a case of true spontaneous JNA involution. Although the involution of residual tumor after surgical resection has previously been reported, to our knowledge, this is the first documented case of spontaneous JNA involution following a period of tumor growth post-treatment. Laryngoscope, 131:1455-1457, 2021. more...
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- 2020
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23. Atypical Ocular Coloboma in Tuberous Sclerosis-2: Report of Two Novel Cases
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Roberto Caputo, Renzo Guerrini, Valerio Conti, Giacomo Bacci, Francesco Mari, and Silvio Polizzi
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Male ,Fovea Centralis ,congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,DNA Mutational Analysis ,Visual Acuity ,Iris ,Retina ,Tuberous sclerosis ,chemistry.chemical_compound ,Tuberous Sclerosis ,Tuberous Sclerosis Complex 2 Protein ,Humans ,Medicine ,Abnormalities, Multiple ,Papilledema ,business.industry ,Retinal ,DNA ,medicine.disease ,Dermatology ,Iris coloboma ,Angiofibromas ,eye diseases ,nervous system diseases ,Coloboma ,Ophthalmology ,medicine.anatomical_structure ,chemistry ,Child, Preschool ,Mutation ,Female ,sense organs ,Neurology (clinical) ,Eyelid ,TSC1 ,medicine.symptom ,TSC2 ,business ,Tomography, Optical Coherence - Abstract
Tuberous sclerosis complex (TSC) is an autosomal dominant multisystemic disorder caused by mutations in either TSC1 or TSC2 genes and is characterized by hamartomas in multiple organs. The most frequent and best-known ocular manifestation in TSC is the retinal hamartoma. Less frequent ocular manifestations include punched out areas of retinal depigmentation, eyelid angiofibromas, uveal colobomas, papilledema, and sector iris depigmentation. In this article, we report 2 patients carrying known pathogenic variants in the TSC2 gene who exhibited an atypical, unilateral, iris coloboma associated with localized areas of retinal dysembryogenesis. more...
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- 2020
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24. Topical rapamycin combined with calcitriol in treating angiofibromas in tuberous sclerosis complex
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Chuan-Mo Lee
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Sirolimus ,medicine.medical_specialty ,Calcitriol ,business.industry ,Dermatology ,Angiofibroma ,medicine.disease ,Angiofibromas ,Tuberous sclerosis ,Double-Blind Method ,Tuberous Sclerosis ,medicine ,Humans ,Prospective Studies ,Facial Neoplasms ,business ,Prospective cohort study ,Facial neoplasm ,medicine.drug - Published
- 2020
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25. Use of Topical Rapamycin as Maintenance Treatment after a Single Session of Fractionated CO2 Laser Ablation: A Method to Enhance Percutaneous Drug Delivery
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Jihee Kim, Jongwook Oh, Ju Hee Lee, and Won Jai Lee
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medicine.medical_specialty ,congenital, hereditary, and neonatal diseases and abnormalities ,Percutaneous ,CO2 laser ,medicine.medical_treatment ,Case Report ,Dermatology ,Angiofibroma ,Malignant transformation ,030207 dermatology & venereal diseases ,03 medical and health sciences ,Tuberous sclerosis ,0302 clinical medicine ,medicine ,Sirolimus ,business.industry ,Cosmesis ,Ablation ,medicine.disease ,Angiofibromas ,Tuberous sclerosis complex ,030220 oncology & carcinogenesis ,business ,medicine.drug - Abstract
Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous disorder with an incidence of approximately 1 in 5,000 to 10,000 live births. TSC has various clinical manifestations such as multiple hamartomas in systemic organs, including the skin. Angiofibromas are the most common skin lesions in patients with TSC. Although benign, angiofibromas develop in childhood and puberty, and can be psychosocially disfiguring for patients. Skin lesions in TSC, specifically angiofibromas, have no significant risk of malignant transformation after puberty; thus, they require no treatment if not prominent. However, the presentation of TSC is important owing to its impact on patient cosmesis. Surgical treatment and laser therapy are the mainstream treatments for angiofibromas. Although the evidence is limited, topical mammalian target of rapamycin inhibitors such as sirolimus (rapamycin) are effective in facial angiofibroma treatment. We describe an adult patient with an angiofibroma who had an excellent response to treatment with topical rapamycin after a single session of carbon dioxide (CO2) laser ablation. The patient showed no sign of relapse or recurring lesions for a year. CO2 laser ablation may serve as a new paradigm of treatment for angiofibromas in TSC. Since the selection of laser devices can be limited for some institutions, we suggest a rather basic but highly effective approach for angiofibroma treatment that can be generally applied with the classic CO2 device. more...
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- 2019
26. Tuberous Sclerosis Complex Associated Lymphangioleiomyomatosis Presenting with Spontaneous Pneumothorax and Renal Angiomyolipomas
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Shish Mohammad Sarkar, Akm Mosharraf Hossain, Manzurul Ibrahim Musa, Farjana Binte Habib, Mohammed Mirazur Rahman, and Nazmul Hasan
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medicine.medical_specialty ,Angiomyolipoma ,medicine.diagnostic_test ,business.industry ,Physical examination ,General Medicine ,medicine.disease ,Angiofibromas ,Shagreen ,Tuberous sclerosis ,medicine.anatomical_structure ,Pneumothorax ,Lymphangioleiomyomatosis ,Medicine ,Abdomen ,Radiology ,business - Abstract
Tuberous sclerosis complex (TSC) is a rare autosomal dominant disorder manifested by involvement of multisystem including skin, central nervous system, heart, kidneys and eyes. Lymphangioleiomyomatosis (LAM) is also a multisystem disorder that primarily affects the lungs. We report a case of tuberous sclerosis complex associated lymphangio-leiomyomatosis (TSC-LAM) in a 26-year-old female patient who was presented with spontaneous pneumothorax and renal angiomyolipomas. In clinical examination; We found multiple angiofibromas over her face, shagreen patches over upper and lower back and ungual fibromas in both fingers and toes. HRCT of chest revealed right sided pneumothorax with multiple thin walled cysts in both lungs. Ultrasonogram (USG) and Computer Tomography (CT) scan of abdomen revealed bilateral angiomyolipomas. We managed her pneumothorax with intercostal chest tube drainage and oxygen inhalation. Bangladesh Med J. 2019 May; 48 (2): 51-54 more...
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- 2019
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27. The Endoscopic Endonasal Approach for the Treatment of Juvenile Angiofibromas
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José Luis Llorente and Fernando López
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Adult ,Male ,Natural Orifice Endoscopic Surgery ,medicine.medical_specialty ,Adolescent ,Nose Neoplasms ,Blood Loss, Surgical ,Juvenile angiofibroma ,Angiofibroma ,Young Adult ,03 medical and health sciences ,Postoperative Complications ,0302 clinical medicine ,Blood loss ,medicine ,Humans ,Stage iib ,Juvenile ,Stage (cooking) ,Child ,030223 otorhinolaryngology ,Neoplasm Staging ,Retrospective Studies ,business.industry ,Medical record ,Retrospective cohort study ,General Medicine ,Magnetic Resonance Imaging ,Angiofibromas ,Surgery ,Female ,business ,030217 neurology & neurosurgery - Abstract
Background and Objectives Juvenile angiofibroma (JA) is a benign tumour, for which the treatment of choice is surgery. It may be associated with significant morbidity because of its anatomical location and its locally destructive growth pattern. Severe haemorrhage constitutes a high risk in JA and its surgical management can be complex. The management of JA remains a challenge. The objective of this study was to review a series of patients with JA treated via the endonasal/endoscopic approach. Material and Methods Medical records of patients operated for JA were reviewed. Main outcome measures: tumour stage, intraoperative blood loss, complications and persistence/recurrence rates. Results A total of 30 male patients and one female were included. The mean age was 17 years. Using the Radkowski classification, one JA was classified as stage I, 5 stage IIA, 9 stage IIB, 4 stage IIC, 10 stage IIIA and 2 stage IIIB. Thirty-nine percent of the JA was classified as advanced stage JA (IIIA and IIIB). The mean blood loss was 1.156 mL except in one case, no significant complications were observed. Tumour persistence/recurrence was observed in 2 JA (6%), at the end of the follow-up. Mean postoperative follow-up time was 86 months. Conclusions This retrospective study supports the notion that endonasal endoscopic approaches for a JA are a feasible option associated with good long-term results. more...
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- 2019
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28. Topical use of mammalian target of rapamycin inhibitors in dermatology: A systematic review with meta-analysis
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Sophie Leducq, Elsa Tavernier, Bruno Giraudeau, Annabel Maruani, MethodS in Patients-centered outcomes and HEalth ResEarch (SPHERE), Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Nantes - UFR des Sciences Pharmaceutiques et Biologiques, Université de Nantes (UN)-Université de Nantes (UN), Institut National de la Santé et de la Recherche Médicale (INSERM), and Université de Tours-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Nantes - UFR des Sciences Pharmaceutiques et Biologiques more...
- Subjects
medicine.medical_specialty ,Skin Neoplasms ,[SDV]Life Sciences [q-bio] ,Port-Wine Stain ,Dermatology ,Administration, Cutaneous ,Angiofibroma ,Placebo ,law.invention ,030207 dermatology & venereal diseases ,03 medical and health sciences ,Tuberous sclerosis ,0302 clinical medicine ,Randomized controlled trial ,Tuberous Sclerosis ,law ,medicine ,Humans ,Psoriasis ,Adverse effect ,Sirolimus ,Antibiotics, Antineoplastic ,business.industry ,TOR Serine-Threonine Kinases ,medicine.disease ,Angiofibromas ,3. Good health ,030220 oncology & carcinogenesis ,Meta-analysis ,Topical Sirolimus ,business ,medicine.drug - Abstract
Background Systemic mammalian target of rapamycin (mTOR) inhibitors are currently used in many dermatologic indications. Their topical use is recent and poorly codified. Objective To provide an overview of the topical use of mTOR inhibitors in dermatologic conditions and evaluate their efficacy and safety. Methods A literature search was performed in January 2017. Reports of all studies investigating the use of topical mTOR inhibitors in any dermatology diseases were included. The exclusion criteria were systemic use and mucosal administration. Results We included 40 studies with a total of 262 patients. In all, 11 dermatologic conditions were found, the most frequent being angiofibromas linked to tuberous sclerosis complex (157 patients). Topical mTOR inhibitors were significantly more efficient than placebo for angiofibromas (relative risk, 2.52; 95% confidence interval, 1.27-5.00; I2 = 0%). The median concentration of sirolimus was 0.1%, with a median treatment duration of 12 weeks. Topical mTOR inhibitors were well tolerated, with only mild or moderate local side effects (mostly irritative) reported. Blood level of sirolimus was not detected in 90% of patients. Limitations High heterogeneity in most studies. Conclusion This systematic review supports the efficacy of topical sirolimus for angiofibromas linked to tuberous sclerosis complex, with only local side effects reported. Other indications require further research. more...
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- 2019
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29. Surgical management of juvenile nasopharyngeal angiofibroma
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Brad E. Zacharia, Daniel W. Nuss, Carl H. Snyderman, and Meghan N. Wilson
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medicine.medical_specialty ,medicine.diagnostic_test ,business.industry ,Juvenile nasopharyngeal angiofibroma ,Disease ,Angiofibromas ,Resection ,Otorhinolaryngology ,Biopsy ,Medicine ,Juvenile ,Surgery ,Radiology ,business ,Vascular supply ,Anterior skull base - Abstract
Juvenile nasopharyngeal angiofibromas are benign yet aggressive anterior skull base tumors that occur almost exclusively in male adolescents. Diagnosis is typically made through radiographic findings and clinical characteristics; biopsy is not recommended due to the vascular nature of the disease. As with most tumors, there is a spectrum of tumor extent and invasion. In juvenile nasopharyngeal angiofibromas, one of the most important components of extent is the vascular supply. Once the vascular supply is identified, a systematic approach to resection can be planned. The majority of these tumors can be excised endoscopically, and those approaches are the focus of this article. more...
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- 2019
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30. Fibrous papules on the lips
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Xiuyan Shi, Lin Ma, and Bin Zhang
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medicine.medical_specialty ,business.industry ,Mucosal lesions ,food and beverages ,Dermatology ,Angiofibroma ,medicine.disease ,Angiofibromas ,Lip ,030207 dermatology & venereal diseases ,03 medical and health sciences ,Tuberous sclerosis ,0302 clinical medicine ,Tuberous Sclerosis ,030220 oncology & carcinogenesis ,Pediatrics, Perinatology and Child Health ,Medicine ,Humans ,In patient ,Fibrous Papule ,Presentation (obstetrics) ,Facial Neoplasms ,business - Abstract
Multiple angiofibromas are commonly found in patients with tuberous sclerosis complex. We report a rare presentation of multiple congenital fibrous papules occurring only on the lips with no syndromic associations. more...
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- 2021
31. Preoperative Embolization of Fisch Grades II–IVa Juvenile Nasopharyngeal Angiofibromas: Transarterial Embolization in the Age of Onyx
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Muhammad Waqas, Jason M Davies, Jaims Lim, Michael K Tso, Adnan H. Siddiqui, Kunal Vakharia, and Elad I. Levy
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onyx ,medicine.medical_specialty ,medicine.diagnostic_test ,business.industry ,Juvenile nasopharyngeal angiofibroma ,medicine.medical_treatment ,Neurosurgery ,General Engineering ,Angiofibromas ,tumor devascularization ,Surgery ,Otolaryngology ,juvenile nasopharyngeal angiofibroma ,Preoperative embolization ,transarterial embolization ,Blood loss ,Transarterial embolization ,medicine ,Fluoroscopy ,Embolization ,Headaches ,medicine.symptom ,business - Abstract
Objective The current mainstay treatment for juvenile nasopharyngeal angiofibromas (JNAs) is surgical resection, but embolization of primary feeding arteries through endovascular transarterial and direct tumoral puncture embolizations with various agents has been described. We describe a single institutional experience with JNA embolization utilizing Onyx (Medtronic, Dublin, Ireland). Methods A retrospective records review was performed to identify patients who underwent embolization for devascularization of Fisch grades II-IVa JNA (tumor extension beyond the sphenopalatine region) before surgical resection between 2010 and 2019. Fluoroscopy time, grade, intraoperative blood loss, and clinical follow-up data were compiled. Tumor devascularization percentage was calculated using ImageJ software (public domain, BSD-2) by measuring the ratio of preoperative and postoperative embolization tracing. Results Five consecutive patients (ages 12-16 years [average 14 years]; all male) with JNAs underwent preoperative transarterial embolizations performed under general anesthesia. All patients presented with epistaxis; two also presented with headaches. Fisch grades were II in two patients, IIIa in two, and IVa in one. The patient with the grade IVa lesion underwent direct transtumoral puncture and Onyx embolization. The mean percentage of all tumor devascularizations postembolization was 86.0±9.7%.Complete resection 24-48 hours postembolization was obtained for grades II and IIIa lesions with more...
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- 2021
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32. Folliculocystic and Collagen Hamartoma: A Subset of Fibrous Cephalic Plaque
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Kyle Norton, Anita S. Savell, and Michael R. Heaphy
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Pathology ,medicine.medical_specialty ,congenital, hereditary, and neonatal diseases and abnormalities ,business.industry ,General Engineering ,Dermatology ,tuberous sclerosis complex ,030204 cardiovascular system & hematology ,medicine.disease ,Hypomelanotic macule ,Angiofibromas ,fibrous cephalic plaque ,folliculocystic and collagen hamartoma ,Shagreen ,03 medical and health sciences ,Tuberous sclerosis ,0302 clinical medicine ,medicine ,Cutaneous hamartoma ,hamartoma ,Hamartoma ,business ,030217 neurology & neurosurgery ,Collagen hamartoma - Abstract
Tuberous sclerosis complex is known to cause a variety of cutaneous hamartomas, most commonly hypomelanotic macules, angiofibromas, shagreen patches, and fibrous cephalic plaques. In recent years, a new cutaneous hamartoma that bears physical and histological resemblance to fibrous cephalic plaque has been proposed called folliculocystic and collagen hamartoma. The primary difference between the two diagnoses is the histologic presence of infundibular cysts in the latter. However, some authors have called into question if the two diagnoses are truly distinct. In this case report, we present a patient with tuberous sclerosis complex and fibrous cephalic plaque with infundibular cysts and propose that the presence of cysts should be incorporated into the possible histologic features of fibrous cephalic plaque. more...
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- 2021
33. Nasal septal angiofibroma: less vascular behaviour than observed in radiological imaging, a rare case in a 51-year-old woman
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C Burgess and S Law
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Nasal cavity ,medicine.medical_specialty ,Biopsy ,Nose Neoplasms ,030230 surgery ,Angiofibroma ,03 medical and health sciences ,0302 clinical medicine ,otorhinolaryngologic diseases ,medicine ,Nasal septum ,Humans ,Nasal polyps ,Nasal Septum ,Hemangiopericytoma ,Pyogenic granuloma ,business.industry ,Endoscopy ,General Medicine ,respiratory system ,Middle Aged ,medicine.disease ,Angiofibromas ,Magnetic Resonance Imaging ,Surgery ,medicine.anatomical_structure ,Online Case Report ,Female ,Differential diagnosis ,Nasal Obstruction ,business ,Tomography, X-Ray Computed - Abstract
Angiofibroma of the head and neck is classically found in the nasopharynx of an adolescent male. We present a rare case where an angiofibroma was diagnosed in the anterior nasal septum of a 51-year-old woman, the eighth nasal septal angiofibroma reported in a female. This case highlights how an angiofibroma in an older woman has a less vascular behaviour than one in an adolescent male, even though radiological enhancement may suggest that the lesion is highly vascularised. Nasal septal angiofibromas can be managed by biopsy and removed by simple endoscopic resection with minimal haemorrhage. Although very rare, extranasopharyngeal angiofibroma/nasal septal angiofibroma should be included in the differential diagnosis when patients of all ages present with nasal obstruction secondary to a nasal mass, with or without epistaxis. more...
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- 2021
34. Tuberous Sclerosis: A Case Report and Review of the Literature
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Obed Cudjoe, Philip Narteh Gorleku, Albert Dayor Piersson, Klenam Dzefi-Tettey, and Emmanuel Kobina Mesi Edzie
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Pathology ,medicine.medical_specialty ,Dermatology ,tuberous sclerosis ,030204 cardiovascular system & hematology ,Pediatrics ,03 medical and health sciences ,Tuberous sclerosis ,0302 clinical medicine ,Subependymal nodules ,Subependymal zone ,medicine ,angiofibromas ,seizures ,Subependymal giant cell astrocytoma ,medicine.diagnostic_test ,renal angiomyolipomas ,business.industry ,subependymal giant cell astrocytoma ,General Engineering ,Genetic disorder ,food and beverages ,Magnetic resonance imaging ,medicine.disease ,medicine.anatomical_structure ,TSC1 ,TSC2 ,Radiology ,business ,030217 neurology & neurosurgery - Abstract
Tuberous sclerosis (TS) is a rare genetic disorder of autosomal-dominant inheritance. Mutations on either of the two genes Tuberous Sclerosis Complex 1 (TSC1) or Tuberous Sclerosis Complex 2 (TSC2) play a role and result in hamartomas involving many organs, like the brain, heart, kidneys, skin, lungs, and liver. This case report is about a four-year-old boy with facial angiofibromas, hypo-pigmented skin lesions on the lower back and dorsum of the right wrist, and previous history of seizures who was referred to the radiology department of the Korle Bu Teaching Hospital for Magnetic Resonance Imaging (MRI) of the brain. The MRI of the brain revealed subependymal giant cell astrocytomas, subependymal nodules, and cortical tubers. Ultrasonography of the abdomen also showed multiple angiomyolipomas and multiple simple cysts in both kidneys. The aim of this case report is to present the imaging findings and create awareness that this rare genetic disorder does exist in Ghana and advocate for formation of support groups for parents with children with tuberous sclerosis. more...
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- 2021
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35. Pre-Operative Embolization with Direct Onyx Tumoral Puncture Prior to Endoscopic Resection of Massive Juvenile Nasopharyngeal Angiofibroma
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Arif Janjua, Vinay Varadarajan, and Manraj K.S. Heran
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medicine.medical_specialty ,business.industry ,Juvenile nasopharyngeal angiofibroma ,medicine.medical_treatment ,Angiofibromas ,Skull ,medicine.anatomical_structure ,medicine.artery ,Medicine ,Embolization ,Radiology ,Internal carotid artery ,Stage (cooking) ,business ,Foramen rotundum ,Artery - Abstract
Juvenile angiofibromas (JNA) of Fisch Stage 3 and above often take a direct blood supply via the internal carotid artery (ICA) due to skull base involvement. These blood vessels, including the artery of the foramen rotundum and/or the vidian artery, are not easily accessed via traditional embolization methods. Employing a novel technique we can embolize residual vascular areas within these tumors prior to endoscopic resection. more...
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- 2021
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36. Miliary fibromas in tuberous sclerosis complex
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A M Cartron, D Buccine, Thomas N. Darling, A.M. Treichel, Joel Moss, and Chyi-Chia Richard Lee
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0301 basic medicine ,Adult ,Pathology ,medicine.medical_specialty ,congenital, hereditary, and neonatal diseases and abnormalities ,Dermatology ,Fibroma ,Angiofibroma ,Article ,Shagreen patch ,030207 dermatology & venereal diseases ,03 medical and health sciences ,Tuberous sclerosis ,0302 clinical medicine ,Tuberous Sclerosis ,medicine ,otorhinolaryngologic diseases ,Hamartoma ,Nevus ,Humans ,Retrospective Studies ,business.industry ,medicine.disease ,Angiofibromas ,Shagreen ,030104 developmental biology ,Infectious Diseases ,business - Abstract
Background Tuberous sclerosis complex (TSC) is a hamartoma syndrome characterized by multiple skin lesions, such as angiofibromas, shagreen patch and miliary fibromas (MiF). Objective To determine the clinical and histological features of MiF. Methods A retrospective analysis was conducted on 133 adults with TSC. Photography was used to characterize the appearance and location of MiF. Histological features in five skin samples from four individuals were evaluated by a board-certified dermatopathologist. Results MiF were observed in 19 of 133 (14%) individuals with TSC. MiF were 1- to 3-mm skin-coloured, sessile papules scattered on the back and rarely buttocks or thighs. Most were scattered in a bilaterally symmetric distribution, but others were asymmetric or associated with a shagreen patch. Histological features of MiF included expansion of the papillary and periadnexal dermis with variable hamartomatous abnormalities involving adjacent epithelial components. Conclusions MiF are distinct from other cutaneous lesions in TSC such as shagreen patches and angiofibromas. Recognition of this entity is important in defining the spectrum of TSC disease and reassuring individuals with TSC that these lesions are benign. more...
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- 2020
37. Uncommon presentations of angiofibroma in sinonasal tract: a two-case report/ Apresentações incomuns de angiofibromas no trato nasossinusal: relato de dois casos
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Regiane Matos Batista, Luigi Ferreira e Silva, Diego Gadelha Vaz, Ana Larisse Godim Barbosa, Leonardo Mendes Acatauassu Nunes, and Luísa Corrêa Janaú
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Frontal sinus ,medicine.medical_specialty ,Meatus ,Maxillary sinus ,Juvenile nasopharyngeal angiofibroma ,business.industry ,juvenile nasopharyngeal angiofibroma, JNA, extranasopharyngeal angiofibroma, ENPA, endoscopic surgery ,General Medicine ,Sinonasal Tract ,Angiofibroma ,medicine.disease ,Angiofibromas ,Surgery ,JNA ,juvenile nasopharyngeal angiofibroma ,ENPA ,medicine.anatomical_structure ,endoscopic surgery ,otorhinolaryngologic diseases ,medicine ,extranasopharyngeal angiofibroma ,business ,Sinus (anatomy) - Abstract
Juvenile nasopharyngeal angiofibroma (JNA) are rare, histologically benign, but locally aggressive, vascular tumors of the nasopharynx that affects predominantly male adolescents, its origins from other sites is denominated extranasopharyngeal angiofibroma (ENPA), which is an even rarer entity afflicting more females and young adults, representing a diagnostic and therapeutic challenge to rhinologists. In this report we present 2 cases of similar clinical presentation, with unilateral nasal obstruction and previous episodes of nasal bleeding. One patient is a 14-year-old male with a soft-tissue mass occupying nasopharynx, middle meatus, and right nasal cavity. The second patient is a 12-year-old male with a soft-tissue lesion rising from right sphenoid sinus and obliterating right nasal cavity, obstructing ipsilateral maxillary sinus, ethmoidal cells, and frontal sinus. Both patients underwent complete endoscopic excision of the lesions, the first one recurred on sphenoid sinus and the second showed no recurrence after 2 years of post-operative follow-up. The unusual sites and presentations make these rare cases and thus worth of reporting. more...
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- 2020
38. Novel Modifications to Total Maxillary Swing Approach in Extensive Nasopharyngeal Angiofibroma to Minimize Complications
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Manu Malhotra, Amit Kumar Tyagi, Arpana Singh, Saurabh Varshney, Madhu Priya, and Abhishek Bhardwaj
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Infraorbital margin ,medicine.medical_specialty ,business.industry ,Osteomyelitis ,Nasopharyngeal neoplasm ,medicine.disease ,Angiofibromas ,Surgery ,03 medical and health sciences ,0302 clinical medicine ,medicine.anatomical_structure ,Otorhinolaryngology ,Nasopharyngeal angiofibroma ,030220 oncology & carcinogenesis ,medicine ,Malocclusion ,030223 otorhinolaryngology ,Abscess ,business - Abstract
Maxillary swing approaches provide excellent exposure of the anterior, mid, and anterolateral skull base offering a wide window to approach nasopharyngeal neoplasms; however, they are also associated with complications. The present study aimed to evaluate the results of a modified total maxillary swing (TMS) approach developed to minimize postoperative complications. The modified TMS approach was used to treat five patients who had extensive juvenile nasopharyngeal angiofibromas between March and October 2019 at our tertiary care center. Surgical technique, preoperative image findings, and intra-operative findings were recorded. In the postoperative follow-up, patients were examined to rule out all possible complications associated with the procedure according to the literature. A retrospective analysis was performed to assess tumor extensions, surgical modifications, and postoperative complications. All tumors had orbital and infratemporal (lateral limit) involvement while four had intracranial involvement. No per-operative complications were reported, and postoperative clinical and endoscopic evaluation was performed at 1, 2, and 3 months. There was no evidence of complications including residue, recurrence, maxillary necrosis, ophthalmoplegia, epiphora, palatal fistula, or jaw malocclusion. Besides, minor complications such as infraorbital margin skin retraction, infraorbital serous collection, maxillo-zygomatic abscess, and unsightly scar were also not seen. Only one case presented with maxillary osteomyelitis which was resolved with mini-plate removal and antibiotics. A modified TMS approach is a prudent option to ensure complete removal of juvenile nasopharyngeal angiofibromas with negligible complications. more...
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- 2020
39. Cervicovaginal cellular angiofibroma
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Glenn Broeckx, Ellen Faes, Yves Jacquemyn, and Sarah Van Mulders
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Adult ,Pathology ,medicine.medical_specialty ,CD34 ,Uterine Cervical Neoplasms ,Cervix Uteri ,Angiofibroma ,03 medical and health sciences ,0302 clinical medicine ,obstetrics and gynaecology ,medicine ,Humans ,Vulvovaginal Region ,030219 obstetrics & reproductive medicine ,Unusual Presentation of More Common Disease/Injury ,business.industry ,Ultrasound ,Mesenchymal stem cell ,Fornix ,General Medicine ,Angiofibromas ,030220 oncology & carcinogenesis ,Vagina ,Immunohistochemistry ,Female ,pathology ,Human medicine ,Pouch ,business - Abstract
Cellular angiofibroma is a rare type of benign mesenchymal tumour that arises mostly in middle-aged women. It needs to be distinguished from other neoplasms and has a predilection for the vulvovaginal region. To our knowledge, this is the first case of a cervical cellular angiofibroma. A 34-year-old nulligravid woman was referred with a large mass bulging in the fornix posterior. Ultrasound scanning and MRI showed a large solid mass projecting in the pouch of Douglas. Laparoscopic surgical excision was performed. Histopathological examination showed a well-demarcated, unencapsulated tumour, consisting of short fascicles of spindle cells in-between thick-walled medium-sized vessels. On immunohistochemistry, there was strong reactivity with antibodies against CD34 and oestrogen receptor. Angiofibromas are benign mesenchymal tumours mostly occurring in middle-aged women. They can cause abnormal swelling and uterine bleeding and need to be distinguished from other (malignant) neoplasms. more...
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- 2020
40. Management of a Patient with Tuberous Sclerosis with Urological Clinical Manifestations
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Victor Emanuel Stoenescu, Vlad Pădureanu, Octavian Dragoescu, Tomescu P, Radu Cristian Cimpeanu, Dop Dalia, Ionica Pirici, Alexandru Radu Mihailovici, and Rodica Pădureanu
- Subjects
medicine.medical_specialty ,Kidney ,Medicine (General) ,business.industry ,angiomyolipomatosis ,Ultrasound ,Case Report ,General Medicine ,tuberous sclerosis ,uretero-hydronephrosis ,medicine.disease ,Skull ,Left Lumbar Region ,Tuberous sclerosis ,medicine.anatomical_structure ,R5-920 ,medicine ,Dysuria ,Radiology ,medicine.symptom ,Presentation (obstetrics) ,business ,Pelvis ,angiofibromas - Abstract
The tuberous sclerosis complex (TSC) is highly variable as far as its clinical presentation is concerned. For the implementation of appropriate medical surveillance and treatment, an accurate diagnosis is compulsory. TSC may affect the heart, skin, kidneys, central nervous system (epileptic seizures and nodular intracranial tumors—tubers), bones, eyes, lungs, blood vessels and the gastrointestinal tract. The aim of this paper is to report renal manifestations as first clinical signs suggestive of TSC diagnosis. A 20-year-old patient was initially investigated for hematuria, dysuria and colicky pain in the left lumbar region. The ultrasound examination of the kidney showed bilateral hyperechogenic kidney structures and pyelocalyceal dilatation, both suggestive of bilateral obstructive lithiasis, complicated by uretero-hydronephrosis. The computer tomography (CT) scan of the kidney showed irregular kidney margins layout, undifferentiated images between cortical and medullar structures, with non-homogenous round components, suggestive of kidney angiomyolipomas, bilateral renal cortical retention cysts, images of a calculous component in the right middle calyceal branches and a smaller one on the left side. The clinical manifestations and imaging findings (skull and abdominal and pelvis CT scans) sustained the diagnosis. more...
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- 2020
41. Bilateral synchronous juvenile nasopharyngeal angiofibromas: a rare entity
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Thomas Hendriks, Tim Cooney, Ziyad Khaleel, Katherine Pollaers, Latif Kadhim, and Neil Giblett
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medicine.medical_specialty ,business.industry ,Rare entity ,Nasopharyngeal Neoplasms ,General Medicine ,Angiofibroma ,Angiofibromas ,Dermatology ,Text mining ,medicine ,Juvenile ,Humans ,Surgery ,business - Published
- 2020
42. The Efficacy of Everolimus for Facial Angiofibromas in Tuberous Sclerosis Complex Patients Treated for Renal Angiomyolipoma/Subependymal Giant Cell Astrocytoma
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Meng-Hsuan Wu, Shuo-Yan Gau, Chang-Ching Wei, Jeng-Dau Tsai, Chi-Tang Wu, Yue-Ting Wu, and Yu-Ping Hsiao
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Adult ,Male ,medicine.medical_specialty ,Erythema ,Adolescent ,Angiomyolipoma ,Antineoplastic Agents ,Dermatology ,Astrocytoma ,Angiofibroma ,Lesion ,Tuberous sclerosis ,Young Adult ,Tuberous Sclerosis ,medicine ,Humans ,Everolimus ,Child ,Retrospective Studies ,Subependymal giant cell astrocytoma ,business.industry ,medicine.disease ,Angiofibromas ,Kidney Neoplasms ,medicine.anatomical_structure ,Forehead ,Female ,medicine.symptom ,Facial Neoplasms ,business ,medicine.drug - Abstract
Background: Facial angiofibromas may be present since early childhood in individuals with tuberous sclerosis complex (TSC), causing substantial cosmetic disfigurement. Current therapies are partially effective, but they are uncomfortable, produce scarring, and are especially expensive. Objective: The aim of the present study was to evaluate the efficacy of oral everolimus for TSC-associated angiofibromas. Methods: This retrospective study included TSC patients being treated with oral everolimus for subependymal giant cell astrocytomas (SEGAs) and angiomyolipomas (AMLs). We recorded the changes in facial angiofibromas. Changes in the Angiofibroma Grading Scale (AGS) indicators were recorded according to erythema, average lesion size, lesion density, and percent involvement on the forehead, nose, cheeks, and chin. The scores were recorded before and after the administration of oral everolimus. Results: Twenty-one patients being treated with oral everolimus were enrolled in this study. The mean age was 20.5 years (range 11–44 years, 4 males, and 17 females). The mean dose of oral everolimus was 3.6 mg/day. Clinically meaningful and statistically significant improvement was observed in erythema (p = 0.001), average lesion size (p < 0.001), lesion density (p < 0.001), and percent involvement (p < 0.001). Changes in the AGS findings were statistically significant on the forehead (p = 0.001), nose (p < 0.001) cheeks (p < 0.001), and chin (p = 0.004). Conclusion: Everolimus shows evident improvement and is approved for TSC-associated SEGAs and AMLs. The current study demonstrated the efficacy of oral everolimus in reducing facial angiofibromas, showing the parallel benefits of the treatment protocol for TSC. more...
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- 2020
43. Combined endoscopic and transoral resection of a high-staged juvenile nasopharyngeal angiofibroma: A pictorial essay
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Gabriel A Arom, Wilson P Lao, Kristelle J. Lagabon, Paul C. Walker, and Steve C. Lee
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Male ,medicine.medical_specialty ,Adolescent ,Juvenile nasopharyngeal angiofibroma ,medicine.medical_treatment ,Nose ,Angiofibroma ,Resection ,03 medical and health sciences ,0302 clinical medicine ,medicine ,Humans ,030223 otorhinolaryngology ,Craniotomy ,business.industry ,Infratemporal fossa ,Transoral approach ,Endoscopy ,Nasopharyngeal Neoplasms ,Angiofibromas ,Surgery ,Skull ,medicine.anatomical_structure ,Otorhinolaryngology ,Buccal space ,business ,030217 neurology & neurosurgery - Abstract
Juvenile nasopharyngeal angiofibromas (JNAs) are highly vascular and benign tumors that can expand into the skull base. Delay of treatment can result in intracranial invasion, requiring extensive open approaches such as a facial translocation, maxillary swing, or an orbitozygomatic craniotomy. We describe a single-stage, combined endoscopic and transoral approach on a 14-year-old male with extensive high-stage dumbbell-shaped JNA involving the infratemporal fossa, orbit, buccal space, and intracranial extension into Meckel's cave. Successful resection of the tumor and good postoperative outcome was achieved. A transoral approach allowed for greater access to the infratemporal fossa, where endonasal resection was not possible, allowing for improved visualization, greater traction, and dissection. In select highly staged JNAs with significant lateral extension and intracranial involvement, successful and complete resection may be accomplished with this combined approach. Utilization of this approach avoids the morbidity of more invasive open approaches. more...
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- 2020
44. Robotic-assisted transmaxillary approach for removal of juvenile nasopharyngeal angiofibroma of the pterygopalatine and infratemporal fossa
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Habib G. Zalzal and Meghan T. Turner
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medicine.medical_specialty ,military ,Assistant surgeon ,Endoscope ,Robotic assisted ,Juvenile nasopharyngeal angiofibroma ,Pterygopalatine Fossa ,Angiofibroma ,03 medical and health sciences ,0302 clinical medicine ,Robotic Surgical Procedures ,medicine ,Humans ,030223 otorhinolaryngology ,Pterygopalatine fossa ,business.industry ,Infratemporal fossa ,Endoscopy ,Nasopharyngeal Neoplasms ,030206 dentistry ,Angiofibromas ,Surgery ,body regions ,Dissection ,surgical procedures, operative ,medicine.anatomical_structure ,Otorhinolaryngology ,military.rank ,business ,Infratemporal Fossa - Abstract
Endoscopic transmaxillary approaches to remove juvenile nasopharyngeal angiofibromas (JNAs) have been described previously and are used for tumors that extend laterally in the pterygopalatine fossa (PPF) and infratemporal fossa (ITF). There is no previous description of robotic-assisted JNA removal in the literature. The video will demonstrate the success of this technique. Robotic transmaxillary approaches to the lateral PPF and ITF are easily achieved through an extended Caldwell-Luc antrostomy. Total operative time was 519 minutes. Total robotic operative time was 283 minutes. The endoscopic approach provided no assistance in tumor dissection or removal. Robotic-assisted transmaxillary JNA removal allows the operating surgeon to control the endoscope and to operate free of collisions from the robotic console while having an active assistant surgeon at the patient bedside. A video demonstrating the technique is found on Head & Neck's web site. more...
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- 2020
45. Aggressive Juvenile Angiofibromas
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Spencer C. Payne, Jose L. Mattos, and Steven A. Newman
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medicine.medical_specialty ,business.industry ,medicine ,Juvenile ,business ,Angiofibromas ,Dermatology - Published
- 2020
- Full Text
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46. Sirolimus for the treatment of juvenile nasopharyngeal angiofibroma
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Karen S. Fernández, Adrienne M. Hammill, Denise M. Adams, and Alessandro de Alarcon
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Male ,Surgical resection ,medicine.medical_specialty ,Adolescent ,Juvenile nasopharyngeal angiofibroma ,medicine.medical_treatment ,Angiofibroma ,03 medical and health sciences ,0302 clinical medicine ,medicine ,Humans ,Embolization ,Child ,Sirolimus ,Antibiotics, Antineoplastic ,business.industry ,Nasopharyngeal Neoplasms ,Hematology ,Discovery and development of mTOR inhibitors ,Angiofibromas ,Treatment Outcome ,Oncology ,030220 oncology & carcinogenesis ,Pediatrics, Perinatology and Child Health ,Radiology ,business ,Antiangiogenic drug ,030215 immunology ,medicine.drug - Abstract
Juvenile nasopharyngeal angiofibroma (JNA) is a pathologically benign yet locally aggressive and destructive tumor that develops in the choana and nasopharynx. Historical treatment of JNA has included embolization, surgical resection, and radiation. Here, we describe three patients who received therapy with the mTOR inhibitor sirolimus with improvement in clinical symptoms, imaging, and overall well-being. more...
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- 2020
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47. Lady with seizure and skin lesions
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Abheek Sil and Avik Panigrahi
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medicine.medical_specialty ,business.industry ,gingival fibroma ,tuberous sclerosis ,General Medicine ,Images in Medicine ,medicine.disease ,Angiofibromas ,Dermatology ,Tuberous sclerosis ,Epilepsy ,ash-leaf macule ,Ungual fibroma ,Gingival fibroma ,medicine ,epilepsy ,business ,Skin lesion ,ungual fibroma - Published
- 2020
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48. Extranasopharyngeal Angiofibroma: A Diagnostic Dilemma
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Shashikant Anil Pol, Nitin Gupta, Vaibhav Saini, Arjun Dass, and Lovekesh Mittal
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medicine.medical_specialty ,Extranasopharyngeal angiofibroma ,Soft palate ,diagnosis ,diagnostic imaging ,business.industry ,Stridor ,Diagnostic dilemma ,Angiofibroma ,lcsh:Otorhinolaryngology ,medicine.disease ,lcsh:RF1-547 ,Dysphagia ,Angiofibromas ,extranasopharyngeal ,medicine.anatomical_structure ,angiofibroma ,classification ,Nasal septum ,Medicine ,Radiology ,medicine.symptom ,business - Abstract
Objective: To report two cases of extranasopharyngeal angiofibroma, highlighting the diagnostic challenges involved. Methods: Design: Case Report Setting: Tertiary Teaching Hospital and Medical School Patients: Two (2) Results: A 13-year-old boy who presented with epistaxis and a vascular mass on the posterior nasal septum that enhanced on contrast-enhanced computed tomography (CECT), and a 3-year-old boy who presented with dysphagia and mild respiratory difficulty, with a large naso-oropharyngeal mass arising from the soft palate on physical and x-ray examination that could not be corroborated because stridor developed during sedation for CECT, both underwent endoscopic tumor excision. Conclusion: As illustrated in these cases, atypical presentations of extranasopharyngeal angiofibromas can pose a considerable diagnostic and surgical challenge for clinicians. Key words: angiofibroma: classification; diagnosis; diagnostic imaging; extranasopharyngeal more...
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- 2018
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49. Morbidity and Volumetric Progression in Juvenile Nasopharyngeal Angiofibroma in a Long-Term Follow-Up
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Marc Mosimann, Lorenz Epprecht, David Holzmann, Domenic Vital, University of Zurich, and Epprecht, Lorenz
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medicine.medical_specialty ,Long term follow up ,Juvenile nasopharyngeal angiofibroma ,Clinical Neurology ,610 Medicine & health ,10045 Clinic for Otorhinolaryngology ,morbidity ,Asymptomatic ,follow ,endoscopic surgery ,03 medical and health sciences ,0302 clinical medicine ,medicine ,infratemporal approach ,Risk factor ,030223 otorhinolaryngology ,up ,medicine.diagnostic_test ,business.industry ,Magnetic resonance imaging ,Angiofibroma ,medicine.disease ,Angiofibromas ,2728 Neurology (clinical) ,angiofibroma ,030220 oncology & carcinogenesis ,Radiological weapon ,Neurology (clinical) ,Radiology ,medicine.symptom ,business - Abstract
Objective We compare the open and transnasal approaches for the excision of juvenile nasopharyngeal angiofibromas regarding the rate of morbidity, and residual tumor and its symptomatic recurrence over time. In addition, we present volumetric measurements of juvenile nasopharyngeal angiofibromas over time. Methods All surgically treated patients of our institution were reviewed back to 1969 for type of surgery, residual tumor by magnetic resonance imaging (MRI)-based volumetry, recurrence, and morbidity. We performed a prospective clinical and radiological follow-up on reachable patients. Results In total, 40 patients were retrievable from our records. We were able to follow up on 13 patients after a mean of 15.7 years since surgery (range: 1–47 years). Patients operated by the open approach had a higher rate of postoperative complications and thus a higher morbidity than endoscopic patients (4/4 vs 3/9; p = 0.007), although tumor sizes were equal among groups (p = 0.12). Persisting tumor was noted in 3/4 and 4/9 (p = 0.56) patients, respectively. The corresponding mean volumes of residual tumors were 16.2 ± 14.4 cm3 and 10.8 ± 6.6 cm3 (p = 0.27). No progression could be noted in endoscopically treated patients (p = 0.24, mean time between scans 2 years). Conclusions Our analysis shows that the endoscopic approach results in less morbidity. The open approach does not guarantee freedom from persisting tumor tissue. Age seems to be a most important risk factor for the conversion of an asymptomatic persistence into a symptomatic recurrence. more...
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- 2018
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50. Current Management and Controversies of Juvenile Angiofibromas
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Peter Clarke, Ann Sandison, Gitta Madani, Daniel W Scholfield, and Colin Butler
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medicine.medical_specialty ,business.industry ,Building and Construction ,Angiofibromas ,Dermatology ,03 medical and health sciences ,0302 clinical medicine ,Current management ,Medicine ,Juvenile ,Electrical and Electronic Engineering ,030223 otorhinolaryngology ,business ,030217 neurology & neurosurgery - Published
- 2018
- Full Text
- View/download PDF
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