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Preoperative embolisation of juvenile nasopharyngeal angiofibromas (JNAs) is a well-established treatment that reduces intraoperative blood loss and improves surgical outcomes. While the bulk of arterial supply to the tumour is derived from the external carotid system, some degree of contribution from the internal carotid artery (ICA) is common. ICA branch embolisation in this setting has previously been avoided due to concerns over ischaemic neurological complications, possibly contributing to the increased intraoperative blood loss observed in patients with tumours with ICA supply. There is a marked paucity of reports of embolisation of ICA branches supplying JNA in the medical literature. We present a case of successful embolisation of an aberrant pharyngeal branch of the ascending pharyngeal artery arising from the proximal cervical ICA, which was making a significant contribution to tumour blood supply in a male adolescent with a very large JNA.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Angiofibroma is a relatively rare vascular lesion originating predominantly in the nasopharynx and occurs typically in male adolescents. Extranasopharyngeal sites of origin, such as nasal cavity, paranasal sinuses, pharynx, and larynx, are extremely rarely seen in clinical practice. In the nasal cavity, extranasopharyngeal angiofibromas (ENAs) have been reported to originate from the nasal septum, as well as from the middle and inferior turbinate. In this study, we report a case of a 35-year-old female patient with angiofibroma originating from the anterior part of the nasal septum with left-sided nasal obstruction and mild-to-moderate epistaxis as main complaints. After the radiological diagnostics and embolization of sphenopalatine artery, we performed the excision of the lesion with removal of small part of septal mucosa and perichondrium around the attachment of tumor pedicle. Histopathological examination suggested the presence of angiofibroma and diagnosis was confirmed by immunohistochemical analysis. This is the fifth reported case of septal ENA in female population in the English-language literature.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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

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.

Background: The skin is one of the most affected organs in tuberous sclerosis complex and angiofibromas are seen in almost 80% of such patients. These benign tumors impose a great psycho-social burden on patients. Objective: The aim of the study was to evaluate the effectiveness and tolerability of topical sirolimus for facial angiofibromas in patients with tuberous sclerosis complex. Methods: This was a prospective, single-blinded, cross-over study which involved twelve patients. We investigated the effect and safety of topical 0.1 % sirolimus, which was obtained by crushing sirolimus tablets and mixing it with petrolatum. The patients were asked to apply the cream to one side of their face, and vaseline to the other side. The effect of topical sirolimus was evaluated using the "facial angiofibroma severity index." Results: There was a significant improvement in the redness and extension of the tumors on the sides to which the active ingredient was applied. Some side effects such as itching and irritation occurred in three patients, which were treated with topical hydrocortisone cream. Conclusion: Topical sirolimus appears to be a promising, fairly well tolerated treatment for facial angiofibromas in patients with tuberous sclerosis complex. Although its efficacy diminishes with time, repetitive usage is effective.

Introduction: Tuberous sclerosis complex (TSC) is a multisystem neurocutaneous disorder. Angiofibromas (AF), fibrous plaques, and hypopigmented macules are the major skin findings in TSC. Topical sirolimus reduces the volume and redness of AF and other skin findings. However, the efficacy of early intervention and long-term treatment remains to be clarified. We investigated the efficacy of sirolimus gel for AF in children with TSC. Methods: We recruited nine children (five boys; four girls) with TSC and AF. We used 0.2% sirolimus gel over 6 months. We reviewed each patient's medical records and photographs for clinical information and data related to improvements in skin lesions. We evaluated the size of AF, fibrous plaques, and color changes in AF and hypopigmented macules. Results: Age at the initiation of treatment ranged from 3.5 to 11.0 years. The follow-up period ranged from 6 to 36 months (≥24 months in 3 children). Patients presented with papular AF (9), miliary AF (8), AF redness (9), fibrous plaques (5), and hypopigmented macules (2). After 6 months of treatment, improvement of AF size and redness was seen in all nine patients. Patients treated for ≥24 months showed significant decrease in AF size that persisted until the final follow-up. Gradual improvement in fibrous plaques was observed, and marked reduction in size was achieved by 4–18 months. Conclusion: Early sirolimus gel intervention is effective for the treatment of AF and fibrous plaques in children with TSC. Early intervention with sirolimus gel may maintain the skin at near-normal levels in patients with TSC.

Background. Angiofibroma is an uncommon type of vascular benign tumor that is made up of blood vessels and fibrous (connective) tissue. First described in 1997, it usually occurs in middle aged females and is clinically often thought to represent a cyst. Unlike most of the other site specific vulvovaginal mesenchymal lesions, cellular angiofibroma has a marked predilection for the vulva with only occasional examples reported in the vagina. Case Detail. A 17-year adolescent nulligravid girl presented with a history of irregular vaginal bleeding of two-year duration and history of lower abdominal swelling; on examination, she had pale conjunctiva, 20-week sized firm, irregular, nontender abdominopelvic mass, and a firm huge anterior vaginal wall mass, with difficulty to reach at the cervix and hemoglobin of 9.7 gm/dL, and a diagnosis of cervical myoma plus anemia was made, which was supported by imaging studies. Finally it was found to be angiofibroma of the vagina. Conclusion. Angiofibromas are benign tumors, which rarely occur in vagina. Although middle aged females are affected more, angiofibromas can affect females of reproductive age group and can cause abnormal uterine bleeding.

The characteristics of extra-nasopharyngeal angiofibromas tend to be different from angiofibromas of the nasopharynx according to patient gender, patient age, prevalence, affected site, pathogenesis, and clinical and epidemiological features. We report a case of an extra-nasopharyngeal angiofibroma in a 28-year-old man referred to the ENT Clinic for right-sided epistaxis, airflow impairment and nasal swelling. The right nostril was completely occluded works by a reddish-yellow mass that bled easily. The computed tomography scan revealed an “inhomogeneous solid lesion in the nasal fossa”. With the patient under general anesthesia, the formation in the anterior portion of the right side of the nasal septum was removed up to its vascular base. Although electrical cauterization efficiently controlled the bleeding, we abraded the sub-perichondral area to prevent further bleeding as well as recurrence. The histological exam report confirmed the diagnosis of angiofibroma. As in our case, epistaxis is commonly the presenting sign of angiofibroma. Yet its onset was peculiar, given that the bleeding started with a low impact trauma. The nasal swelling was also a relevant feature as well as the breathing impairment. Although uncommon, nasal septal angiofibromas should considered in patients with epistaxis.

Purpose of review To illustrate the latest developments and trends in the management of juvenile angiofibroma. Recent findings Endoscopic surgery is currently the primary management strategy for juvenile angiofibroma. Recent reports on the use of multiportal approaches have contributed to further extend its indications. Studies from different countries suggest that the lesion can display variable growth rates not only in relation to patient age. The same concept applies to residual lesions. For this reason, retreatment of persistent juvenile angiofibromas is indicated when serial imaging clearly shows that the lesion is growing. When redo surgery is potentially associated with high morbidity for the critical relationships of the lesion with adjacent structures, stereotactic or intensity-modulated radiation therapy can be an alternative. Early use of MRI in the postoperative course is a highly effective way to detect residual lesions. Summary Contemporary management of juvenile angiofibroma should primarily rely on endoscopic surgery to obtain radical tumor resection. Recent evidence on the behavior of residual postoperative juvenile angiofibroma and the development of conformal RT techniques have helped to clarify the role of watchful waiting and radiotherapy (RT) as alternatives to aggressive procedures in cases with critical extension of the lesion. Although radical excision is the primary therapeutic objective, the benign nature of juvenile angiofibroma and the reported tendency of small residual lesions to remain stable or involute, especially in postpubertal patients, should always be kept in mind to avoid unnecessary morbidity. Video abstract In the video, two of the authors describe the content of the review and present the main topics discussed in the article. http://links.lww.com/COOH/A37.

Cellular angiofibroma is a recently described rare benign soft-tissue tumor more commonly presenting in middle-aged women, often mimicking malignancy. The vulva is most common location. Complete local excision is the best curative treatment and usually there is no recurrence after surgery. We describe a 49-year-old woman with a painless tumor in the left ischiorectal fossa. It was a random finding in a routine computed tomography (CT) scan after resection of ear melanoma 3 years previously. Ultrasonography showed a solid mass, and further magnetic resonance imaging (MRI) suggested a rhabdomyosarcoma. Altogether, these findings indicated malignant disease. An uncomplicated simple excision of the tumor was done in the operating theatre. The mass measured 7×5×5 cm and the histopathological examination found that it was a cellular angiofibroma, a benign lesion. There were no postoperative complications. This case report highlights the need for multidisciplinary team management of rare tumors such as cellular angiofibromas., Highlights • Soft tissue angiofibromas are benign, fibrovascular tumors with average size

In just the past 5 years, dramatic changes have occurred in the clinical management of tuberous sclerosis complex (TSC). Detailed knowledge about the role of the TSC proteins in regulating the activity of the mammalian target of rapamycin complex 1 (mTORC1) underlies this paradigm-shifting progress. Advances continue to be made in understanding the genetic pathogenesis of the different tumours that occur in TSC, including pivotal discoveries using next-generation sequencing (NGS). For example, the pathogenesis of angiofibromas is now known to involve UV-induced mutations, and the pathogenesis of multifocal renal cell carcinoma (RCC) in TSC is now known to result from distinct second-hit mutations. In parallel, the pathological features of TSC-associated tumours, including TSC-associated renal cell carcinoma, continue to be defined, despite the fact that TSC was first described 180 years ago. Here, we review recent discoveries related to the pathological features and genetic pathogenesis of TSC-associated tumours. Copyright © 2016 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

Nevertheless, our ability to identify meaningful differences between these two populations suggests that this is a reasonable cutpoint. One hypothesis for the increasing incidence rates of early-onset BCC has been increased awareness and skin surveillance. However, lesion size has not decreased over time, as might be anticipated if earlier detection were the underlying cause of increased incidence (Christenson et al., 2005). Our results likewise suggest that early-onset BCC is associated with aggressive histologic characteristics, as opposed to a less aggressive phenotype that might be expected if surveillance bias were operating. Although additional studies are needed, these results suggest there may be underlying biological differences between earlyand late-onset BCC.

Somatostatin cell surface receptors are expressed on the peritumoral vessels of various benign and malignant neoplasms including angiofibromas. Positive initial uptake on positron emission tomography-computed tomography (PET/CT) imaging with 68Ga-labeled DOTANOC is noted in a patient with juvenile nasal angiofibroma (JNA), and the same is noted to be absent following complete surgical excision. Functional 68Ga-DOTANOC PET/CT may be an early useful tool for JNA residual/recurrence identification and precise postoperative surveillance.

Objectives In recent years, various formulations containing rapamycin, mainly petrolatum-based, have been tested on facial angiofibromas in tuberous sclerosis. They are often poorly tolerated due to irritation and bleeding. In addition, their effectiveness was insufficient in young adults. The objective of this study was to develop and characterise a hydro-alcoholic gel containing solubilised rapamycin. The stability of the product stored at 4°C was evaluated over 1 year. Methods Two different 0.1% rapamycin gels were formulated with or without α-tocopherol and urea. Different methods were used to characterise the gels: HPLC, gas chromatography, pH, visual observation and optical microscopy. A physico-chemical and microbiological stability study was also conducted for 1 year at 4°C. Results Gels were physically and microbiologically stable after 1 year at 4°C: organoleptic characteristics and pH unchanged, no significant decrease in rapamycin was observed, tocopherol droplet size was constant and rheological behaviour was not altered. Conclusions This study describes a new gel formulation to improve skin penetration using various excipients to promote skin tolerance. This study provides, for the first time, detailed stability data for a hydro-alcoholic rapamycin gel.

Juvenile nasopharyngeal angiofibroma is a vascular tumor that typically occurs in men. Vestibular schwannoma (acoustic neuroma) is a tumor affecting the vestibulocochlear nerve. A 38-year-old man with various pigmented lesions and history of juvenile nasopharyngeal angiofibroma, vestibular schwannoma, and cleft lip and cleft palate is described. Characteristics of patients with coexisting juvenile nasopharyngeal angiofibroma and vestibular schwannoma are summarized. A search, using PubMed, was performed for the following terms: acoustic, angiofibroma, blue, cleft, combined, dysplastic, juvenile, lip, nasopharyngeal, neuroma, nevus, palate, schwannoma, and vestibular. The relevant papers were obtained and their references were reviewed. Only one man with coincident juvenile nasopharyngeal angiofibroma and vestibular schwannoma has previously been described. The juvenile nasopharyngeal angiofibromas were both right-sided and diagnosed when the patients were 13 years old and 20 years old. Our patient’s vestibular schwannoma was ipsilateral with his juvenile nasopharyngeal angiofibroma and incidentally diagnosed on a magnetic resonance imaging (MRI) scan that was performed to monitor for recurrent juvenile nasopharyngeal angiofibroma when he was in his 30s. The other man’s vestibular schwannoma and juvenile nasopharyngeal angiofibroma were diagnosed concurrently, when he was 20 years old. The observation of a patient with cleft lip and cleft palate, various melanocytic nevi, juvenile nasopharyngeal angiofibroma, and vestibular schwannoma is unique. Although the appearance of juvenile nasopharyngeal angiofibroma and vestibular schwannoma may be coincidental, the occurrence of these tumors in the same individual may suggest an association with regards to their pathogenesis.

Introduction: Angiofibroma is a benign, un-encapsulated, and highly vascular tumor which typically arises from the pterygopalatine fossa in the young adolescent males. It predominantly extends into the nasopharynx and rarely develops extranasopharyngeally. However, the most common origins of extranasopharyngeal angiofibromas are the maxillary sinus, ethmoid sinus and nasal cavity. Case Presentation: Here, we report a 40-year-old male who presented with headache and epistaxis and was diagnosed as an extranasopharyngeal angiofibroma originated from the sphenoid sinus. The tumor was completely resected endoscopically. The 24-month follow-up showed no recurrence. Conclusions: Angiofibromas should be considered as a possible diagnosis in any unilateral vascular mass of the nasal cavity, even in the older patients without typical imaging patterns.

Angiofibromas are the most recognized cutaneous manifestations of tuberous sclerosis complex. Angiofibromas can be associated with disfigurement, bleeding, pruritus, and erythema and may lead to significant psychosocial consequences for affected patients. Histopathologically, angiofibromas consist of a mixture of vascular and interstitial cells. Traditional treatment options include cryotherapy, electrocoagulation, radiofrequency ablation, dermabrasion, laser treatment, and topical agents such as podophyllotoxin. However, complications such as pain, postinflammatory hyperpigmentation, scarring, and the frequent recurrence rate reduce the utility of these treatment options. The introduction of topical rapamycin marked a turning point for treatment of facial angiofibromas; however, the lack of a standardized formulation, limited insurance coverage, and significant financial cost restrict universal access for patients and their caregivers. Both oral and topical β-blockers have proven extremely effective treatments for superficial vascular tumors such as hemangiomas and pyogenic granulomas. Topical β-blockers may potentially be useful for treatment of angiofibromas considering these lesions also contain a vascular component. Here we present an exploratory case report of a patient with tuberous sclerosis complex who had significant clinical improvement of her facial angiofibromas utilizing a “split-face” comparison protocol of topical timolol 0.5% gel after full-field treatment with ablative fractional laser resurfacing and pulsed-dye laser.

Mesenchymal-endothelial transition is proposed for juvenile angiofibromas (JAs). Propranolol might be an interesting new medical option in JA treatment, as it reduces mesenchymal cell growth and decreases the number of CD31-positive cells in vitro.Juvenile angiofibromas (JAs) are rare fibro-vascular tumors affecting almost exclusively adolescent males. Based on morphological aspects of irregularly configured vascular spaces and clinical features, JAs have been proposed to represent a vascular malformation. In general, mesenchymal-endothelial transition has been noted as an important process in tumorigenesis as well as in embryonal development.The study analyzed effects of vascular endothelial growth factor (VEGF)/basic fibroblast growth factor (bFGF) and propranolol on endothelial differentiation (CD31+) of cultured JA cells and their expression of angiogenic growth factors using aortic ring assay and enzyme-linked immunosorbent assay.Following VEGF/bFGF supplement to cultured mesenchymal cells, an average of 4.47% (± 2.35%) CD31-positive cells were found (p0.001). Propranolol addition reduced the number of CD31-positive cells and inhibited mesenchymal cell growth. The aortic ring assay and ELISA investigation indicated no increased angiogenic growth factor secretion from cultured JA mesenchymal cells.