Search

Fibromatosis is a benign lesion of the breast that can rarely occur in men, and requires good imaging and biopsy to make a differential diagnosis to breast carcinoma. Primary breast fibromatosis predominately affects females but can rarely affect the male breast (1,2). Due toits rarity, the condition has often been confused with breast carcinoma. Significant variability has been reported regarding imaging characteristics of fibromatosis using ultrasound and MRI. MRI is particularly useful in the evaluation of tumor extent and preoperative planning (3). We report the case of a 66-year old male who presented with a palpable mass within the external quadrant of his right breast. Ultrasound-guided core needle biopsy revealed diagnosis of fibromatosis. Follow-up performed 11 months after resection demonstrated no radiographic evidence of recurrence., (Celsius.)

Three-dimensional (3D) methods for visualizing volumetric computed tomography (CT) data can aid in understanding the extent of a disease process and planning necessary surgical interventions. Recently, a new method of 3D image creation, known as cinematic rendering (CR), has been developed; it produces photorealistic images from standard CT acquisitions. We describe the CT appearance of a rare tumor type, odontogenic myxofibroma, in the mandible of a 33-year-old woman, and we focus on the potential applications of CR visualizations in this and similar scenarios. Although prospective data quantifying the advantages of CR relative to traditional methods of 3D visualization are still forthcoming, the photorealistic quality of CR images, nonetheless, suggests the important potential utility of this method. Realistic shadowing effects in the images create depth and show the relative positions of objects within a visualized volume in an advantageous manner. Furthermore, soft tissue details allow for visualization of structures that can otherwise be difficult to render with traditional methods., (Copyright © 2018 Elsevier Inc. All rights reserved.)

A 72-year-old man without obvious risk factors initially presented with acute ischemic stroke and fever, without concomitant infection. Broad spectrum antibiotic therapy was initiated. Transthoracic and Transesophageal echocardiography, and cardiac MRI revealed a 20 mm round mass attached to the anterior mitral valve leaflet, suggesting the diagnosis of a benign cardiac tumor or a vegetation. At the site of infarction an abscess of 11 mm in diameter developed 30 days later. The patient underwent surgical valve repair for the prevention of further embolic complications. Histology revealed a cardiac papillary fibroelastoma (PFE). He made complete clinical recovery. Secondary abscess formation after ischemic stroke is rare: 11 other cases have been reported. Because they develop at the site of a previous ischemic infarct, these abscesses usually do not manifest by additional neurologic deficits, making difficult their diagnosis. In most cases a concomitant infection cannot be individualized. Even if these abscesses are a rare entity, patients with cerebral infarct presenting with fever must be closely follow-up with cerebral imaging. Even if PFE is a rare cardiac source of embolic stroke, it should be considered in the differential diagnosis of stroke in a patient where an alternative etiology has not been established., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Introduction: To determine the diagnostic yield of repeat ultrasound (US)-guided biopsy of musculoskeletal soft-tissue lesions with initially inconclusive biopsy results, and to explore predictive factors for success of repeat biopsy., Materials and Methods: This retrospective study included 42 patients who underwent a repeat (second) US-guided biopsy session to target a musculoskeletal soft-tissue lesion because an initial US-guided biopsy session provided inconclusive results. Both biopsy sessions were performed in a tertiary referral center for soft-tissue sarcomas., Results: The diagnostic yield of repeat US-guided biopsy was 47.6%. Malignant nature of the lesion (P = 0.031), sharp lesion borders on US (P = 0.011), and good to very good lesion visibility on US (P = 0.017) were significantly associated with a diagnostic repeat US-guided biopsy. There was also a trend towards significance (P = 0.073) for a higher number of biopsy passes through the lesion. Other patient characteristics (age and gender), magnetic resonance imaging features (lesion homogeneity on T1-weighted, T2-weighted, and gadolinium chelate enhanced sequences, borders, enhancement pattern, depth and size), US features (lesion appearance, vascular flow, and depth), biopsy-related factors (days between initial and repeat US-guided biopsy, needle diameter, maximum length of acquired samples), and operator-related factors (same or different radiologists/pathologists for initial and repeat biopsies), were not associated with the diagnostic success of the repeat US-guided biopsy., Conclusions: Repeat US-guided biopsy of a musculoskeletal soft-tissue lesion with initially inconclusive biopsy results can be useful to establish a final diagnosis. Lesion features on US (borders and visibility) may be used to prospectively determine the utility of a repeat US-guided biopsy., (Copyright © 2019 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)

The aortic valve (AV) is the most commonly affected site in multiple papillary fibroelastomas, but the frequency of embolism caused by the attachment side of the AV has not been elucidated. According to the review of the previous literature, 16 cases have been found attached to the AV. Of these, 6 of these have been found to be attached on the aortic side and 4 on the left ventricular side, 1 was bilateral, and 5 cases were unknown. Of the cases found on the aortic side, embolism occurred in 3 of them, and of the left ventricular side cases, embolism occurred in 2 of them. The frequency of embolism is equivalent even if papillary fibroelastoma attached to either side of the AV., (© 2019 Wiley Periodicals, Inc.)

Chondromyxoid fibroma (CMF) is a relatively uncommon benign bone tumor of cartilaginous differentiation. The primary pitfall in the histopathologic diagnosis of CMF is confusion with a high-grade chondrosarcoma, owing to the atypical stellate and spindled cells set within a chondromyxoid background. CMF is particularly challenging to diagnose within the pelvis, where clinical suspicion for chondrosarcoma is high and benign lesions may grow to a large size to occupy the pelvic bones. In our practice, we noted this difficulty in several consecutive cases, especially when older patients presented with CMF within the pelvis. This prompted an institutional retrospective case review of all CMF within the pelvis. In 10 cases, we found overall that CMF of pelvis occurred in an older age range (mean age = 48.6 years), was larger in size (mean size = 6.0 cm), and showed a higher rate of soft tissue extension (50%) as compared with prior reports of nonpelvic CMF. Typical histologic features of CMF were seen in all cases; however, a high frequency of dystrophic calcification (50%) and necrosis (30%) was observed. Of interest, these aggregate demographic, radiologic, and histologic findings are all consistent with a benign neoplasm that has grown undetected within the pelvis over a long period of time. Recognition of these differences between pelvic CMF and tumors involving other sites will aid in avoiding misdiagnosis of this uncommon entity.

Objective: The aim of this study was to determine the prevalence, demographic data of elastofibroma dorsi (ED) in adult population who had undergone chest CT examination and to discuss clinical, and radiological presentations, and treatment options of ED., Methods: We retrospectively reviewed 4074 chest CT examinations for ED from July 2014 to April 2015. Lesion size, side, and patient demographics were analyzed for positive cases of ED. The initial radiology reports of patients with ED were also reviewed., Results: Of the 4074 patients, 111 patients (2.73%) (77 women and 34 men; mean age: 68.2 years; range: 35-91 years) had a total of 168 ED. The females had a 1.96 -fold higher prevalence of ED than the males (OR, 1.96; 95% CI, 1.481-2.59). The mean lesion thickness was found to be significantly greater in the female patients compared with the male patients (p = 0.001). The prevalence of the disease was estimated to be 4.98 times higher in patients aged 65 years or older (CI 95%, 3.25-7.36). In 111 ED patients, the lesions were only noted in 9 patients' initial radiology report., Conclusion: Here, we present a prevalence study with the largest population in the literature concerning ED. Our study shows that ED is not as uncommon as previously thought and should be especially suspected in females and older age groups., Level of Evidence: Level IV, Diagnostic Study., (Copyright © 2019 Turkish Association of Orthopaedics and Traumatology. Production and hosting by Elsevier B.V. All rights reserved.)

Subtotal tumour resection is used to treat infants with congenital cardiac fibroma and medication-resistant ventricular arrhythmias; however, complete elimination of arrhythmogenic substrates has been unclear. A 4-month-old male infant with congenital cardiac fibroma and ventricular fibrillation underwent subtotal tumour resection and implantable cardioverter-defibrillator implantation. Five years later, angiography revealed impending compression of the left coronary artery. Elimination of the arrhythmogenic substrate was confirmed and the device was removed successfully.

Cardiac papillary fibroelastoma is a rare but increasingly recognized cause of embolic stroke that is prevalent in the older population and requires prompt surgical management. We report an unusual case of left atrial appendage cardiac fibroelastoma in a 76-year-old gentleman who presented with left internuclear ophthalmoplegia and ataxia, with corresponding diffusion-weighted imaging on magnetic resonance imaging of the brain. This case illustrates the importance of echocardiographic imaging in the workup of cardioembolic stroke in the older adult population in the acute setting., (Crown Copyright © 2019. Published by Elsevier Inc. All rights reserved.)

Chondromyxoid fibroma (CMF) is a rare benign bone neoplasm that typically occurs in young adults. Juxtacortical or surface-type CMF are rarer still and we present the case of a surface-type CMF in a 78-year-old woman, with only one other case described in a patient of a similar age previously. This patient was an otherwise healthy woman who presented for evaluation of a palpable lump in the anterior proximal tibia. Initial radiographs obtained demonstrated a focal soft tissue fullness immediately anterior to the anterior cortex of the proximal tibia, which contained faint chondroid-like matrix internally. There was associated scalloping of the anterior tibial cortex. MRI confirmed the presence of a juxtacortical, enhancing lesion. Subsequent excisional biopsy was performed and histopathology demonstrated features, which was consistent with surface-type CMF. At a 6-month follow-up the patient remained free of recurrence. In a patient of this age, paraosteal chondrosarcoma should be excluded. Surface-type CMF, although rare, has been described in older patients and while it is unlikely to feature in a list of differential considerations on initial imaging, awareness of the entity is important.

Stroke occurring during pregnancy is linked to high mortality. Stroke may be directly related to pregnancy (e.g. eclampsia, cerebral venous thrombosis), but all the other causes of stroke are possible. Brain magnetic resonance imaging that is not contraindicated during pregnancy remains the reference in this context. We report the case of a woman with severe headache associated with visual disturbances, without hypertension or proteinuria at 32 weeks of pregnancy. MRI revealed multiple recent cerebral vascular accidents. An echocardiogram detected a papillary fibroelastoma of 5mm. Maternal fetal experts determined it safe to continue the pregnancy. Childbirth at 39 weeks was normal uneventful for the newborn and mother. In the postpartum, despite the theoretical operative indication to resect the small residual papillary fibroelastoma, the patient was followed expectantly because of the stable neuro-cardiologic state. A fibroelastoma can be revealed by a stroke during pregnancy. In the postpartum period, an expectant attitude can be reasonably chosen in case of small size of the fibroelastoma., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Rationale: Fibroma of tendon sheath is a rare entity that is typically attached to the tendon sheath., Patient Concerns: A 43-year-old man presented with a painful mass in his right wrist, which was initially misdiagnosed as an enchondroma., Diagnosis: Fibroma embedded into carpal bones, which exhibited lytic radiographic features similar to those of enchondroma. Excisional biopsy demonstrated spindle-shaped cells and collagen-like stroma., Interventions: The patient underwent lesion resection surgery., Outcomes: The patient recovered well and showed no signs of recurrence at 6-month follow-up., Lessons: This case provides valuable insights for hand surgeons. While radiograph is helpful in multiple diseases, histological examination is indispensable for establishment of final diagnosis.

Cardiac fibromas are the second most common primary cardiac tumors in childhood. They only rarely present in adults. They are mainly located in the free wall of the ventricles and attached to the interventricular septum. Despite their benign pathology, fibromas in some locations can cause significant complications. This video tutorial illustrates the resection of a septo-apical cardiac fibroma in an asymptomatic 26-year-old woman, diagnosed with transthoracic echocardiography and cardiac magnetic resonance imaging after an abnormal ECG on an occupational medical examination. Surgery was performed through a median sternotomy using cardiopulmonary bypass. A nodular intramyocardial mass was found at the left ventricular apex and resection was carried out. After removing the fibroma, a defect was found at the apex affecting both ventricles. The defects were closed using a direct suture of 4-0 polypropylene "U" stitches reinforced with a Teflon patch to exclude the ventricular cavity.  Pathology confirmed the diagnosis of benign cardiac fibroma with tumor-free surgical margins. The patient recovered uneventfully and was discharged on postoperative day 5. The outpatient follow-up cMRI 10 months after surgery showed no signs of tumor recurrence and normal biventricular function., (© The Author 2016. Published by MMCTS on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background and Importance: Liposclerosing myxofibrous tumors (LSMFTs) are rare benign fibro-osseous tumors most frequently occurring in the proximal femur. We report the first case of this rare tumor occurring within the calvarium., Clinical Presentation: Our patient presented with a 2-yr history of enlarging, painless, fixed mass over the left forehead. She underwent surgical resection and the mass was histologically confirmed to be a LSMFT., Conclusion: LSMFT is a rare tumor that should remain on the differential for lesions of the calvarium. When diagnosed, this lesion can be removed with the goal of gross total resection and excellent cosmesis can be achieved., (Copyright © 2018 by the Congress of Neurological Surgeons.)

Competing Interests: All authors have disclosed no conflicts of interest.

Embolic stroke secondary to cardiac tumors is uncommon. However, 25-30% of cardiac tumors may cause systemic emboli. We report a 29-year-old male consulting for a sudden episode of aphasia and right hemiparesis, compatible with infarct of the left middle cerebral artery territory. Transthoracic echocardiography reported an ovoid tumor of 8 × 7 × 7 mm in relation to the sub valvular apparatus of the mitral valve. After neurologic stabilization, surgical treatment was performed. Approached by median sternotomy and in cardiopulmonary bypass, the mitral valve was explored. A macroscopic tumor consistent with a papillary fibroelastoma curled in sub valvular chordae was found. It was deployed and resected from its base, while the anterior mitral leaflet was preserved intact. Histopathological examination confirmed the intraoperative macroscopic diagnosis. The patient recovered uneventfully postoperatively and was discharged on the fifth day after surgery. He currently is in functional capacity I without cardiovascular symptoms at five years follow-up.

Acquired digital fibrokeratoma (ADFK) is a rare, benign, fibrous tumor that most often occurs on fingers. It may resemble a rudimentary supernumerary digit and is often misdiagnosed as another common benign condition, such as common wart. It is typically asymptomatic and occurs as a solitary nodule less than 1 cm in diameter. Oftentimes ADFK shares clinical and/or histologic resemblance with other benign and malignant cutaneous conditions so it is crucial that careful examination is undertaken. In this article, we will discuss the clinical presentation, epidemiology, etiology, dermoscopic and histologic findings, management, and differential diagnoses for ADFK, with the hope that this review will facilitate timely diagnosis and management for this distinct condition., (© 2018 The International Society of Dermatology.)

Subtotal tumour resection is used to treat infants with congenital cardiac fibroma and medication-resistant ventricular arrhythmias; however, complete elimination of arrhythmogenic substrates has been unclear. A 4-month-old male infant with congenital cardiac fibroma and ventricular fibrillation underwent subtotal tumour resection and implantable cardioverter-defibrillator implantation. Five years later, angiography revealed impending compression of the left coronary artery. Elimination of the arrhythmogenic substrate was confirmed and the device was removed successfully.

Objective: To explore the clinical features and surgical treatment experience of primary cardiac valve tumor., Methods: The present study retrospectively analyzed the clinical data of 11 patients with primary valvular tumors who underwent surgical treatment in our department from 1980 to 2016. Echocardiography of preoperative patients was indicated as a heart valve tumor. All patients underwent cardiopulmonary bypass surgery after endocardial angiography and positron emission tomography-computed tomography diagnosis, including four tumor resections-valvuloplasty and seven tumor resections-heart valve replacement. Pathological analysis was performed on all tumors. Postoperative cardiac ultrasound was followed up. Pathological analysis was performed on all tumors. All patients underwent postoperative ultrasound follow-up examination., Results: Primary valvular tumors are rare, accounting for only 0.034% (11/32.728) of extracorporeal circulation surgery in our center. It accounts for 2.8% (11/399) of primary cardiac tumors in the same period. Pathological study indicated that there were 10 cases of benign tumor and 1 case of low-grade sarcoma. After 0.6-16 years of follow-up, the operation effect was satisfactory., Conclusion: Most of these tumors are papillary fibroelastoma located on the mitral valve, and surgical operation is the best strategy for cardiac primary valve tumors.

Fibromyxomas are rare tumors that are not commonly seen in the orbit. The authors present a case of orbital fibromyxoma. A 42-year-old male presented with proptosis of the right eye that began 1 month prior. Magnetic resonance imaging revealed the presence of an orbital mass in the right eye, with low signal intensity on T1-weighted images and heterogeneous high-signal intensity on T2-weighted images. The patient underwent excisional biopsy of the orbital tumor through a Krönlein approach. The tumor originated from the inferior rectus muscle. Histopathologic analysis demonstrated scattered spindle cells, with both fibrous and myxoid stroma. Immunohistochemical staining was positive for Vimentin and negative for S-100 protein and CD34. The tumor was diagnosed as orbital fibromyxoma. The patient showed no evidence of recurrence over 18 months of follow-up after operation.

Background: Tricho-rhino-phalangeal syndrome (TRPS) is a rare autosomal dominant genetic disorder characterized by distinctive craniofacial and skeletal abnormalities, while non-ossifying fibroma (NOF) is a common benign bone tumour in children and adolescents. To date, no case of TRPS coexisting with NOF has been reported. This report presents a 12-year-old girl who had the characteristic features of tricho-rhino-phalangeal syndrome and non-ossifying fibroma with a fibula fracture., Case Presentation: A 12-year-old girl was admitted to the Department of Endocrinology and Diabetes for evaluation of brachydactyly and a right fibula fracture. Clinical examination revealed sparse scalp hair, a characteristic bulbous pear-shaped nose, and brachydactyly with significant shortening of the fourth metatarsal. Neither intellectual disability nor multiple exostoses were observed. Radiography of both hands showed brachydactyly and cone-shaped epiphyses of the middle phalanges of the digits of both hands with deviation of the phalangeal axis. Genetic analysis of TRPS1 identified a heterozygous germline sequence variant (p.Ala932Thr) in exon 6 in the girl and her father. Approximately 1 month before being admitted to our department, the girl experienced a minor fall and suffered a fracture of the proximal fibula in the right lower limb. The pathological cytological diagnosis of the osteolytic lesion was NOF. Ten months following the surgery, the lesion on the proximal fibula of the girl disappeared., Conclusions: In conclusion, the present study is the first to report a rare case of NOF with a pathologic fracture in the fibula of a girl with TRPS. The identification of a missense mutation, (p.Ala932Thr), in exon 6 of TRPS1 in this kindred further suggested that the patient had type I TRPS and indicated that mutations in this exon may be correlated with more pronounced features of the syndrome. Radiological techniques and genetic analysis played key roles in the definitive diagnosis.

A 37-year-old man presented with acute chest pain, an unremarkable ECG and mildly elevated cardiac troponin. Coronary CT angiography showed a calcium score of 0 together with the absence of atherosclerotic plaques and normal origin and course of the coronary arteries. Transthoracic and transoesophageal echocardiography revealed an infracentimetric round-shaped mobile mass attached to a secondary tendinous chord of the anterior mitral valve leaflet. Cardiac magnetic resonance further evidenced localised contrast uptake supporting vascular irrigation, making thrombus unlikely. After surgical excision, the patient had an uneventful postoperative course. Histopathology disclosed the typical collagenous matrix covered by a single cell layer. Although mostly benign, cardiac tumours are prone to embolisation and can thus mimic an acute coronary syndrome. Multimodality imaging has an important role in unmasking the true mechanism, revealing less common aetiologies and elucidating the possibility of curative surgical resection., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2018. No commercial re-use. See rights and permissions. Published by BMJ.)

Aim: Ameloblastic fibroma (AF) is a rare benign odontogenic tumor of slow-growing behaviour characterised by proliferation of both epithelial and mesenchymal odontogenic tissues. Treatment of AF is usually conservative by means of enucleation, curettage and long term follow-up., Case Report: This report describes a case of AF in a 12-year-old boy. Examination of the oral cavity revealed absence of tooth 3.7 and mild swelling in the same mandibular area. Orthopantomography and Cone Beam Computed Tomography (CBCT) showed a wide multilocular radiolucent lesion at the left mandibular angle, extending from the first molar to the lower part of the mandibular branch; agenesis of tooth 3.7 and displacement of tooth 3.8. Surgical excision was performed under general anaesthesia. A fiberscope was used to perform a more conservative bone removal and extraction of tooth 3.8. Furthermore, an accurate curettage of the bone site was performed. The histological investigation showed an AF pattern: an epithelial cell component arranged in nests and tubules immersed in a stroma of low differentiated mesenchymal tissue. No sign of recurrence has been observed during the 6-month follow-up period., Conclusion: Although AF is a rare tumor, it is more prevalent in children's jaw. A new intra-operative visual technique could provide a conservative treatment by minimising bone deformities and permitting an accurate bone curettage.

Background/objectives: Little is known about the dermoscopic features of atypical fibroxanthoma., Methods: This was a case-control study. Atypical fibroxanthoma lesions were compared with a control group with non-melanoma skin cancer., Results: Altogether 40 atypical fibroxanthoma were collected. Most developed in men (93%), appearing mainly as nodular (63%), amelanotic (93%) and ulcerated (78%) lesions. Most lesions were located on the scalp (55%) and the ears (13%). Dermoscopically, most atypical fibroxanthoma displayed red (83%) and white (70%) structureless areas and irregular linear vessels (43%). A series of features achieved statistical significance when comparing atypical fibroxanthoma with non-melanoma skin cancer. The presence of red and white structureless areas and white lines, and the absence of yellowish-white opaque scales, hairpin vessels and arborising vessels were predictive of atypical fibroxanthoma in univariate analysis. However, when squamous cell carcinoma was excluded from the analysis, none of the criteria achieved statistical significance. When basal cell carcinoma was excluded, three variables achieved statistical significance in predicting atypical fibroxanthoma: red, structureless areas, the absence of opaque yellowish-white scales and absence of white circles., Conclusions: Atypical fibroxanthomas seem to be barely distinguishable from basal cell carcinoma dermoscopically, but they are more easily distinguishable from a well to moderately differentiated squamous cell carcinoma. A histopathological examination is needed for the final diagnosis., (© 2018 The Australasian College of Dermatologists.)

Fibromas are benign, well-circumscribed tumors that are characterized as spindle-cell lesions with interlacing fibrous stroma. Here, we describe the clinical presentation, management, and outcome of a patient with an orbital fibroma. To our knowledge, this is the second case report of a biopsy-proven subperiosteal orbital fibroma to date.

Elastofibroma dorsi is a reactive pseudotumor of connective tissue, typically located in the infrascapular region. Awareness of this benign entity is crucial for radiologists, as well as clinicians, to avoid misdiagnosis and potential patient harm. In this report, we present clinical and imaging findings of an elastofibroma dorsi after a thoracotomy procedure., (Copyright © 2017 National Medical Association. Published by Elsevier Inc. All rights reserved.)

Gorlin-Goltz Syndrome also known as Nevoid Basal Cell Carcinoma Syndrome is an autosomal dominant multisystem disorder. It is characterized by basal cell carcinomas, odontogenic keratocysts, skeletal abnormalities and in a minority of female patients bilateral calcified ovarian fibromas. It is challenging to radiologically assess ovarian fibromas as they have similar imaging patterns to some malignant ovarian lesions. However, it is vitally important to differentiate between benign and malignant lesions to determine patients' suitability for fertility-sparing surgery. This report describes a case of a 25 year-old patient with Gorlin-Goltz Syndrome and bilateral ovarian fibromas.

We present the first case of upper airway obstruction secondary to a retropharyngeal Gardner-associated fibroma (GAF). A 16-month-old infant presented with a 3-month history of worsening dyspnoea and apnoeic episodes. Examination revealed stridor and left-sided retropharyngeal asymmetry. MRI demonstrated a mass in the retropharynx. Tracheostomy and pharyngeal biopsy under anaesthesia were performed, and histology confirmed a diagnosis of GAF. The mass was excised using a transcervical approach, and postoperative recovery was unremarkable. GAF is associated with Gardner's syndrome (GS) and familial adenomatous polyposis (FAP), both of which are associated with multiple colonic polyps and increased risk of colorectal malignancy. Subsequent testing for an APC mutation seen in GS and FAP was negative in our patient. The details of this unusual presentation of a rare disease are given in addition to a review of the literature., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2018. No commercial re-use. See rights and permissions. Published by BMJ.)

We present a case of 20-year-old woman who presented with a large pedunculated skin covered mass lesion arising from the left thigh, measuring 40 × 25 cm, with no history of pain or skin ulceration and a feeling of a lump with dragging pain in the left side of the abdomen for about 7 years. Subsequently, ultrasound, contrast-enhanced computed tomography, and magnetic resonance imaging of abdomen and left thigh region were carried out. The lesion was broad-based toward the left upper thigh with a central core of interspersed fat supplied by branches of the superficial and deep femoral arteries. Another lesion was seen in the left retroperitoneum anterior to the psoas muscle in a left paravertebral location encasing the left common iliac vessels extending into the left pelvic cavity and inguinal region inferiorly. The lesion showed dense post-acoustic shadowing on ultrasound, mild enhancement on contrast-enhanced computed tomography, and appeared hypointense on T1- and T2-weighted images. A left thigh lesion was excised, whereas incisional biopsy was done for the left retroperitoneal lesion. The diagnosis of a giant fibroepithelial polyp arising from the left thigh and left retroperitoneal fibromatosis was made. This is the first report of such a giant fibroepithelial polyp arising from the thigh with associated retroperitoneal fibromatosis.

Background: Chondromyxoid fibromas (CMFs) are benign tumors that occur rarely in the skull base., Objective: To conduct a preliminary exploration of the diagnosis and treatment of cranial CMFs., Methods: A retrospective analysis of 19 cases of CMFs in the base of the skull between 2009 and 2014 in our hospital was conducted. The clinical manifestations, imaging characteristics, pathology, treatment strategies, and outcomes were examined., Results: The study cohort included 7 women (36.8%) and 12 men (63.2%), and symptom duration ranged from 1 mo to 5 yr. Of the 19 intracranial CMF cases examined, 15 (78.9%) conformed with the diagnostic criteria for extracranial CMF. Resection operations yielded subtotal removal of 13 tumors (68.4%) and partial removal of 6 tumors (31.6%). Postoperative pathological analysis demonstrated that the tumors were characterized by spindle-shaped or stellate cells arranged in a myxoid matrix without mitoses or permeation. Follow-up (range 2-7.3 yr; mean, 4.4 ± 1.7 yr) revealed that symptoms improved postoperatively in 15 cases (78.9%), were maintained in 2 cases (10.5%), and worsened in 2 cases (10.5%). Imaging follow-up revealed that residual tumors were stable in 18 cases (94.7%) and enlarged in 1 case (5.3%)., Conclusion: An accurate diagnosis should involve comprehensive consideration of clinical, radiological, and pathological features. The treatment strategy for CMFs consists of maximizing tumor removal while protecting adjacent key structures. Postoperative stereotactic radiotherapy is appropriate for residual tumors.

Plexiform fibromyxoma (PF) is a recently-described and rare mesenchymal neoplasm of the gastric wall. A few small case series reports of this spindle cell entity exist in the surgical pathology literature, but to our knowledge no prior endoscopic ultrasound guided fine needle aspiration cytology examples have been reported. In clinical practice, mural gastrointestinal (GI) lesions are often initially evaluated by endoscopic ultrasound guided (EUS) fine needle aspiration (FNA). In addition, newer EUS fine needle biopsy techniques also allow for reliable retrieval of core tissue samples with intact cellular architecture, making EUS histopathologic analyses possible. We report a combined EUS FNA and core biopsy case of PF and correlate the findings with imaging results. The cytomorphology of PF is described and illustrated, and important entities in the differential diagnosis of upper GI spindle cell lesions (including GI stromal tumor, leiomyoma, schwannoma, carcinoid tumor, desmoid-type fibromatosis, and inflammatory fibroid polyp) are reviewed. Illustrated examples of relevant cytomorphologic, cell block histomorphologic and immunohistochemical characteristics are emphasized., (© 2018 Wiley Periodicals, Inc.)

Tuberous sclerosis complex (TSC) is an autosomal dominant inherited disease characterized by systemic hamartoma and diverse systemic features. TSC1 and TSC2 are the causative genes, and mental retardation, epileptic seizures, and facial angiofibroma develop in many patients with the disease. The case of a patient with TSC who developed a central odontogenic fibroma of the mandible is reported here. The patient was a 21-year-old woman who was referred with a swelling of the labial gingiva in the region of the right lower lateral incisor and canine. Dental radiography revealed a multilocular radiolucent region with a clear boundary. The right lower lateral incisor and canine were continuous with the lesion and thus were excised en bloc. The lesion was encapsulated and easily dissected. The diagnosis on immunohistological staining was odontogenic fibroma without an epithelial component. TSC1/2 gene mutation causes abnormal activation of mammalian target of rapamycin (mTOR) downstream of the PI3K-AKT pathway. The odontogenic fibroma in this patient was positive for mTOR, suggesting that the development of the odontogenic fibroma was the result of abnormal activation of mTOR, as in angiofibroma. The clinical course of this patient is presented and the developmental mechanism of central odontogenic fibroma is discussed., (Copyright © 2018 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)