scholarly journals Biphenotypic Sinonasal Sarcoma: A Review and Update

2018 ◽  
Vol 142 (10) ◽  
pp. 1196-1201 ◽  
Author(s):  
Cody S. Carter ◽  
Ellen G. East ◽  
Jonathan B. McHugh

Context.— Biphenotypic sinonasal sarcoma (BSNS) is a rare, slow-growing soft tissue sarcoma of the sinonasal tract, typically presenting with nonspecific obstructive nasal symptoms. Although recurrences are common, no metastases have been reported, and only 1 patient has died of disease thus far. It characteristically demonstrates rearrangements of PAX3 with multiple fusion partners, the most common of which is MAML3. Objectives.— To highlight the most important diagnostic features, including morphologic, immunohistochemical, and molecular findings, and to provide comparisons to other entities in the differential diagnosis. We also aim to provide a summary of the clinical features and outcomes in cases reported to date. Data Sources.— Recently published literature encompassing BSNS and its synonym, low-grade sinonasal sarcoma with neural and myogenic differentiation. Conclusions.— BSNS is a sinonasal tumor that is important to recognize because its biologic behavior differs from most of the entities in the differential diagnosis. The diagnosis can typically be rendered through a combination of morphology, immunohistochemical stains, and ancillary testing for characteristic PAX3 rearrangements.

2019 ◽  
Vol 143 (12) ◽  
pp. 1504-1512
Author(s):  
Cody S. Carter ◽  
Rajiv M. Patel

Context.— Ossifying fibromyxoid tumor (OFMT) is a rare, slow-growing mesenchymal neoplasm of uncertain histogenesis with intermediate malignant potential. Objective.— To highlight the most important diagnostic features, including morphologic, immunohistochemical, and molecular findings; to provide comparisons to other entities in the differential diagnosis; and to provide a summary of the clinical features and outcomes in cases reported to date. Data Sources.— The data sources include recently published literature encompassing OFMT and tumors in the histologic differential diagnosis, and cases from institutional files. Conclusions.— Ossifying fibromyxoid tumor is important to recognize because of its low-grade morphology but potential for recurrence and metastasis. Recent molecular analysis has expanded the morphologic spectrum of OFMT, with additional cases discovered that are enriched for aggressive behavior. The diagnosis can often be rendered through a combination of morphology and coexpression of S100 protein and desmin, although only a minority of cases described contain all of these primary features. In cases that do not have all of these features, a high index of suspicion guided by morphology and exclusion of other tumors in the histologic differential diagnosis can lead to the correct diagnosis. Growing access to molecular genetic testing will become increasingly important for correct diagnosis of tumors at the ends of the morphologic spectrum.


2020 ◽  
Vol 13 (4) ◽  
pp. e232485
Author(s):  
Beena R Varma ◽  
Krishna Santhosh Kumar ◽  
Rhea Susan Verghese ◽  
Mahija Janardhanan

Lipomas are benign soft tissue neoplasm which rarely occur in the oral cavity. Of the total reported cases of lipoma, only about 15% to 20% of cases have occurred in the head and neck region and the tongue is an even rarer site with only about 4% of the reported cases occurring in that region. They are slow growing and usually asymptomatic in nature. When it grows to a large size, it can hinder the physiological processes that are associated with the area. This case report describes the diagnostic features of tongue lipoma with a brief review of literature.


2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
P. Gongidi ◽  
J. Meshekow ◽  
T. Holdbrook ◽  
P. Germaine

Pilomatrixoma is a common benign soft tissue neoplasm arising from hair follicle cells, typically not exceeding 3 cm and located mainly within the head and neck regions. Lesions greater than 3 cm or those located elsewhere are rare and are often not thought of or high on a differential diagnosis. Moreover, the radiographic features of pilomatrixoma are very nonspecific making the diagnosis even more difficult and rarely described in the radiology literature. We present the largest reported case of pilomatrixoma measuring 24 cm arising from the posterior thorax. Our hope is to increase awareness of this diagnosis for slow-growing soft tissue masses not located in the classically described locations of head and neck, explore the radiographic features on various imaging modalities, and review the current radiology literature.


2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Anastasios V. Koutsopoulos ◽  
Efthimios Sivridis ◽  
Panagiotis Tsikouras ◽  
Vasileios Liberis ◽  
Georgia Karpathiou ◽  
...  

With the exception of leiomyomas, soft tissue tumors of the uterine corpus are not common. This is particularly true for vascular neoplasms, with the epithelioid hemangioendothelioma being a curiosity; not more than twenty-two cases of malignant hemangioendotheliomas have been reported in the literature so far, all of which were high-grade hemangioendotheliomas (hemangiosarcomas). We present herewith a unique case of low-grade epithelioid hemangioendothelioma of the uterus in a pregnant woman aged 29 years. The clinical, histological, and immunohistochemical characteristics of this entity, together with its differential diagnosis, are discussed.


2021 ◽  
pp. 014556132199919
Author(s):  
Evan Kominsky ◽  
Andre E. Boyke ◽  
Daniel Madani ◽  
Ameet Kamat ◽  
Bradley A. Schiff ◽  
...  

Objectives: Biphenotypic sinonasal sarcoma (BSNS), previously low-grade sinonasal sarcoma with neural and myogenic features, is a rare tumor of the sinonasal tract first described in 2012. Due to its rarity, limited literature is available in providing clinicians with a standardized treatment regimen, particularly in cases of positive surgical margins. This article aims to provide a clinical review of the currently available reported cases of BSNS, as well as presenting clinical, radiologic, and pathologic details of 2 novel cases. Methods: Online electronic databases include PubMed and Embase where queried for reports of biphenotypic sinonasal sarcoma or low-grade sinonasal sarcoma with neural and myogenic features. Two previously unpublished cases were included in the results. Data including clinical presentation, epidemiologic data, radiologic evaluation, intraoperative details, histopathology, treatment modality, and postoperative follow-up information were included. Results: A total of 100 previously published cases were identified in 12 prior articles. Mean age at presentation was 52.9 years. Extrasinonasal extension was observed in 27.4% of cases with most common site of extension being cribriform plate. Forty-seven cases included treatment details with surgical excision being the most common modality. Recurrence rates were identical for both surgical excision alone and surgical excision with adjuvant radiotherapy (33.3%). Conclusions: Biphenotypic sinonasal sarcoma is a slow-growing tumor that is amenable to surgical resection. Recurrence rates are similar between surgical excision and surgical excision with adjuvant radiation therapy, but limited data in reported cases preclude a determination of treatment superiority.


2021 ◽  
Author(s):  
Yiyan Lu ◽  
Changhai Qi ◽  
Hongbin Xu ◽  
Mulan Jin

Abstract Purpose: To identify clinicopathological features for the differential diagnosis of appendiceal serrated lesions and polyps (SPs) and low-grade appendiceal mucinous neoplasm (LAMN) for the purpose of avoiding over‐diagnosis.Methods: Clinical data and pathological features of 66 patients with SPs diagnosed at the Aerospace Center Hospital between January 2013 and January 2021 were collected and compared to 22 cases of LAMN.Results: SPs, compared with LAMN, are likely to be associated with acute inflammation (SPs 53.0% vs. LAMN 18.2%), and may be located in the appendix partly, although with smaller diameter (average 9.6 vs. 27.2 mm); epithelial structures of serrated (100% vs. 22.7%) and filiform villous (47.0% vs. 18.2%) were often found in SPs. SPs occasionally show attenuated or flattened morphology (16.7% vs. 100%) and undulating or scalloped (7.6% vs. 40.9%) structures, and can also be accompanied by diverticulum (18.2% vs. 18.2%) and acellular mucin in the appendiceal wall (16.7% vs. 54.5%), which causes confusion with LAMN. The key point of the differential diagnosis is to observe whether the muscularis mucosa exists (loss, 0% vs. 100%) and fibrosis of the appendiceal wall (0% vs. 100%). SMA immunohistochemistry can assist in the diagnosis. Calcification is also indicative of LAMN.Conclusions: The epithelial structure of SPs can appear flattened and focally scalloped, and can be accompanied by mucin in the appendiceal wall, which may appear as complex lesions, easily over-diagnosed as LAMN. Key differential diagnostic features are identifying the structure of lamina propria, determining whether the muscularis mucosa exists, and whether the appendiceal wall is fibrotic.


2018 ◽  
Vol 108 (3) ◽  
pp. 262-266 ◽  
Author(s):  
Ariel Lepoff ◽  
Vyacheslav Makarov ◽  
Marie Williams

A 68-year-old man with a slow-growing lesion in the distal medial band of the plantar fascia of the left foot is presented. Clinical photographs, ultrasound and magnetic resonance images, histologic results, and immunochemical staining are disclosed. This case study presentation aims to highlight the importance of including angioleiomyoma in the differential diagnosis of plantar foot soft-tissue masses.


2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Tian-shi Ma ◽  
Ling Zhou ◽  
Quan Zhou ◽  
Xiang-lei He ◽  
Ming Zhao

Abstract Background Soft tissue perineurioma of the kidney is rare, with only a few reported cases. We report two additional cases with histologic, immunohistochemical and genetic analyses. Case presentation Both tumors were from adults (1 female aged 49 years and 1 male aged 42 years) and grossly had maximum diameters of 6.5 and 10 cm, respectively. The tumors were overall well circumscribed but unencapsulated, with focally entrapped benign native renal tubules in one case; both tumors seemed to arise in the capsular areas. The tumors had histologic and immunohistochemical profiles consistent with soft tissue perineurioma. Fluorescence in situ hybridization analyses demonstrated that the tumors were negative for amplification of MDM2 and rearrangements of ESWR1, FUS, and KMT2A. Targeted next-generation sequencing revealed a low tumor mutation burden and likely pathogenic mutations (CYP2B6 and FLT1 mutations for 1 each). Follow-up data were available for both patients; neither had tumor recurrence or metastasis. Conclusions In conclusion, renal perineurioma is rare, usually arises in the capsular areas, and is cured by resection. Low-grade dedifferentiated liposarcoma and low-grade fibromyxoid sarcoma as well as other spindle cell lesions should be considered in the differential diagnosis.


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