Biphenotypic sinonasal sarcoma: Report of 3 cases with a review of literature

Sethi, Shenon; Cody, Brittany; Farhat, Nada; Pool, Mark; Katabi, Nora

doi:10.1016/j.ehpc.2021.200491

Cited by 8 publications

(6 citation statements)

References 32 publications

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“…Rooper et al and Kakkar et al showed 10/11 and 5/6 cases to have focal nuclear expression of beta catenin however, Sethi et al described 3 case of BSNS with absence of nuclear beta catenin expression. 10,14,15 The current case also lacked beta catenin expression. A plethora of differential diagnosis were considered and ruled out one by one the basis of morphology and IHC.…”

Section: Discussionmentioning

confidence: 59%

Sinonasal biphenotypic sarcoma of uvula: the first case report

et al. 2023

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Biphenotypic sinonasal sarcoma is a rare entity and it is a new term included in literature recently. It affects sinonasal tract exclusively but we report the first case of biphenotypic sinonasal sarcoma of uvula. The patient is a 56-year-old male who had a mass in the oropharynx hanging from the uvula exclusively. Contrast enhanced computed tomography of oral cavity reported homogenously enhancing benign mass arising from uvula probably pleomorphic adenoma. It was excised in toto and sent for histopathological examination. The detailed histopathological analysis concluded the diagnosis as sinonasal biphenotypic sarcoma of uvula. Sinonasal biphenotypic sarcoma is exclusively found in sinonasal tract. Occurrence of this entity in uvula is first of its kind and needs special attention.

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Section: Discussionmentioning

confidence: 59%

Sinonasal biphenotypic sarcoma of uvula: the first case report

et al. 2023

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“…It was included in the most recent edition of the World Health Organization classification of head and neck tumour -β-version in 2022 [10]. Only 6 series have been published with 28, 44, 41, 11, 15, and 3 cases, respectively [1][2][3][4][5][6]. Among these cases, 5 showed PAX3/FOXO1 fusion.…”

Section: Discussionmentioning

confidence: 99%

“…Biphenotypic sinonasal sarcoma (BSNS), previously known as low-grade sinonasal sarcoma, is a rare tumour of the sinonasal tract, which was first described in 2012 [1]. There are case series consisting of 28 patients, 44 patients, 41 patients, 11 patients, 15 patients, and 3 patients that have been reported in the literature to date [1][2][3][4][5][6], of which there were 5 cases showing PAX3/ FOXO1 fusion; 3 cases in a series of 44 patients, and one case in a series of 41 patients. In addition to these, there is also one case report in the literature [2,3,7] (Table I).…”

Section: Introductionmentioning

confidence: 99%

Biphenotypic sinonasal sarcoma with PAX3/FOXO1 fusion

Tosun,

Karabulut,

Yilmaz

et al. 2023

pjp

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Biphenotypic sinonasal sarcoma (BSNS), previously known as low-grade sinonasal sarcoma, is a rare tumour of the sinonasal tract, first described in 2012. It involves both myogenic and neural differentiation and is characterized by PAX3 rearrangement. MAML3 is the most frequent fusion partner of PAX3; however, its partner remains unidentified in a subset of cases. These tumours have significant local recurrence rates but lack metastatic potential. Here, we report a case of BSNS with PAX3/ FOXO1 fusion and discuss its clinicopathological features and differential diagnosis.

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“…These findings supported that CT and MRI were not specific for diagnosis. The key histological features of BSNS are that it is infiltrative, showing an insufficient matrix arranged in fascicles or “herringbone”, a hemangiopericytoma-like vascular pattern and a highly cellular uniformity of spindle cell proliferation with benign sinonasal-type glands interspersed with spindle cell proliferation ( 10 ). The nuclei are uniform, pale, and thin.…”

Section: Discussionmentioning

confidence: 99%

A Rare Sinonasal Malignancy: Biphenotypic Sinonasal Sarcoma

Sudabatmaz¹,

Abakay²,

Koçbıyık³

et al. 2022

TAO

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Biphenotypic sinonasal sarcoma (BSNS), which has been described in the recent years, is a low-grade spindle cell sinonasal sarcoma characterized by rare neural and myogenic features. It has a slow growth pattern; does not metastasize, but local recurrences are common after surgery. Non-specificity of examination findings and symptoms and similarities of its histopathological features with other spindle cell sarcomas, neural tumors, and skeletal muscle-derived tumors involving the nasal cavity make the diagnosis difficult. Therefore, histopathological features should be evaluated together with immunophenotyping and molecular studies for differential diagnosis. There are very few BSNS cases or case series in the literature. In this report, we reported our clinical approach to a case with BSNS in the right nasal cavity and the histopathological features of the disease in the light of the current literature.

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Biphenotypic sinonasal sarcoma: Report of 3 cases with a review of literature

Cited by 8 publications

References 32 publications

Sinonasal biphenotypic sarcoma of uvula: the first case report

Sinonasal biphenotypic sarcoma of uvula: the first case report

Biphenotypic sinonasal sarcoma with PAX3/FOXO1 fusion

A Rare Sinonasal Malignancy: Biphenotypic Sinonasal Sarcoma

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