ReviewVirchows Archiv : an international journal of pathology2018
Biphenotypic sinonasal sarcoma: demographics, clinicopathological characteristics, molecular features, and prognosis of a recently described entity.
Review in Virchows Archiv : an international journal of pathology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers, 1 of them a synthesis that pooled it.
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Who cites it
20 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Biphenotypic sinonasal sarcoma: European multicentre case-series and systematic literature review.Acta otorhinolaryngologica Italica : organo ufficiale della Societa italiana di otorinolaringologia e chirurgia cervico-facciale · 2022Pooled it
- Biphenotypic Sinonasal Sarcoma With a Novel PAX3::MAML2 Fusion.Genes, chromosomes & cancer · 2026Article
- Rhabdomyosarcoma in children and young adults.Virchows Archiv : an international journal of pathology · 2025Review
- Biphenotypic Sinonasal Sarcoma: Literature Review of a Peculiar Pathological Entity-The Neurosurgical Point of View.Cancers · 2024Review
- Biphenotypic Sinonasal Sarcoma with Orbital Invasion: A Literature Review and Modular System of Surgical Approaches.Cancers · 2024Review
- Integrated Molecular and Histological Insights for Targeted Therapies in Mesenchymal Sinonasal Tract Tumors.Current oncology reports · 2024Review
- Biphenotypic Sinonasal Sarcoma with Orbital and Skull Base Involvement Report of 3 Cases and Systematic Review of the Literature.Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India · 2023Article
- Biphenotypic Sinonasal Sarcoma with a Novel PAX7::PPARGC1 Fusion: Expanding the Spectrum of Gene Fusions Beyond the PAX3 Gene.Head and neck pathology · 2023Article
- Translocations and Gene Fusions in Sinonasal Malignancies.Current oncology reports · 2023Review
- Top 10 Significant Spindled Head and Neck Lesions to Scrutinze.Head and neck pathology · 2023Review
- Recurrent VGLL3 fusions define a distinctive subset of spindle cell rhabdomyosarcoma with an indolent clinical course and striking predilection for the head and neck.Genes, chromosomes & cancer · 2022Article
- Small Biopsy Samples: Are They Representative for Biphenotypic Sinonasal Sarcoma?Diagnostics (Basel, Switzerland) · 2022Article
- RREB1-MKL2 fusion in a spindle cell sinonasal sarcoma: biphenotypic sinonasal sarcoma or ectomesenchymal chondromyxoid tumor in an unusual site?Genes, chromosomes & cancer · 2021Article
- Biphenotypic sinonasal sarcoma: Report of 3 cases with a review of literature.Human pathology (New York) · 2021Article
- Soft Tissue Special Issue: Biphenotypic Sinonasal Sarcoma: A Review with Emphasis on Differential Diagnosis.Head and neck pathology · 2020Review
- Uterine Tumor Resembling Ovarian Sex Cord Tumor (UTROSCT): A Morphologic and Molecular Study of 26 Cases Confirms Recurrent NCOA1-3 Rearrangement.The American journal of surgical pathology · 2020Article
- PRRX-NCOA1/2 rearrangement characterizes a distinctive fibroblastic neoplasm.Genes, chromosomes & cancer · 2019Article
- Updated WHO nomenclature of head and neck lesions and associated imaging findings.Insights into imaging · 2019Review
- Recurrence of a complex anterior skull base tumour managed with an uncommon surgical approach.BMJ case reports · 2019Article
- Biphenotypic Sinonasal Sarcoma with Intracranial Extension - A Case Report with Review of Literature.Annals of maxillofacial surgeryArticle
Corrections and comments
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Authors and funding
10 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Biphenotypic sinonasal sarcoma (BSNS) is a recently recognized type of sarcoma arising exclusively in the sinonasal tract displaying unique clinical course, histopathology, and genetics. Due to its rarity, only case series and case reports are available. In order to provide an overview of the current understanding of this disease, we present a comprehensive review of the literature and present three previously unreported cases of BSNS. A total of 55 genetically characterized and 41 cases without molecular data were identified in the literature. Two-thirds of patients were female and the peak incidence was in the fifth decade. Fatal outcome was rare (two cases with intracranial extension) and local recurrence occurred in 31.6%, all occurring within 5 years after initial treatment. Histologically, BSNS is highly cellular in the majority of cases and composed of fascicles of spindle cells, with entrapped hyperplastic surface epithelium being a frequent finding. The immunohistochemical profile is characteristic due to the biphasic nature of this lesion, with shared features of both myogenic and neural origin. Rhabdomyoblastic differentiation is apparent in a subset of cases. The most common genetic event is the PAX3-MAML3 fusion (58.6%) but isolated PAX3 rearrangement (19.2%), absence of rearrangements (9.1%), PAX3-FOXO1 (8.1%), PAX3-NCOA1 (4%), and isolated MAML3 rearrangement (2%) have also been reported. In conclusion, the recognition of BSNS is crucial due to its relatively indolent clinical course. A selected immunohistochemical panel and/or molecular confirmation can be used to aid in appropriate diagnosis and consequently in prognostication and to avoid overtreatment with chemotherapy regimens used in its mimics.
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