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Biphenotypic Sinonasal Sarcoma

MedGen UID:
1702786
•Concept ID:
C5230517
•
Neoplastic Process
Synonyms: Biphenotypic sinonasal sarcoma; Low Grade Sinonasal Sarcoma with Neural and Myogenic Differentiation; Low-Grade Sinonasal Sarcoma with Neural and Myogenic Differentiation; Sinonasal Biphenotypic Sarcoma
SNOMED CT: Biphenotypic sinonasal sarcoma (789405004)

Definition

A rare, slow-growing, low-grade soft tissue sarcoma arising from the sinonasal tract. It presents with non-specific obstructive nasal symptoms. Morphologically, it is an infiltrative, cellular spindle cell neoplasm. It is associated with rearrangement of the PAX3 gene. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBiphenotypic Sinonasal Sarcoma

Professional guidelines

PubMed

Challenging differential diagnoses in small biopsies from the sinonasal tract.
Israel AK, Cracolici V, Griffith CC
Semin Diagn Pathol 2023 Sep;40(5):321-332. Epub 2023 Apr 14 doi: 10.1053/j.semdp.2023.04.011. PMID: 37085435
Clinical management of emerging sinonasal malignancies.
Contrera KJ, Woody NM, Rahman M, Sindwani R, Burkey BB
Head Neck 2020 Aug;42(8):2202-2212. Epub 2020 Mar 25 doi: 10.1002/hed.26150. PMID: 32212360
Soft Tissue Special Issue: Biphenotypic Sinonasal Sarcoma: A Review with Emphasis on Differential Diagnosis.
Gross J, Fritchie K
Head Neck Pathol 2020 Mar;14(1):33-42. Epub 2020 Jan 16 doi: 10.1007/s12105-019-01092-4. PMID: 31950469Free PMC Article

Recent clinical studies

Etiology

Challenging differential diagnoses in small biopsies from the sinonasal tract.
Israel AK, Cracolici V, Griffith CC
Semin Diagn Pathol 2023 Sep;40(5):321-332. Epub 2023 Apr 14 doi: 10.1053/j.semdp.2023.04.011. PMID: 37085435
Recurrent EWSR1::COLCA2 Fusions Define a Novel Sarcoma With Spindle/Round Cell Morphology and Strong Predilection for the Sinonasal Tract.
Agaimy A, Baněčková M, De Almeida J, Dickson BC, Dimmler A, Hartmann W, Laé M, Pablik J, Schubart C, Skálová A, Stoehr R, Trautmann M, Wardelmann E, Wassef M, Weinreb I
Am J Surg Pathol 2023 Mar 1;47(3):361-369. Epub 2022 Nov 14 doi: 10.1097/PAS.0000000000002000. PMID: 36580038
Biphenotypic sinonasal sarcoma: European multicentre case-series and systematic literature review.
Turri-Zanoni M, Dalfino G, Lechner M, Dallan I, Battaglia P, Facco C, Franzi F, Gravante G, Ferrari M, Terzakis D, Jay A, Forster MD, Ambrosoli AL, Bignami M, Georgalas C, Herman P, Nicolai P, Lund VJ, Castelnuovo P
Acta Otorhinolaryngol Ital 2022 Dec;42(6):545-553. doi: 10.14639/0392-100X-N2087. PMID: 36654521Free PMC Article
Diagnostic Immunohistochemistry for Soft Tissue and Bone Tumors: An Update.
Schaefer IM, Hornick JL
Adv Anat Pathol 2018 Nov;25(6):400-412. doi: 10.1097/PAP.0000000000000204. PMID: 30134255Free PMC Article
Recently described neoplasms of the sinonasal tract.
Bishop JA
Semin Diagn Pathol 2016 Mar;33(2):62-70. Epub 2015 Dec 23 doi: 10.1053/j.semdp.2015.12.001. PMID: 26776744

Diagnosis

Biphenotypic Sinonasal Sarcoma: A Case Report and Review of Literature.
Kominsky E, Boyke AE, Madani D, Kamat A, Schiff BA, Agarwal V
Ear Nose Throat J 2023 Jun;102(6):385-390. Epub 2021 Apr 5 doi: 10.1177/0145561321999196. PMID: 33813901
Clinical management of emerging sinonasal malignancies.
Contrera KJ, Woody NM, Rahman M, Sindwani R, Burkey BB
Head Neck 2020 Aug;42(8):2202-2212. Epub 2020 Mar 25 doi: 10.1002/hed.26150. PMID: 32212360
Soft Tissue Special Issue: Biphenotypic Sinonasal Sarcoma: A Review with Emphasis on Differential Diagnosis.
Gross J, Fritchie K
Head Neck Pathol 2020 Mar;14(1):33-42. Epub 2020 Jan 16 doi: 10.1007/s12105-019-01092-4. PMID: 31950469Free PMC Article
Biphenotypic Sinonasal Sarcoma: A Review and Update.
Carter CS, East EG, McHugh JB
Arch Pathol Lab Med 2018 Oct;142(10):1196-1201. doi: 10.5858/arpa.2018-0207-RA. PMID: 30281368
Distinctive Head and Neck Bone and Soft Tissue Neoplasms.
Purgina B, Lai CK
Surg Pathol Clin 2017 Mar;10(1):223-279. doi: 10.1016/j.path.2016.11.003. PMID: 28153134

Prognosis

Clinical management of emerging sinonasal malignancies.
Contrera KJ, Woody NM, Rahman M, Sindwani R, Burkey BB
Head Neck 2020 Aug;42(8):2202-2212. Epub 2020 Mar 25 doi: 10.1002/hed.26150. PMID: 32212360
Biphenotypic sinonasal sarcoma: demographics, clinicopathological characteristics, molecular features, and prognosis of a recently described entity.
Andreasen S, Bishop JA, Hellquist H, Hunt J, Kiss K, Rinaldo A, Skálová A, Willems SM, Williams M, Ferlito A
Virchows Arch 2018 Nov;473(5):615-626. Epub 2018 Aug 14 doi: 10.1007/s00428-018-2426-x. PMID: 30109475
Biphenotypic sinonasal sarcoma: an expanded immunoprofile including consistent nuclear β-catenin positivity and absence of SOX10 expression.
Rooper LM, Huang SC, Antonescu CR, Westra WH, Bishop JA
Hum Pathol 2016 Sep;55:44-50. Epub 2016 Apr 29 doi: 10.1016/j.humpath.2016.04.009. PMID: 27137987Free PMC Article
Recently described neoplasms of the sinonasal tract.
Bishop JA
Semin Diagn Pathol 2016 Mar;33(2):62-70. Epub 2015 Dec 23 doi: 10.1053/j.semdp.2015.12.001. PMID: 26776744
Soft tissue tumors of the sinonasal tract.
Johncilla M, Jo VY
Semin Diagn Pathol 2016 Mar;33(2):81-90. Epub 2015 Sep 9 doi: 10.1053/j.semdp.2015.09.009. PMID: 26472693

Clinical prediction guides

Biphenotypic Sinonasal Sarcoma: A Case Report and Review of Literature.
Kominsky E, Boyke AE, Madani D, Kamat A, Schiff BA, Agarwal V
Ear Nose Throat J 2023 Jun;102(6):385-390. Epub 2021 Apr 5 doi: 10.1177/0145561321999196. PMID: 33813901
Biphenotypic sinonasal sarcoma: European multicentre case-series and systematic literature review.
Turri-Zanoni M, Dalfino G, Lechner M, Dallan I, Battaglia P, Facco C, Franzi F, Gravante G, Ferrari M, Terzakis D, Jay A, Forster MD, Ambrosoli AL, Bignami M, Georgalas C, Herman P, Nicolai P, Lund VJ, Castelnuovo P
Acta Otorhinolaryngol Ital 2022 Dec;42(6):545-553. doi: 10.14639/0392-100X-N2087. PMID: 36654521Free PMC Article
Diagnostic Immunohistochemistry for Soft Tissue and Bone Tumors: An Update.
Schaefer IM, Hornick JL
Adv Anat Pathol 2018 Nov;25(6):400-412. doi: 10.1097/PAP.0000000000000204. PMID: 30134255Free PMC Article
Expression of PAX3 Distinguishes Biphenotypic Sinonasal Sarcoma From Histologic Mimics.
Jo VY, Mariño-Enríquez A, Fletcher CDM, Hornick JL
Am J Surg Pathol 2018 Oct;42(10):1275-1285. doi: 10.1097/PAS.0000000000001092. PMID: 29863547
Biphenotypic sinonasal sarcoma: an expanded immunoprofile including consistent nuclear β-catenin positivity and absence of SOX10 expression.
Rooper LM, Huang SC, Antonescu CR, Westra WH, Bishop JA
Hum Pathol 2016 Sep;55:44-50. Epub 2016 Apr 29 doi: 10.1016/j.humpath.2016.04.009. PMID: 27137987Free PMC Article

Recent systematic reviews

Biphenotypic sinonasal sarcoma: European multicentre case-series and systematic literature review.
Turri-Zanoni M, Dalfino G, Lechner M, Dallan I, Battaglia P, Facco C, Franzi F, Gravante G, Ferrari M, Terzakis D, Jay A, Forster MD, Ambrosoli AL, Bignami M, Georgalas C, Herman P, Nicolai P, Lund VJ, Castelnuovo P
Acta Otorhinolaryngol Ital 2022 Dec;42(6):545-553. doi: 10.14639/0392-100X-N2087. PMID: 36654521Free PMC Article

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