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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
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

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVBiphenotypic Sinonasal Sarcoma

Disorder by Site
- Head and Neck Disorder
  - Head and neck neoplasm
    - Head and neck cancer
      - Head and Neck Sarcoma
        Sinonasal Sarcoma
        Biphenotypic 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

Clinical Utility of In Situ Hybridization Assays in Head and Neck Neoplasms.

Luk PP, Selinger CI, Cooper WA, Mahar A, Palme CE, O'Toole SA, Clark JR, Gupta R
Head Neck Pathol 2019 Sep;13(3):397-414. Epub 2018 Nov 22 doi: 10.1007/s12105-018-0988-1. PMID: 30467669 Free PMC Article

See all (5)

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: 36654521 Free 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: 30134255 Free 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

See all (8)

Diagnosis

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

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: 31950469 Free 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

See all (35)

Prognosis

Biphenotypic sinonasal sarcoma - a case report.

Bartoš V, Rác P, Skálová A
Klin Onkol 2022 Fall;35(5):402-406. doi: 10.48095/ccko2022402. PMID: 36443095

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

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

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

See all (11)

Clinical prediction guides

Biphenotypic Sinonasal Sarcoma: A Genetically Confirmed Case Showing Bone Invasion Accompanying a Non-neoplastic Respiratory Epithelium.

Muraoka E, Kato I, Matsumura M, Arai Y, Suenaga J, Yamanaka S, Fujii S
Int J Surg Pathol 2023 Oct;31(7):1414-1419. Epub 2023 Feb 19 doi: 10.1177/10668969231152577. PMID: 36802923

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.

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: 30134255 Free 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

See all (7)