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Sinonasal Desmoid Fibromatosis

MedGen UID:
1750489
•Concept ID:
C5420053
•
Neoplastic Process
Synonym: Sinonasal Desmoid-Type Fibromatosis

Definition

Desmoid fibromatosis that arises from the nasal cavity and paranasal sinuses. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSinonasal Desmoid Fibromatosis

Recent clinical studies

Etiology

Desmoid fibromatosis of the sinonasal tract and nasopharynx. A clinicopathologic study of 25 cases.
Gnepp DR, Henley J, Weiss S, Heffner D
Cancer 1996 Dec 15;78(12):2572-9. doi: 10.1002/(sici)1097-0142(19961215)78:12<2572::aid-cncr17>3.0.co;2-t. PMID: 8952566

Diagnosis

CD34-Positive Spindle Cell Tumor With CTNNB1 Mutation: An Unusual Spindle Cell Variant of Sinonasal Glomangiopericytoma.
Iwamura R, Nawata A, Akaike R, Kitamura T, Hisaoka M
Int J Surg Pathol 2024 Apr;32(2):408-413. Epub 2023 Jun 8 doi: 10.1177/10668969231177889. PMID: 37287277
Sinonasal Myxoma: A Distinct Entity or a Myxoid Variant of Desmoid Fibromatosis?
Velez Torres JM, Mata DA, Briski LM, Green DC, Cloutier JM, Kerr DA, Montgomery EA, Rosenberg AE
Mod Pathol 2023 Jul;36(7):100189. Epub 2023 Apr 13 doi: 10.1016/j.modpat.2023.100189. PMID: 37059229
Next-Generation Sequencing in the Diagnosis of Rare Pediatric Sinonasal Tumors.
Ahmed AA, Vundamati D, Farooqi M, Repnikova E, Zinkus T, Hetherington M, Paulson L
Ear Nose Throat J 2021 Jun;100(5):NP263-NP268. Epub 2019 Sep 24 doi: 10.1177/0145561319863371. PMID: 31550935
CTNNB1 (β-Catenin)-altered Neoplasia: A Review Focusing on Soft Tissue Neoplasms and Parenchymal Lesions of Uncertain Histogenesis.
Agaimy A, Haller F
Adv Anat Pathol 2016 Jan;23(1):1-12. doi: 10.1097/PAP.0000000000000104. PMID: 26645457
Aggressive psammomatoid ossifying fibroma of the inferior turbinate and lateral nasal wall.
Danielides V, Ingels K, Patrikakos G, de Wilde PC
Acta Otorhinolaryngol Belg 2003;57(1):87-90. PMID: 12642959

Prognosis

Sinonasal Myxoma: A Distinct Entity or a Myxoid Variant of Desmoid Fibromatosis?
Velez Torres JM, Mata DA, Briski LM, Green DC, Cloutier JM, Kerr DA, Montgomery EA, Rosenberg AE
Mod Pathol 2023 Jul;36(7):100189. Epub 2023 Apr 13 doi: 10.1016/j.modpat.2023.100189. PMID: 37059229
Desmoid fibromatosis of the sinonasal tract and nasopharynx. A clinicopathologic study of 25 cases.
Gnepp DR, Henley J, Weiss S, Heffner D
Cancer 1996 Dec 15;78(12):2572-9. doi: 10.1002/(sici)1097-0142(19961215)78:12<2572::aid-cncr17>3.0.co;2-t. PMID: 8952566

Clinical prediction guides

Sinonasal Myxoma: A Distinct Entity or a Myxoid Variant of Desmoid Fibromatosis?
Velez Torres JM, Mata DA, Briski LM, Green DC, Cloutier JM, Kerr DA, Montgomery EA, Rosenberg AE
Mod Pathol 2023 Jul;36(7):100189. Epub 2023 Apr 13 doi: 10.1016/j.modpat.2023.100189. PMID: 37059229

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