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Ossifying fibromyxoid tumor

MedGen UID:
226846
•Concept ID:
C1266128
•
Neoplastic Process
Synonyms: OFMT; Ossifying Fibromyxoid Neoplasm; ossifying fibromyxoid neoplasm; Ossifying Fibromyxoid Tumor; ossifying fibromyxoid tumor; ossifying fibromyxoid tumor (morphologic abnormality); ossifying fibromyxoid tumour (morphologic abnormality); Ossifying Fibromyxoma; ossifying fibromyxoma
SNOMED CT: Ossifying fibromyxoid tumor (404076001); Ossifying fibromyxoid tumor (128745002)
 
Monarch Initiative: MONDO:0006330

Definition

A rare soft tissue tumor of uncertain lineage characterized by the presence of neoplastic spindle to round cells forming cords in a fibromyxoid stroma. The lesions are associated with the formation of metaplastic bone. Most patients present with painless subcutaneous masses. Recurrences have been reported in a minority of patients. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVOssifying fibromyxoid tumor

Professional guidelines

PubMed

Differential diagnosis of T2 hypointense masses in musculoskeletal MRI.
Finkelstein D, Foremny G, Singer A, Clifford P, Pretell-Mazzini J, Kerr DA, Subhawong TK
Skeletal Radiol 2021 Oct;50(10):1981-1994. Epub 2021 Mar 2 doi: 10.1007/s00256-021-03711-0. PMID: 33651128
Ossifying Fibromyxoid Tumor: A Review With Emphasis on Recent Molecular Advances and Differential Diagnosis.
Carter CS, Patel RM
Arch Pathol Lab Med 2019 Dec;143(12):1504-1512. doi: 10.5858/arpa.2019-0371-RA. PMID: 31765250
Ossifying fibromyxoid tumor: morphology, genetics, and differential diagnosis.
Schneider N, Fisher C, Thway K
Ann Diagn Pathol 2016 Feb;20:52-8. Epub 2015 Dec 2 doi: 10.1016/j.anndiagpath.2015.11.002. PMID: 26732302

Recent clinical studies

Etiology

A review of neoplasms with MITF/MiT family translocations.
Wei S, Testa JR, Argani P
Histol Histopathol 2022 Apr;37(4):311-321. Epub 2022 Feb 2 doi: 10.14670/HH-18-426. PMID: 35107169
Soft Tissue Tumors of Uncertain Histogenesis: A Review for Dermatopathologists.
Buehler D, Weisman P
Clin Lab Med 2017 Sep;37(3):647-671. doi: 10.1016/j.cll.2017.06.005. PMID: 28802505
Chondro-Osseous Lesions of Soft Tissue.
Cho SJ, Horvai A
Surg Pathol Clin 2015 Sep;8(3):419-44. doi: 10.1016/j.path.2015.05.004. PMID: 26297064
Ossifying fibromyxoid tumor of head and neck region: case report and systematic review of literature.
Kondylidou-Sidira A, Kyrgidis A, Antoniades H, Antoniades K
J Oral Maxillofac Surg 2011 May;69(5):1355-60. Epub 2010 Oct 14 doi: 10.1016/j.joms.2010.05.011. PMID: 20950910
Ossifying fibromyxoid tumor of soft parts: a clinicopathologic study of 70 cases with emphasis on atypical and malignant variants.
Folpe AL, Weiss SW
Am J Surg Pathol 2003 Apr;27(4):421-31. doi: 10.1097/00000478-200304000-00001. PMID: 12657926

Diagnosis

Ossifying Fibromyxoid Tumor: A Review With Emphasis on Recent Molecular Advances and Differential Diagnosis.
Carter CS, Patel RM
Arch Pathol Lab Med 2019 Dec;143(12):1504-1512. doi: 10.5858/arpa.2019-0371-RA. PMID: 31765250
Ossifying Fibromyxoid Tumor: An Update.
Bakiratharajan D, Rekhi B
Arch Pathol Lab Med 2016 Apr;140(4):371-5. doi: 10.5858/arpa.2014-0590-RS. PMID: 27028395
Ossifying fibromyxoid tumor: morphology, genetics, and differential diagnosis.
Schneider N, Fisher C, Thway K
Ann Diagn Pathol 2016 Feb;20:52-8. Epub 2015 Dec 2 doi: 10.1016/j.anndiagpath.2015.11.002. PMID: 26732302
Chondro-Osseous Lesions of Soft Tissue.
Cho SJ, Horvai A
Surg Pathol Clin 2015 Sep;8(3):419-44. doi: 10.1016/j.path.2015.05.004. PMID: 26297064
Atypical ossifying fibromyxoid tumor.
Lagergren E, Powell MS
Am Surg 2014 Aug;80(8):E257-8. PMID: 25105387

Therapy

Novel MEAF6-SUZ12 fusion in ossifying fibromyxoid tumor with unusual features.
Killian K, Leckey BD, Naous R, McGough RL, Surrey LF, John I
Genes Chromosomes Cancer 2021 Sep;60(9):631-634. Epub 2021 Apr 21 doi: 10.1002/gcc.22951. PMID: 33840146
Atypical and malignant variants of ossifying fibromyxoid tumor. Clinicopathologic analysis of six cases.
Kilpatrick SE, Ward WG, Mozes M, Miettinen M, Fukunaga M, Fletcher CD
Am J Surg Pathol 1995 Sep;19(9):1039-46. doi: 10.1097/00000478-199509000-00007. PMID: 7661277
Ossifying fibromyxoid tumor of soft parts: a morphological and immunohistochemical study.
Yang P, Hirose T, Hasegawa T, Gao Z, Hizawa K
Pathol Int 1994 Jun;44(6):448-53. doi: 10.1111/j.1440-1827.1994.tb01709.x. PMID: 8055111

Prognosis

Ossifying fibromyxoid tumor: morphology, genetics, and differential diagnosis.
Schneider N, Fisher C, Thway K
Ann Diagn Pathol 2016 Feb;20:52-8. Epub 2015 Dec 2 doi: 10.1016/j.anndiagpath.2015.11.002. PMID: 26732302
Chondro-Osseous Lesions of Soft Tissue.
Cho SJ, Horvai A
Surg Pathol Clin 2015 Sep;8(3):419-44. doi: 10.1016/j.path.2015.05.004. PMID: 26297064
Diagnostically Challenging Epithelioid Soft Tissue Tumors.
James AW, Dry SM
Surg Pathol Clin 2015 Sep;8(3):309-29. doi: 10.1016/j.path.2015.05.002. PMID: 26297059
Ossifying fibromyxoid tumor of head and neck region: case report and systematic review of literature.
Kondylidou-Sidira A, Kyrgidis A, Antoniades H, Antoniades K
J Oral Maxillofac Surg 2011 May;69(5):1355-60. Epub 2010 Oct 14 doi: 10.1016/j.joms.2010.05.011. PMID: 20950910
Ossifying fibromyxoid tumor of soft parts: a clinicopathologic study of 70 cases with emphasis on atypical and malignant variants.
Folpe AL, Weiss SW
Am J Surg Pathol 2003 Apr;27(4):421-31. doi: 10.1097/00000478-200304000-00001. PMID: 12657926

Clinical prediction guides

Differential diagnosis of T2 hypointense masses in musculoskeletal MRI.
Finkelstein D, Foremny G, Singer A, Clifford P, Pretell-Mazzini J, Kerr DA, Subhawong TK
Skeletal Radiol 2021 Oct;50(10):1981-1994. Epub 2021 Mar 2 doi: 10.1007/s00256-021-03711-0. PMID: 33651128
Non-epithelial tumors of the larynx: a single institution review.
Karatayli-Ozgursoy S, Bishop JA, Hillel AT, Akst LM, Best SR
Am J Otolaryngol 2016 May-Jun;37(3):279-85. Epub 2016 Jan 27 doi: 10.1016/j.amjoto.2016.01.005. PMID: 27178524
Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems.
Rodriguez FJ, Folpe AL, Giannini C, Perry A
Acta Neuropathol 2012 Mar;123(3):295-319. Epub 2012 Feb 12 doi: 10.1007/s00401-012-0954-z. PMID: 22327363Free PMC Article
Ossifying fibromyxoid tumor in the mandibular gingiva: case report and review of the literature.
Nonaka CF, Pacheco DF, Nunes RP, Freitas Rde A, Miguel MC
J Periodontol 2009 Apr;80(4):687-92. doi: 10.1902/jop.2009.080535. PMID: 19335090
Ossifying subcutaneous tumor with myofibroblastic differentiation: a variant of ossifying fibromyxoid tumor of soft parts?
Fukunaga M, Ushigome S, Ishikawa E
Pathol Int 1994 Sep;44(9):727-34. doi: 10.1111/j.1440-1827.1994.tb02954.x. PMID: 7804436

Recent systematic reviews

Ossifying Fibromyxoid Tumor of the Retromolar Trigone: A Case Report and Systematic Review of the Literature.
Titsinides S, Nikitakis NG, Tasoulas J, Daskalopoulos A, Goutzanis L, Sklavounou A
Int J Surg Pathol 2017 Sep;25(6):526-532. Epub 2017 Apr 24 doi: 10.1177/1066896917705197. PMID: 28436288
Ossifying fibromyxoid tumor of head and neck region: case report and systematic review of literature.
Kondylidou-Sidira A, Kyrgidis A, Antoniades H, Antoniades K
J Oral Maxillofac Surg 2011 May;69(5):1355-60. Epub 2010 Oct 14 doi: 10.1016/j.joms.2010.05.011. PMID: 20950910

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