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Rhabdoid tumor of the ovary

MedGen UID:
1841849
•Concept ID:
C5826477
•
Neoplastic Process
HPO: HP:0034558

Definition

A highly aggressive, extremely rare neoplasm of the ovary that is usually seen in children. The term rhabdoid is used because the tumor cells resemble rhabdomyoblasts but lack myogenic markers. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRhabdoid tumor of the ovary

Professional guidelines

PubMed

Rare ovarian tumors: an update on diagnosis and treatment.
Debuquoy C, Romeo C, Vanacker H, Ray-Coquard I
Int J Gynecol Cancer 2020 Jun;30(6):879-887. Epub 2020 May 26 doi: 10.1136/ijgc-2020-001235. PMID: 32461259
Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 1.
Chokoeva AA, Tchernev G, Cardoso JC, Patterson JW, Dechev I, Valkanov S, Zanardelli M, Lotti T, Wollina U
Int J Immunopathol Pharmacol 2015 Jun;28(2):168-77. Epub 2015 Mar 26 doi: 10.1177/0394632015576029. PMID: 25816394
Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 2.
Chokoeva AA, Tchernev G, Cardoso JC, Patterson JW, Dechev I, Valkanov S, Zanardelli M, Lotti T, Wollina U
Int J Immunopathol Pharmacol 2015 Jun;28(2):178-86. Epub 2015 Mar 26 doi: 10.1177/0394632015575977. PMID: 25816393

Recent clinical studies

Diagnosis

Biallelic somatic SMARCA4 mutations in small cell carcinoma of the ovary, hypercalcemic type (SCCOHT).
Bailey S, Murray MJ, Witkowski L, Hook E, Hasselblatt M, Crawford R, Foulkes WD, Tischkowitz M, Nicholson JC
Pediatr Blood Cancer 2015 Apr;62(4):728-30. Epub 2014 Oct 12 doi: 10.1002/pbc.25279. PMID: 25307865

Supplemental Content

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