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Vulvar Rhabdomyoma

MedGen UID:
1793824
•Concept ID:
C5557383
•
Neoplastic Process

Definition

A rare benign polypoid skeletal muscle neoplasm arising from the vulva. It is characterized by the presence of round striated muscle cells and strap-like skeletal muscle cells, and vascular space formations within a fibrous and myxoid stroma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVVulvar Rhabdomyoma

Recent clinical studies

Diagnosis

Vulvar fetal rhabdomyoma mimicking 46XX sex differentiation disorder.
Martos-Moreno GÁ, de Prada I, Riñón C, Argente J
J Pediatr Endocrinol Metab 2016 Feb;29(2):217-20. doi: 10.1515/jpem-2015-0202. PMID: 26352088
Myogenin expression in vulvovaginal spindle cell lesions: analysis of a series of cases with an emphasis on diagnostic pitfalls.
McCluggage WG, Longacre TA, Fisher C
Histopathology 2013 Oct;63(4):545-50. Epub 2013 Aug 14 doi: 10.1111/his.12205. PMID: 23944986
Epididymal rhabdomyoma: report of a case, including histologic and immunohistochemical findings.
Wehner MS, Humphreys JL, Sharkey FE
Arch Pathol Lab Med 2000 Oct;124(10):1518-9. doi: 10.5858/2000-124-1518-ER. PMID: 11035587
Malignant rhabdoid tumor of the vulva: is distinction from epithelioid sarcoma possible? A pathologic and immunohistochemical study.
Perrone T, Swanson PE, Twiggs L, Ulbright TM, Dehner LP
Am J Surg Pathol 1989 Oct;13(10):848-58. PMID: 2476944
Extracardiac rhabdomyoma: a clinicopathologic study and review of the literature.
Di Sant'Agnese PA, Knowles DM 2nd
Cancer 1980 Aug 15;46(4):780-9. doi: 10.1002/1097-0142(19800815)46:4<780::aid-cncr2820460423>3.0.co;2-k. PMID: 7397640

Prognosis

Malignant rhabdoid tumor of the vulva: is distinction from epithelioid sarcoma possible? A pathologic and immunohistochemical study.
Perrone T, Swanson PE, Twiggs L, Ulbright TM, Dehner LP
Am J Surg Pathol 1989 Oct;13(10):848-58. PMID: 2476944

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