Embryonal rhabdomyosarcoma of the perineum in an adult: a case reportReport as inadecuate

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Journal of Medical Case Reports

, 10:353

First Online: 20 December 2016Received: 10 November 2015Accepted: 01 December 2016DOI: 10.1186-s13256-016-1166-2

Cite this article as: Ka, S., Gnangnon, F., Dieng, M.M. et al. J Med Case Reports 2016 10: 353. doi:10.1186-s13256-016-1166-2


BackgroundWe report the case of an adult patient with embryonal rhabdomyosarcoma of the perineum admitted to our practice at Joliot Curie Institute in Dakar. It is a rare tumor at this age and has a bad prognosis at this localization.

Case presentationWe describe the case of a 22-year-old African man admitted for a perineal mass that had evolved over 6 months. He complained of tenesmus, obstinate constipation, and dysuria. A clinical examination revealed perineal swelling spread over his anus, scrotum, penis, testicles, and inguinal lymph nodes. A perineal ultrasound and computed tomography scan showed a large mass driving his testicles forward with regional lymph node metastases. An ultrasound-guided biopsy showed embryonal rhabdomyosarcoma on histology and immunohistochemistry, with strong positivity of neural cell adhesion molecule and myogenin while results for cytokeratin AE1-AE3, cluster of differentiation 45, synaptophysin, and chromogranin were negative. Our patient was classified T2N1M1. Outcome was quickly marked by occlusive syndrome and colostomy. Our patient did not opt for chemotherapy and died after 6 months of follow-up.

ConclusionsThe embryonic RMS of the adult is a rare disease. Despite the sensitivity to chemotherapy and surgery. Localization to perineum remains poor prognosis.

KeywordsEmbryonal Rhabdomyosarcoma Adult Immunohistochemistry AbbreviationsCD45cluster of differentiation 45

CK AE1-AE3cytokeratin AE1-AE3

CTcomputed tomography

ERMSembryonal rhabdomyosarcoma

MRImagnetic resonance imaging

NCAMneural cell adhesion molecule

VACvincristine, actinomycin D, and cyclophosphamide

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Author: Sidy Ka - Freddy Gnangnon - Mamadou Moustapha Dieng - Doudou Diouf - Jaafar Thiam - Pape Macoumba Gaye - Ahmadou Dem

Source: https://link.springer.com/

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