Pleomorphic Rhabdomyosarcoma of vulva in middle-aged
woman;
case-report of an extremely rare neoplasm
Despotelli M, Papaliodi E, Pandazopoulou A, Frangia K, Kontogeorgos
G.
Department of Pathology, General Hospital of Athens, «G. Gennimatas»
We report a case of pleomorphic rhabdomyosarcoma (RMS) of vulva
in a middle aged woman with unusual correlation between patient’s
age and tumor localization that fulfill the newly established
morphological and immunohistochemical diagnostic criteria of
pleomorphic RMS; [Desmin (+), Smooth Muscle Actin (+), Sarcomeric
Actin (HHF-35) (+), S-100 protein (+)]. A few scattered cells
were also positive for HMB-45. In our case, patient’s age, anatomical
localization and type of neoplasm are unfavorable survival factors,
according to clinical data 4 . Our patient was alive six months
after extensive surgical therapy. Pleomorphic RMS once considered
being a relatively common entity (18-20% of all soft tissue sarcomas)
has been virtually ignored in the literature and in pathology
practice for almost 20 years. The diagnostic criteria for pleomorphic
RMS (clinical, light microscopy, immunohistochemistry, electron
microscopy) have beeen recently reevaluated, and the biologic
significance and clinical relevance reestimated.
Key words: Pleomorphic, sarcoma, rhabdomyosarcoma, vulva,
adulthood.
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