SclerosingRhabdomyosarcoma: Case Report and Review of Literature
Journal Title: Saudi Journal of Pathology and Microbiology - Year 2018, Vol 3, Issue 11
Abstract
Sclerosingrhabdomyosarcoma (SRMS) is a very rare subtype of rhabdomyosarcoma (RMS) and can lead to diagnostic difficulties especially if one is not advised of this variant. This entity was first described in 2000 by Mentzel and Katenkamp.Morphologically, it is characterized by an abundant hyalinizedstroma that may erroneously lead to the diagnosis of extraskeletalchondrosarcoma, sometimes this stroma simulates a primary osteoid or vascular tumor. Thus, it can lead to confusion with osteosarcoma or angiosarcoma. We report here a case of SRMS mimicking a sclerosingepithelioidfibrosarcoma appearing in calves in a 55-year-old man. The tumor cells were arranged in nests, cords with sometimes a pseudovascular features. Immunostaining showed that the tumor was positive for Desmin, SMA and MyoD1, focally positive for myogenin and negative for CK, P63, EMA, S-100, H-caldesmon, CD34, CD31. Based on morphological discovery and immunostaining, he was diagnosed as an SRMS. This is the first case of SRMS to show a strong and diffuse α-SMA highlighting the risk of misdiagnosis as leiomyosarcoma.
Authors and Affiliations
Mohamed Amine Essaoudi, Mustapha Azzakhmam, Mohamed Allaoui, Mohamed Reda EL Ochi, Abderrahim EL Ktaibi
Keywords
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