Rhabdomyosarcoma, Spindle Cell/Sclerosing Variant: A Clinical and Histopathological Examination of this Rare Variant with Three New Cases from the Oral Cavity

Abstract Spindle cell/sclerosing rhabdomyosarcoma (S-ScRMS) was recently recognized in 2013 by the World Health Organization (WHO) as a stand-alone entity (Parham et al., WHO classification of tumours of soft tissue and bone, IARC Press, Lyon, 2013). Historically, the spindle cell and sclerosing var...
Ausführliche Beschreibung

Abstract Spindle cell/sclerosing rhabdomyosarcoma (S-ScRMS) was recently recognized in 2013 by the World Health Organization (WHO) as a stand-alone entity (Parham et al., WHO classification of tumours of soft tissue and bone, IARC Press, Lyon, 2013). Historically, the spindle cell and sclerosing variants were subcategorized under embryonal type rhabdomyosarcoma. Current data supports that certain S-ScRMS cases have a more aggressive clinical course with reduction of long-term survival, and those found in the head and neck region often exhibit extensive local recurrence. Furthermore, due to variable histopathologic appearances and immunohistochemical findings, misdiagnosis is common. We aim to report the variability in histopathologic patterns, immunohistochemical findings, radiographic features, and clinical data on three new cases of S-ScRMS presenting in the oral cavity of young adult males (ages 22, 24, and 39 years). The English-language literature on S-ScRMS is briefly reviewed as well. Ausführliche Beschreibung