Fetal Type Rhabdomyoma of the Soft Palate in an Adult Patient: Report of One Case and Review of the Literature

Head Neck Pathol. 2019 Jun;13(2):182-187. doi: 10.1007/s12105-018-0931-5. Epub 2018 May 14.

Abstract

Rhabdomyoma is a rare benign tumor with skeletal muscle differentiation. Rhabdomyoma is further classified into cardiac, adult, fetal, and genital subtypes. Out of these, fetal type rhabdomyoma (FTR) is the rarest. Only a small number of cases have been recorded in the literature. FTR typically affects male infants and young children and occurs predominantly in the head and neck region. FTR is exceedingly rare in the adult, with less than 30 cases reported. The classic FTR is composed of primitive undifferentiated spindle cells with scant eosinophilic cytoplasm embedded in a myxoid stroma. Immunohistochemically, the tumor cells are positive for desmin, muscle specific actin, and myogenin. Awareness and proper recognition of this rare entity is of considerable importance to avoid misdiagnosis of embryonal rhabdomyosarcoma. In this study, we report one case of FTR in an adult patient and reviewed the literature about the clinical and pathologic presentation of FTR in the adult.

Keywords: Benign spindle cell tumor; Rhabdomyoma; Rhabdomyosarcoma.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Female
  • Humans
  • Mouth Neoplasms / pathology*
  • Palate, Soft / pathology*
  • Rhabdomyosarcoma, Embryonal / pathology*