Spindle cell rhabdomyosarcoma: a brief diagnostic review and differential diagnosis

Arch Pathol Lab Med. 2013 Aug;137(8):1155-8. doi: 10.5858/arpa.2012-0465-RS.

Abstract

Spindle cell rhabdomyosarcoma is a rare variant of embryonal rhabdomyosarcoma that has a predilection for young males and most commonly involves the paratesticular region followed by head and neck. Histopathology demonstrates elongated spindle cells with fusiform to cigar-shaped nuclei and indistinct eosinophilic cytoplasm arranged in fascicles or whorls. Although the tumor demonstrates increased cellularity and moderate atypia, the microscopic and architectural patterns can allow this tumor to be confused with multiple entities, such as leiomyosarcoma, spindle cell carcinoma, desmoplastic melanoma, or fibrosarcoma, with important therapeutic implications. Immunohistochemical workup demonstrates sarcomeric differentiation with reactivity for desmin, myogenin, and MyoD1 markers. Compared with other subtypes, the spindle cell variant in children is associated with a favorable outcome; however, in the adult population there does not appear to be any prognostic advantage.

Publication types

  • Review

MeSH terms

  • Adult
  • Child
  • Cytogenetic Analysis
  • Diagnosis, Differential
  • Female
  • Head and Neck Neoplasms / diagnosis
  • Head and Neck Neoplasms / metabolism
  • Head and Neck Neoplasms / therapy
  • Humans
  • Immunohistochemistry
  • Male
  • MyoD Protein / metabolism
  • Prognosis
  • Rhabdomyosarcoma, Embryonal / diagnosis*
  • Rhabdomyosarcoma, Embryonal / metabolism
  • Rhabdomyosarcoma, Embryonal / therapy
  • Testicular Neoplasms / diagnosis
  • Testicular Neoplasms / metabolism
  • Testicular Neoplasms / therapy

Substances

  • MyoD Protein
  • MyoD1 myogenic differentiation protein