Combined Sclerosing and Spindle Cell Rhabdomyosarcoma in Previous Craniotomy Site: A Case Report and a Review of the Literature

Int J Surg Pathol. 2019 May;27(3):328-335. doi: 10.1177/1066896918802030. Epub 2018 Oct 1.


Sclerosing rhabdomyosarcoma (RMS) is a rare subtype of RMS with unique prominent stromal hyalinization and a pseudovascular architecture. It overlaps morphologically with spindle cell RMS and poses both diagnostic and therapeutic challenges because of its rarity and aggressive clinical course. In this article, we report a case of sclerosing RMS arising from a prior craniotomy site, which demonstrated both sclerosing and spindle cell components. A literature review of RMS with sclerosing morphology identified 122 cases. Our review documents the following: sclerosing RMS occurs in both childhood and adult populations, has a predilection for the head and neck areas, and has a worse prognosis in adults. Sclerosing RMS harbors a high frequency of MYOD1 mutations, conferring a poor clinical outcome. Sclerosing RMS and spindle RMS likely represent a morphologic spectrum of one entity.

Keywords: pseudovascular; sclerosing rhabdomyosarcoma; soft tissue; spindle cell rhabdomyosarcoma; stromal hyalinization.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aneurysm, Ruptured / etiology
  • Aneurysm, Ruptured / surgery
  • Biopsy
  • Brain / diagnostic imaging
  • Brain / pathology
  • Chemoradiotherapy, Adjuvant
  • Craniotomy / adverse effects*
  • Diagnosis, Differential
  • Head and Neck Neoplasms / diagnostic imaging
  • Head and Neck Neoplasms / etiology
  • Head and Neck Neoplasms / pathology*
  • Head and Neck Neoplasms / therapy
  • Humans
  • Intracranial Aneurysm / complications
  • Male
  • Middle Aged
  • Muscle Neoplasms / diagnostic imaging
  • Muscle Neoplasms / etiology
  • Muscle Neoplasms / pathology*
  • Muscle Neoplasms / therapy
  • Rhabdomyosarcoma / diagnostic imaging
  • Rhabdomyosarcoma / etiology
  • Rhabdomyosarcoma / pathology*
  • Rhabdomyosarcoma / therapy
  • Scalp
  • Sclerosis
  • Subcutaneous Tissue / diagnostic imaging
  • Subcutaneous Tissue / pathology*
  • Tomography, X-Ray Computed