Sclerosing rhabdomyosarcoma of a chest wall in an adult: a case report and review of the literature

Ann Thorac Cardiovasc Surg. 2014:20 Suppl:642-5. doi: 10.5761/ Epub 2013 Oct 3.


Sclerosing rhabdomyosarcoma (SRMS) is a newly recognized and rare variant of rhabdomyosarcoma. This soft tissue tumor has not yet been reported as a thoracic lesion. We report a case of a 26-year-old woman who presented with a large chest wall tumor. The tumor originated from the right anterior chest wall and protruded into the intra- and extrapleural cavity. A transcutaneous needle biopsy revealed spindle cells in an abundant hyalinized and fibrous stroma. Although the tumor was considered as a malignant soft-tissue neoplasm, a definitive diagnosis could not be established. A wide excision of the chest wall including the second, third and fourth rib and a part of sternum was performed. Histologically, cytoplasmic cross-striations were found in a portion of the tumor cells. The tumor cells were positive for muscle markers, and the tumor was diagnosed as rhabdomyosarcoma consistent with a sclerosing type of rhabdomyosarcoma. Eighteen months after the complete resection, the patient has pleural disseminations but is alive and undergoing chemotherapy. This case highlights the histologic features of a rare form of rhabdomyosarcoma, and emphasizes the importance of awareness of its existence and the utility of skeletal muscle markers in distinguishing sclerosing rhabdomyosarcoma from its mimics.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Biomarkers, Tumor / analysis
  • Biopsy
  • Female
  • Humans
  • Immunohistochemistry
  • Neoplasm Invasiveness
  • Osteotomy
  • Pleural Neoplasms / drug therapy
  • Pleural Neoplasms / secondary*
  • Rhabdomyosarcoma / chemistry
  • Rhabdomyosarcoma / secondary*
  • Rhabdomyosarcoma / surgery
  • Sclerosis
  • Sternotomy
  • Thoracic Neoplasms / chemistry
  • Thoracic Neoplasms / pathology*
  • Thoracic Neoplasms / surgery
  • Thoracic Wall / chemistry
  • Thoracic Wall / pathology*
  • Thoracic Wall / surgery
  • Thoracotomy
  • Time Factors
  • Tomography, X-Ray Computed
  • Treatment Outcome
  • Tumor Burden


  • Biomarkers, Tumor