title: Pleomorphic rhabdomyosarcoma with an impressive response to chemotherapy: case report and review of the literature.
abstract: OBJECTIVE
  Pleomorphic rhabdomyosarcoma (RMS) represents a rare sarcoma subtype of the adult population. Due to its clinical characteristics, treatment is currently based on the guidelines for adult soft tissue sarcoma therapy. Hence, in the metastatic setting, doxorubicin-containing regimens are used in the sarcoma community, with limited treatment benefit. Scanty published data are available on the efficacy of systemic treatment. Whether treatment response and outcome of these patients could be improved by using pediatric protocols used typically in other RMS subtypes, like embryonal and alveolar RMS, is unclear. We report on an impressive effect of multiagent pediatric chemotherapy in an adult patient with metastatic pleomorphic RMS.
  
  
  METHODS
  We present the case of a 70-year-old man with metastatic pleomorphic RMS of his left thigh. Systemic chemotherapy according to the VAC regimen (vincristine, actinomycin, cyclophosphamide) was initiated. Follow-up clinical and radiologic assessment demonstrated an impressive treatment response.
  
  
  RESULTS
  Sixteen months after primary diagnosis, computed tomography scan shows no signs of tumor progression.
  
  
  CONCLUSIONS
  Our case report emphasizes that multiagent systemic therapy according to pediatric protocols should be considered in adult patients with pleomorphic RMS.
authors.affiliation:  Department of Medical Oncology, Inselspital, Bern University Hospital, Sarcoma Center, Bern - Switzerland.
authors.forename: Attila
authors.initials: A
authors.lastname: Kollár
authors.affiliation:  Institute of Pathology, University of Bern, Bern - Switzerland.
authors.forename: Rupert
authors.initials: R
authors.lastname: Langer
authors.affiliation:  Department of Radiation Oncology, Inselspital, Bern University Hospital, Sarcoma Center, Bern - Switzerland.
authors.forename: Codruta
authors.initials: C
authors.lastname: Ionescu
authors.affiliation:  Institute for Diagnostic, Interventional, and Pediatric Radiology, Inselspital, University Hospital Bern, Bern - Switzerland.
authors.forename: Jennifer L
authors.initials: JL
authors.lastname: Cullmann
authors.affiliation:  Department of Orthopedic Surgery, Inselspital, Bern University Hospital, Sarcoma Center, Bern - Switzerland.
authors.forename: Frank M
authors.initials: FM
authors.lastname: Klenke
mesh_terms: D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001932:Brain Neoplasms / Q000556:secondary / Q000628:therapy; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008297:Male; D009367:Neoplasm Staging; D012208:Rhabdomyosarcoma / Q000175:diagnosis* / Q000628:therapy*; D016896:Treatment Outcome; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide