title: [Rhabdomyosarcoma in middle to old-aged patients: analysis of clinicopathological features and prognosis in 76 cases].
abstract: OBJECTIVE
  Albeit rare, rhabdomyosarcoma (RMS) may occur in middle to old-aged adults. The purpose of this study was to investigate the clinicopathological characteristics and prognosis of RMS in patients ≥ 40 years of age.
  
  
  METHODS
  The clinical, pathological and immunohistochemical profiles of the 76 cases were reviewed, and the outcomes were analyzed.
  
  
  RESULTS
  There were 46 males and 30 females with a median age of 55 years. Sixty cases occurred in somatic soft tissues, including extremities (n = 25), head and neck (n = 19) and trunk (n = 16). Sixteen developed in the internal organs with predilection for the urogenital tract. Histologically, 38 cases (50.0%) were of embryonal RMS (ERMS) subtype, 29 cases (38.2%) of pleomorphic RMS (PRMS), 7 cases (9.2%) of poorly-differentiated RMS (PdRMS), and 2 cases (2.6%) of alveolar RMS (ARMS). Immunohistiochemically, the tumor cells showed diffuse staining of desmin (68/68, 100%), with a variable expression of myogenin (30/43, 69.8%), MyoD1 (33/51, 64.7%) and MSA (29/40, 72.5%). The therapy data available in 54 cases showed that three inoperable cases were treated with chemotherapy and radiotherapy, 51 cases underwent local excision, including 10 cases with chemotherapy, 9 cases with radiotherapy, 8 cases with chemotherapy and radiotherapy after surgery, and the remaining 24 cases were not administrered with postoperative adjuvant therapy. The follow-up data available in 54 cases showed that 22 patients were alive with unresectable or recurrent/disease and 32 patients were alive with no evidence of disease. The median disease-free and overall survivals were 6.0 months and 7.0 months, respectively. Twenty-seven patients exhibited progression of disease with recurrence in 8 cases and metastasis in 19 cases. The median interval to progression was 6.0 months.
  
  
  CONCLUSIONS
  The most common subtypes of RMS in middle to old aged patients are ERMS and PRMS. In general, Rhabdomyosarcomas of middle to old aged patients shows a highly aggressive behavior. Patients may benefit from multi-disciplinary therapy.
authors: Department of Pathology, Shanghai Cancer Center, Fudan University, Shanghai 200032, China.
authors: Lin
authors: L
authors: Yu
authors: Jian
authors: J
authors: Wang
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D017024:Chemotherapy, Adjuvant; D003893:Desmin / Q000378:metabolism; D018572:Disease-Free Survival; D005121:Extremities; D005260:Female; D005500:Follow-Up Studies; D006258:Head and Neck Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery; D006801:Humans; D008297:Male; D008875:Middle Aged; D017570:MyoD Protein / Q000378:metabolism; D018008:Myogenin / Q000378:metabolism; D009364:Neoplasm Recurrence, Local; D018714:Radiotherapy, Adjuvant; D012208:Rhabdomyosarcoma / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery; D018232:Rhabdomyosarcoma, Alveolar / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery; D018233:Rhabdomyosarcoma, Embryonal / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery; D012983:Soft Tissue Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery; D015996:Survival Rate
chemical_list: D003893:Desmin; D017570:MyoD Protein; C057363:MyoD1 myogenic differentiation protein; D018008:Myogenin