title: Clear cell sarcoma of soft tissue: a retrospective review and analysis of 31 cases treated at Istituto Ortopedico Rizzoli.
abstract: BACKGROUND
  Clear cell sarcoma (CCS) of soft tissue is a rare melanocytic soft tissue sarcoma with different cytogenetic and natural history than that of melanoma. Objective of this study was to determine outcome predictors in patients treated in our Institute. This objective included the effectiveness of surgical intervention and disease progression after surgery.
  
  
  METHODS
  Thirty-one patients were diagnosed at our institute with clear cell sarcoma through tissue pathology and immunohistochemistry. Patients received multimodality treatment (surgery, radiotherapy and chemotherapy). Five-year survival rates and prognostic predictors were determined.
  
  
  RESULTS
  Sixteen patients were males and 15 females with a median age of 37 years (8-72-years). Twenty-eight tumors were located in extremities and 3 in the trunk area. Eight patients had metastases at their first presentation (6 local lymph nodes and 2 pulmonary metastases). Five and ten-year disease-specific survival rates were 56% and 41%. Two-year disease-specific survival rates for lymph node and pulmonary metastasis groups were 40% and 0%. All metastatic patients died within 5 years follow-up. Five and ten-year disease-specific survival rates for localized tumor cases were 72% and 53%. Male gender, less than 30-years of age, trunk tumor location and size greater than 5 cm were poor prognostic factors according to univariate analysis. Tumor location in the trunk was the only negative prognostic determinant in multivariate model.
  
  
  CONCLUSIONS
  Although surgical treatment may be beneficial for tumors without systemic involvement, new chemotherapeutic agents and molecular targeted therapy should be implemented to improve the oncologic outcome in both early and late stage disease.
authors.affiliation: III Clinica Ortopedica, Istituto Ortopedico Rizzoli, Via Pupilli 1, Bologna, Italy. Electronic address: giuseppe.bianchi@ior.it.
authors.forename: G
authors.initials: G
authors.lastname: Bianchi
authors.affiliation: III Clinica Ortopedica, Istituto Ortopedico Rizzoli, Via Pupilli 1, Bologna, Italy. Electronic address: krischaroenlap@hotmail.com.
authors.forename: C
authors.initials: C
authors.lastname: Charoenlap
authors.affiliation: Department of Pathology, Istituto Ortopedico Rizzoli, Via Pupilli 1, Bologna, Italy. Electronic address: stefania.cocchi@ior.it.
authors.forename: S
authors.initials: S
authors.lastname: Cocchi
authors.affiliation: III Clinica Ortopedica, Istituto Ortopedico Rizzoli, Via Pupilli 1, Bologna, Italy. Electronic address: nicola.rani@ior.i.
authors.forename: N
authors.initials: N
authors.lastname: Rani
authors.affiliation: III Clinica Ortopedica, Istituto Ortopedico Rizzoli, Via Pupilli 1, Bologna, Italy. Electronic address: silvia.campagnoni@ior.it.
authors.forename: S
authors.initials: S
authors.lastname: Campagnoni
authors.affiliation: Department of Pathology, Istituto Ortopedico Rizzoli, Via Pupilli 1, Bologna, Italy. Electronic address: alberto.righi@ior.it.
authors.forename: A
authors.initials: A
authors.lastname: Righi
authors.affiliation: III Clinica Ortopedica, Istituto Ortopedico Rizzoli, Via Pupilli 1, Bologna, Italy. Electronic address: tommaso.frisoni@ior.it.
authors.forename: T
authors.initials: T
authors.lastname: Frisoni
authors.affiliation: III Clinica Ortopedica, Istituto Ortopedico Rizzoli, Via Pupilli 1, Bologna, Italy. Electronic address: davide.donati@ior.it.
authors.forename: D M
authors.initials: DM
authors.lastname: Donati
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D002648:Child; D003131:Combined Modality Therapy; D018450:Disease Progression; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000556:secondary; D008198:Lymph Nodes / Q000473:pathology; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009364:Neoplasm Recurrence, Local; D016016:Proportional Hazards Models; D011878:Radiotherapy; D012189:Retrospective Studies; D018227:Sarcoma, Clear Cell / Q000401:mortality / Q000556:secondary / Q000628:therapy*; D012737:Sex Factors; D012983:Soft Tissue Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000970:Antineoplastic Agents
keywords: Clear cell sarcoma of soft tissue; Clear cell sarcoma of tendon and aponeurosis; Melanoma of soft parts; Sarcoma; Soft tissue