title: Soft tissue myxofibrosarcoma: A clinico-pathological analysis of a series of 75 patients with emphasis on the epithelioid variant.
abstract: OBJECTIVE
  The clinical course of soft tissue myxofibrosarcoma is characterized by a high incidence of recurrences and there is no agreement on how to identify patients at major risk. An epithelioid histological variant has been described, with a possible worse prognosis. We reviewed our series to identify prognostic factors and assess clinical significance of the epithelioid variant.
  
  
  METHODS
  We examined the clinico-pathological features of a series of 75 patients affected by soft tissue myxofibrosarcoma at a mean follow-up of 63 months (range 17-132).
  
  
  RESULTS
  Disease specific survival and local recurrence free survival were, respectively, 84.8% and 76.8% at 5 years. Seven patients (8.6%) presented with the epithelioid variant with a survival of 62.5%. High grade and epithelioid morphology were negative prognostic factors for patient survival, high grade, and inadequate surgical margins for local recurrence. Radiotherapy had a local protective effect in high grade tumors.
  
  
  CONCLUSIONS
  Our experience confirms the difficulties in obtaining wide margins in myxofibrosarcoma and the high rate of recurrence. Local recurrences did not significantly affect survival and a limb-sparing approach can be chosen also in recurrences. Patients affected by the epithelioid variant showed a worse prognosis. Chemotherapy should be considered as adjuvant treatment in this subtype. J. Surg. Oncol. 2016;114:50-55. © 2016 Wiley Periodicals, Inc.
authors.affiliation: Department of Orthopaedic Oncology, Careggi University-Hospital, Florence, Italy.
authors.forename: Guido
authors.initials: G
authors.lastname: Scoccianti
authors.affiliation: Department of Anatomic Pathology, Catholic University, Rome, Italy.
authors.forename: Valentina
authors.initials: V
authors.lastname: Ranucci
authors.affiliation: Department of Orthopaedic Oncology, Careggi University-Hospital, Florence, Italy.
authors.forename: Filippo
authors.initials: F
authors.lastname: Frenos
authors.affiliation: Department of Radiotherapy, Careggi University-Hospital, Florence, Italy.
authors.forename: Daniela
authors.initials: D
authors.lastname: Greto
authors.affiliation: Department of Orthopaedic Oncology, Careggi University-Hospital, Florence, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Beltrami
authors.affiliation: Department of Orthopaedic Oncology, Careggi University-Hospital, Florence, Italy.
authors.forename: Rodolfo
authors.initials: R
authors.lastname: Capanna
authors.affiliation: Department of Surgery and Translational Medicine, Section of Anatomic Pathology, University of Florence, Florence, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Franchi
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000970:Antineoplastic Agents / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D015622:Epithelioid Cells / Q000473:pathology*; D005260:Female; D005354:Fibrosarcoma / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D005500:Follow-Up Studies; D006801:Humans; D023821:Limb Salvage; D008297:Male; D000072662:Margins of Excision; D008875:Middle Aged; D009236:Myxosarcoma / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D020360:Neoadjuvant Therapy; D060787:Neoplasm Grading; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology / Q000209:etiology / Q000517:prevention & control; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D012983:Soft Tissue Neoplasms / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents
keywords: epithelioid variant; histopathology; myxofibrosarcoma; prognosis; soft tissue tumors