BACKGROUND AND OBJECTIVES: 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.

Soft tissue myxofibrosarcoma: A clinico-pathological analysis of a series of 75 patients with emphasis on the epithelioid variant / Scoccianti, Guido; Ranucci, Valentina; Frenos, Filippo; Greto, Daniela; Beltrami, Giovanni; Capanna, Rodolfo; Franchi, Alessandro. - In: JOURNAL OF SURGICAL ONCOLOGY. - ISSN 0022-4790. - STAMPA. - 114:(2016), pp. 50-55. [10.1002/jso.24250]

Soft tissue myxofibrosarcoma: A clinico-pathological analysis of a series of 75 patients with emphasis on the epithelioid variant

FRENOS, FILIPPO;GRETO, DANIELA;BELTRAMI, GIOVANNI;CAPANNA, RODOLFO;FRANCHI, ALESSANDRO
2016

Abstract

BACKGROUND AND OBJECTIVES: 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.
2016
114
50
55
Scoccianti, Guido; Ranucci, Valentina; Frenos, Filippo; Greto, Daniela; Beltrami, Giovanni; Capanna, Rodolfo; Franchi, Alessandro
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Utilizza questo identificatore per citare o creare un link a questa risorsa: https://hdl.handle.net/2158/1053372
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