Guido Scoccianti1, Valentina Ranucci2, Filippo Frenos1, Daniela Greto3, Giovanni Beltrami1, Rodolfo Capanna1, Alessandro Franchi4. 1. Department of Orthopaedic Oncology, Careggi University-Hospital, Florence, Italy. 2. Department of Anatomic Pathology, Catholic University, Rome, Italy. 3. Department of Radiotherapy, Careggi University-Hospital, Florence, Italy. 4. Department of Surgery and Translational Medicine, Section of Anatomic Pathology, University of Florence, Florence, Italy.
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. J. Surg. Oncol. 2016;114:50-55.
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. J. Surg. Oncol. 2016;114:50-55.
Authors: Juan Pablo Zumárraga; Felipe Augusto Ribeiro Batista; André Mathias Baptista; Marcelo Tadeu Caiero; Luis Pablo de la Rosa Martino; Olavo Pires de Camargo Journal: Acta Ortop Bras Date: 2018 Impact factor: 0.513
Authors: Jan Marie de Gooyer; Yvonne M H Versleijen-Jonkers; Melissa H S Hillebrandt-Roeffen; Cathelijne Frielink; Ingrid M E Desar; Johannes H W de Wilt; Uta Flucke; Mark Rijpkema Journal: Sci Rep Date: 2020-02-19 Impact factor: 4.379