Myogenic dedifferentiation is associated with poor outcomes in retroperitoneal dedifferentiated liposarcomas

Abstract

Sarcomas are a heterogenous group of malignant tumors with origin or mesenchymal differentiation, they comprise 1–2% of all solid tumors. Retroperitoneum is the second most frequent site affected. Prognosis is worse compared to the limbs, with a 5y OS of 36–58%, and 50–60% patients will relapse. Dedifferentiated liposarcomas (ddLPS) are more aggressive, it is known that presence of a de-differentiated component increases the probability of distant recurrence and lowers OS. There is little information about the specific impact of each type of de-differentiation. To determine if the presence of myogenic differentiation markers in DDLPS is an adverse prognostic factor. A retrospective, observational, analytic cohort study was performed. Cases identified from the electronic clinical files from the National Cancer Institute in Mexico City, we included cases from January 1st 2005 to December 31st 2016. We correlated the presence of expression of myogenic markers (Smooth muscle actin, Calponin, H-caldesmon, Desmin and Myogenin) in the dedifferentiated component of DDLPS with overall survival and surgical outcomes. One hundred and forty-three cases were analyzed. Eighty-two were liposarcomas, and 38 had a dedifferentiated component. Of these 38 cases, 21(55.3%) were males and, 17(44.7%) were females. Median age was 54.1(27–79) years, median tumor size was 28 cm (13–56). Most patients had locally advanced disease: 32(84.2%) were in stage IIIB. 2.6% had metastatic disease and 5(13.2%) had stage Ib at diagnosis. Myogenic marker expression was found in 18.4% of cases; these patients had a worse median survival than cases with no myogenic expression: 18 months (95% CI 15.4–20.5) vs 32 months (95% CI 21.8–42.1) p = 0.01, we also found a relation with higher postoperative morbidity in these cases ( p = 0.045). The presence of myogenic differentiation markers might be associated with a worse prognosis, in our series it corelated with worse OS, however it is not a common event. Relation with surgical morbidity is to be analyzed in further studies.