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.
HighlightsThere is no established diagnostic and treatment approach for laryngeal chondrosarcoma due to its low frequency.90% of laryngeal chondrosarcoma are low-grade tumors and therefore conservative surgery is recommended as initial treatment.Percutaneous biopsy could save patients from a of total laryngectomy.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.