Histiocytic sarcoma is an extremely rare and very aggressive malignancy, with poor prognosis. The cases described in the literature are few and the treatment is not currently considered consensual. The clinical presentation is very variable. Its characterization is made based primarily on the histology. The authors present a case of an 82-year-old woman, with multiple adenopatic retroperitoneal and left iliac conglomerates, with left leg associated edema (extrinsic compression by conglomerates). After intensive study and approach ganglion biopsy, the histologic diagnosis revealed a histiocytic sarcoma.
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