Contributed by Hongbo Yu, MD, PhD, Antonia Sepulveda, MD, PhD and Uma Rao, MD
Published on line in March 2002
This 60-year-old woman presents with history of marked abdominal distention lasted for several months with associated progressive fatigue, progressive weight loss and fever. CT scan revealed an extraordinarily large retroperitoneal mass occupying almost the entire abdominal cavity extending below and anterior to the liver. It surrounds the right kidney which is displaced anteriorly and towards the midline. A moderate amount of hydronephrosis of right kidney is noted. A needle core biopsy of the mass was signed out as myxoid spindle cell sarcoma with necrosis. Immunostains were negative for desmin, cytokeratin, and S100.
The specimen consists of a 45.0 x 34.5 x 14.0 cm multinodular mass with the weight of 20 pounds. On cross section, nodules have approximately four different appearances: soft yellow; gelatinous tan yellow with necrosis; rubbery white without necrosis; and firm, whorled, white with necrosis. The largest nodule measures 15.0 x 14.5 x 11.0 cm. The tumor surrounds the right kidney without direct invasion.