Desmoplastic Small Round Cell Tumor: Report of 2 Cases Treated with Chemotherapy Alone or in Combination with Bevacizumab
A 37-year-old male presented with a 2-month history of poor digestion and weight loss of 7 kg. On clinical examination, a right abdominal tumor mass was found. Magnetic resonance imaging (MRI) confirmed an abdominal lesion, measuring 18.9 × 16.6 cm, on the periphery of the liver segment VI. A chest CT scan was normal. At exploratory laparotomy, on October 7, 2008, a tumor attached to the liver, invading the splenic hilum, omentum, and mesenteric region was found. Partial resection was possible. Histology and IH studies (table ) were compatible with DSRCT.\nHe started chemotherapy (carboplatin AUC5, paclitaxel 180 mg/m2, and bevacizumab 7.5 mg/kg) on November 18, 2008, for 21/21 days, with a covenant provided by private health insurance. He had PS 3 (73 kg) and changes in liver function. After the 3rd round of chemotherapy, on January 16, 2009, MRI already showed partial remission (78.6% reduction). After the 8th cycle, on May 12, 2009, a presurgical positron emission tomography (PET) reassessment was performed. Interestingly, PET showed physiological distribution of F-18-fluorodeoxyglucose (FDG) without identifying any uptake (fig. , fig. ). A concomitant CT scan showed tumor infiltration of the hilum, hepatic parenchyma, and peritoneal implants (fig. ).\nA new surgical approach was contraindicated. On June 16, 2009, chemotherapy was stopped at the patient's request. Two months after that, there was a clinical and liver function worsening due to tumor progression and compression of the biliary tract. A retrograde endoscopic cholangiopancreatography was done and a stent was successfully placed.\nChemotherapy was restarted on August 26, 2009. He already had new partial remission. This scheme was discontinued after the 10th cycle on March 30, 2010 (4.9-cm tumor) due to more frequent occurrences of neutropenia. Chemotherapy was then initiated with cyclophosphamide 50 mg/day, oral, and bevacizumab; this regimen was maintained until November 16, 2010, when it was suspended because of worsening of the patient's clinical state and laboratory findings. He remained in hospice care until his death on July 22, 2011, due to neoplastic cachexia and hepatic encephalopathy. He had had a survival time of 34 months after the initial diagnosis.