Desmoplastic Small Round Cell Tumor: Report of 2 Cases Treated with Chemotherapy Alone or in Combination with Bevacizumab
A 22-year-old male presented with a 3-month history of intensive backaches and weight loss. Later, he noticed a mass in the hypogastrium region and lower limb lymphedema. It evolved into intestinal semiocclusion.\nOn computed tomography (CT), performed on August 18, 2009, the mass was confirmed to be a retrovesical tumor (fig. ), extending to the umbilical region, with peritoneal implants and the liver capsule (7.0 cm). A laparoscopy with biopsy was done, confirming an unresectable retroperitoneal mass. Histology and IH studies (table ) confirmed the diagnosis of DSRCT.\nOn September 19, 2009, with a performance status (PS) of 3, the patient began chemotherapy [doxorubicin 60 mg/m2 and cisplatin 60 mg/m2 on day (D)1], as available from the public service. On D15, resolution of the bowel obstruction was noted. After the 3rd cycle of chemotherapy, abdominal CT still showed a solid heterogeneous mass, but with a reduction of 42%. After the 8th cycle of chemotherapy, on May 3, 2010, a new CT scan showed stabilization of the lesions; however, they remained inoperable. The patient was referred for outpatient palliative care. On September 30, 2010, he unfortunately progressed to death due to neoplastic cachexia. Since the initial diagnosis, he had had a survival time of 13 months.