Requirement for novel surgical and clinical protocols for the treatment of desmoplastic small round cell tumor: a report of two cases and a review of the literature

Abstract

Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive neoplasm that was initially described in 1989. DSRCT predominantly affects young men and typically occurs in the intra-abdominal area. The present study describes the cases of two patients with DSRCT. The first patient was a 23-year-old male who presented with abdominal pain in the right flank, coupled with difficulty urinating and bowel dysfunction. The second patient was 12-year-old female who presented with abdominal pain, emesis and loss of appetite. A computed tomography scan of the abdomen revealed the presence of an extensive pelvic mass in each patient, however, a visceral origin was not clearly identifiable in the first patient. In the second patient, a large soft‑tissue tumor was located posterior to the pancreatic tail and the stomach, with no anatomical line visible between the stomach and splenic vein. Ultrasound‑guided biopsy in the first patient and videolaparoscopy in the second patient followed by immunohistochemical analysis clarified the presence of a malignant neoplasm composed of small, blue, round cells. Due to right ureter involvement and hydronephrosis in the first patient, a treatment strategy of surgical debulking of the tumor was selected. The surgical procedure involved en bloc resection of the lesion associated with a pelvic peritonectomy, followed by post-operative radiotherapy. However, the second patient exhibited extensive disease, therefore, a chemotherapeutic protocol of vincristine, doxorubicin and cyclophosphamide, as well as radiation therapy, was scheduled. Disease relapse was observed in the abdominal cavity of the first patient after one year, while the second patient remains asymptomatic. Following analysis of present two cases, it was concluded that aggressive treatment regimens may induce tumor regression. However, relapse of the disease is frequent and long-term survival is rare with the currently available therapeutic strategies.