Removal of an intra-abdominal desmoplastic small round cell tumor by repetitive debulking surgery: A case report and literature review

Abstract

In the current study, a case of recurrent desmoplastic small round cell tumor (DSRCT) is presented, which was successfully treated by repetitive debulking surgery. In May 2010, a 39-year-old male, with a history of surgical resection of intra-abdominal DSRCT, visited the Ibaraki Medical Center, Tokyo Medical University Hospital (Ami, Japan) with severe lower abdominal discomfort. Abdominal computed tomography revealed a large tumor in the pouch of Douglas with a small number of nodules in the abdominal cavity. The recurrent DSRCT was diagnosed and removed via lower anterior resection; however, complete resection was impossible due to multiple peritoneal metastases. One year later, the patient developed pain in the right groin due to the growth of metastasized tumor cells in the groin lymph nodes. The affected lymph nodes were removed utilizing an extra-peritoneal approach. At the time of writing, the patient continues to survive without any symptoms 60 months since the initial surgery. In conclusion, surgical debulking is a significant procedure for relieving patient symptoms as well as improving the survival time of patients with metastatic and recurrent DSRCT.

Mediastinal desmoplastic small round cell tumor

“…This suggested that effective cytoreduction and R0 resection would be beneficial to early-stage patients with no distant metastasis. [ 26 , 27 ] Some researchers [ 28 , 29 ] have also found that a combination of neoadjuvant chemotherapy and surgery not only brings patients an opportunity of radical surgery, it is also of certain significance for patients who can tolerate secondary surgery by adopting high-intensity radiochemotherapy combined with bone marrow transplantation. Li Gang et al [ 4 ] reviewed 5 patients with DSRCT primarily occurred in the chest.…”