Related Experiment Video
Updated: Jul 1, 2026
12:45
Robot-assisted Total Mesorectal Excision and Lateral Pelvic Lymph Node Dissection for Locally Advanced Middle-low Rectal Cancer
Published on: February 12, 2022
Intra-abdominal desmoplastic small round cell tumor
J D Nathan1, C Gingalewsik, R R Salem
1Department of Surgery, Yale University, School of Medicine, New Haven, Connecticut, USA. nata002@mc.duke.edu
The Yale Journal of Biology and Medicine
|July 25, 2000
Summary
Desmoplastic small round cell tumor is a rare, aggressive cancer primarily affecting young males. Despite aggressive chemotherapy and surgery, complete tumor resection is rarely achieved, highlighting the poor prognosis.
Area of Science:
- Oncology
- Surgical Pathology
- Medical Malignancies
Background:
- Intra-abdominal desmoplastic small round cell tumor (DSRCT) is a rare malignancy in young males.
- DSRCT exhibits unique histological and immunocytochemical features, differentiating it from other small round cell tumors.
- The tumor's aggressive spread, chemotherapy resistance, and challenging resectability contribute to a poor prognosis.
Observation:
- A 39-year-old male presented with diffuse abdominal and pelvic DSRCT.
- Computed-tomography (CT)-guided biopsy confirmed the diagnosis.
- Histology revealed malignant cells in a desmoplastic stroma with positive epithelial, mesenchymal, and neural markers.
Findings:
- The patient received neoadjuvant chemotherapy with a high-dose alkylator-based regimen.
- Chemotherapy resulted in a 10-15% tumor mass regression.
- Surgery revealed extensive omental, colonic, and pelvic masses, with diaphragmatic, peritoneal, and mesenteric involvement, precluding complete resection.
Implications:
- DSRCT remains an aggressive malignancy with a very poor prognosis.
- While chemotherapy may induce partial response, complete surgical resection is seldom achievable.
- Surgical interventions are often palliative in managing advanced DSRCT.
