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Desmoplastic Small Round Cell Tumor: Imaging Pattern of Disease at Presentation
A C Morani1, T K Bathala1, V R Surabhi2
11 Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Unit 1473, Houston, TX 77030.
AJR. American Journal of Roentgenology
|January 24, 2019
Summary
Desmoplastic small round cell tumor (DSRCT) is a rare peritoneal malignancy primarily affecting young males. This study details its clinical, pathologic, and imaging features, noting frequent abdominal dissemination at diagnosis.
Area of Science:
- Oncology
- Radiology
- Pathology
Background:
- Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive malignancy.
- It predominantly affects young males and presents with widespread peritoneal disease.
Purpose of the Study:
- To evaluate the clinical, pathologic, and cross-sectional imaging features of DSRCT.
- To characterize the presentation and metastatic patterns of DSRCT in a cohort of 94 patients.
Main Methods:
- Retrospective analysis of 94 pathologically verified DSRCT cases.
- Data collection included epidemiologic, clinical, pathologic, and imaging findings.
- Analysis of tumor characteristics, location, and metastatic distribution at presentation.
Main Results:
- DSRCT occurred in young patients (median age 21.5 years), predominantly males (86:8 ratio).
- Most patients (90%) presented with multifocal or diffuse peritoneal disease, including omental, peritoneal, and retroperitoneal involvement.
- Liver metastases were present in 34%, diaphragmatic involvement in 40%, and thoracic findings included nodal disease (35%) and pleural effusions (18%).
Conclusions:
- DSRCT is a rare, multifocal peritoneal malignancy with frequent abdominal dissemination at presentation.
- Omentum, peritoneum, and retroperitoneum are common sites of abdominal involvement.
- Liver is the most common visceral metastatic site, while thoracic involvement is more common in nodes and pleura than lungs.
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