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- Title
Desmoplastic Small Round Cell Tumors With Atypical Presentations: A Report of 34 Cases.
- Authors
Al-Ibraheemi, Alyaa; Broehm, Cory; Tanas, Munir R.; Horvai, Andrew E.; Rubin, Brian P.; Cheah, Alison L.; Thway, Khin; Fisher, Cyril; Bahrami, Armita; Folpe, Andrew L.; Fritchie, Karen J.
- Abstract
Objectives. Desmoplastic small round cell tumor (DSRCT) is an aggressive round cell sarcoma that arises in the abdominal cavity/pelvis of young males. We sought to expand its clinicopathologic spectrum. Methods. Cases of DSRCT presenting in patients >30 years of age or tumors arising outside of the abdominal cavity/pelvis were retrieved. Results. Thirty-four cases were identified. Sixteen tumors arose at atypical sites (head/neck, intracranial, thigh, axilla/shoulder, inguinal/paratesticular, intraosseous, and uterine corpus). The remaining 18 patients were older than 30 years, and their tumors involved the abdomen or pelvis. The majority of cases showed areas with classic histology, while 6 cases exhibited solid growth and 5 showed macronodular architecture. Cytologic appearance included round cell, rhabdoid, epithelioid, and small cell. Conclusion. DSRCT may arise at nonabdominal locations in both pediatric and adult populations, as well as intra-abdominally in older adults, and these tumors exhibit high rates of metastasis and morbidity.
- Subjects
DESMOPLASTIC small round cell tumor; CASE studies; ABDOMINAL cancer; CANCER cytopathology; METASTASIS
- Publication
International Journal of Surgical Pathology, 2019, Vol 27, Issue 3, p236
- ISSN
1066-8969
- Publication type
Article
- DOI
10.1177/1066896918817140