Desmoplastic Small Round Cell Tumors With Atypical Presentations: A Report of 34 Cases

International Journal of Surgical Pathology - Tập 27 Số 3 - Trang 236-243 - 2019
Alyaa Al‐Ibraheemi1, Cory Broehm2, Munir R. Tanas3, Andrew E. Horvai4, Brian P. Rubin5, Alison L. Cheah6, Khin Thway7, Cyril Fisher7, Armita Bahrami8, Andrew L. Folpe2, Karen Fritchie2
1Boston Children’s Hospital Boston MA USA
2Mayo Clinic, Rochester, MN USA
3University of Iowa, Iowa City, IA, USA;
4University of California, San Francisco; San Francisco; CA; USA
5Robert J Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH, USA
6Douglass Hanly Moir Pathology, Sydney, New South Wales, Australia
7Sarcoma Unit, Royal Marsden Hospital, London, UK
8St. Jude Children's Research Hospital, Memphis, TN USA

Tóm tắt

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.

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Thông tin
Thông tin xuất bản

International Journal of Surgical Pathology

Tập 27 Số 3

236-243

Thông tin tác giả