Journal article
Desmoplastic Small Round Cell Tumors With Atypical Presentations: A Report of 34 Cases
International journal of surgical pathology, Vol.27(3), pp.236-243
05/2019
DOI: 10.1177/1066896918817140
PMID: 30522375
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.
Details
- Title: Subtitle
- Desmoplastic Small Round Cell Tumors With Atypical Presentations: A Report of 34 Cases
- Creators
- Alyaa Al-Ibraheemi - Boston Children’s Hospital, Boston, MA, USACory Broehm - Mayo Clinic, Rochester, MN, USAMunir R Tanas - University of Iowa, Iowa City, IA, USAAndrew E Horvai - University of California San Francisco, San Francisco, CA, USABrian P Rubin - Robert J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH, USAAlison L Cheah - Douglass Hanly Moir Pathology, Sydney, New South Wales, AustraliaKhin Thway - Sarcoma Unit, Royal Marsden Hospital, London, UKCyril Fisher - Sarcoma Unit, Royal Marsden Hospital, London, UKArmita Bahrami - St. Jude Children’s Research Hospital, Memphis, TN, USAAndrew L Folpe - Mayo Clinic, Rochester, MN, USAKaren J Fritchie - Mayo Clinic, Rochester, MN, USA
- Resource Type
- Journal article
- Publication Details
- International journal of surgical pathology, Vol.27(3), pp.236-243
- DOI
- 10.1177/1066896918817140
- PMID
- 30522375
- ISSN
- 1066-8969
- eISSN
- 1940-2465
- Language
- English
- Date published
- 05/2019
- Academic Unit
- Pathology
- Record Identifier
- 9984047786302771
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