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 J Fritchie2. 1. 1 Boston Children's Hospital, Boston, MA, USA. 2. 2 Mayo Clinic, Rochester, MN, USA. 3. 3 University of Iowa, Iowa City, IA, USA. 4. 4 University of California San Francisco, San Francisco, CA, USA. 5. 5 Robert J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH, USA. 6. 6 Douglass Hanly Moir Pathology, Sydney, New South Wales, Australia. 7. 7 Sarcoma Unit, Royal Marsden Hospital, London, UK. 8. 8 St. Jude Children's Research Hospital, Memphis, TN, USA.
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.
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.
Entities:
Keywords:
DSRCT; desmoplastic small round cell tumor; sarcoma
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