Desmoplastic Small Round Cell Tumors With Atypical Presentations: A Report of 34 Cases
| العنوان: | Desmoplastic Small Round Cell Tumors With Atypical Presentations: A Report of 34 Cases |
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| المؤلفون: | Andrew E. Horvai, Karen J. Fritchie, Munir R. Tanas, Khin Thway, Cory Broehm, Cyril Fisher, Armita Bahrami, Brian P. Rubin, Alyaa Al-Ibraheemi, Alison L. Cheah, Andrew L. Folpe |
| المصدر: | International Journal of Surgical Pathology. 27:236-243 |
| بيانات النشر: | SAGE Publications, 2018. |
| سنة النشر: | 2018 |
| مصطلحات موضوعية: | Adult, Male, 0301 basic medicine, Pathology, medicine.medical_specialty, Adolescent, Oncogene Proteins, Fusion, Desmoplastic small-round-cell tumor, Abdominal cavity, Desmoplastic Small Round Cell Tumor, Round cell sarcoma, Pathology and Forensic Medicine, Cohort Studies, Young Adult, 03 medical and health sciences, Sex Factors, 0302 clinical medicine, Round cell, Humans, Medicine, Child, WT1 Proteins, Young male, Pelvis, business.industry, Middle Aged, medicine.disease, 030104 developmental biology, medicine.anatomical_structure, Child, Preschool, 030220 oncology & carcinogenesis, Female, Surgery, Sarcoma, RNA-Binding Protein EWS, Anatomy, business |
| الوصف: | 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. |
| تدمد: | 1940-2465 1066-8969 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5cbb869bf260053ddd8ba58c642e7d2fTest https://doi.org/10.1177/1066896918817140Test |
| حقوق: | CLOSED |
| رقم الانضمام: | edsair.doi.dedup.....5cbb869bf260053ddd8ba58c642e7d2f |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 19402465 10668969 |
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