Rare Pediatric Tumors

Pater, Luke; Breneman, John C.

doi:10.1016/b978-0-323-24098-7.00075-7

Cited by 1 publication

(1 citation statement)

References 203 publications

(139 reference statements)

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“…DSRCTs were identified and characterized by Gerald and Rosai in 1989 [9]. The mean age of diagnosis is approximately between 19 and 22 years, ranging from 6 to 49 years, with a male predominance of 75% [5,10]. In accordance, the present case was a male in his forties.…”

Section: Discussionsupporting

confidence: 79%

Desmoplastic Small Round Cell Tumor: A Case Report

Rebaz Haji Ali,

Shano M. Ali,

Rawa M. Ali

et al. 2023

Barw Medical Journal

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Introduction Desmoplastic small round cell tumor (DSRCT) is an ultra-rare and highly aggressive mesenchymal tumor that primarily affects the peritoneum, and to a lesser extent, the visceral organs in the abdominal cavity. This study presents a case of a patient who has been diagnosed with DSRCT and has been managed with VAC-IE chemotherapy. Case presentation A 43-year-old male presented with a complaint of epigastric pain for 20 days but no other clinical symptoms. A CT scan and an abdominal ultrasound showed multiple lesions in the peritoneum, and biopsies were taken from all the sites. Histological analysis revealed nests of small blue round cells surrounded by an abundant stroma. Immunohistochemistry results confirmed the disease to be a desmoplastic small round cell tumor. Conclusion This disease is yet to be fully understood, and the response of our case to VAC-IE chemotherapy has shown promising outcomes. This form of treatment can be further studied to establish and help in finding a treatment with a better prognosis.

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Section: Discussionsupporting

confidence: 79%