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A man in his 20s presented with a 2-month history of vague, right upper-quadrant abdominal pain, with associated 5-kg weight loss, loss of appetite, and increasing abdominal distension. He reported a small mass in the right scrotum that was evaluated 5 years previously and was reassured to be a nonmalignant lesion. Physical examination was notable for a distended abdomen with a palpable left abdominal mass. Testicular examination revealed an approximately 1-cm firm right spermatic cord nodule. Laboratory tests showed lactate dehydrogenase levels of 818 U/L (to convert to μkat/L, multiply by 0.0167), with no significant β-human chorionic gonadotropin and α-fetoprotein levels. Computed tomography (CT) of the abdomen (Figure, A and B) revealed innumerable masses throughout the abdomen and pelvis, with the largest measuring 17.5 × 14 × 14 cm and a 3.9-cm enhancing lesion in the left hepatic lobe, and a small amount of ascites. Testicular sonogram disclosed a 1.5-cm right epididymal cyst. Percutaneous biopsy and histologic examination of the dominant left abdominal mass revealed neoplastic cells (Figure, C) that were positive for cytokeratin, desmin but negative for S100. Fluorescence in situ hybridization (FISH) was positive for EWS-WT1 rearrangement.
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D. Advanced desmoplastic small round cell tumor
Histopathologic analysis revealed islands and nests of uniform small round cells with round hyperchromatic nuclei and minimal cytoplasm embedded in a prominent fibroblastic stroma characteristic of desmoplastic small round cell tumor (DSRCT). Immunoprofile showed varying degrees of epithelial, muscle, and neural marker expressions. Results of FISH yielded pathognomonic EWSR1-WT1 fusion oncogene that results from t(11;22)(p13;q12) translocation.1
Desmoplastic small round cell tumor is a rare and highly fatal soft tissue sarcoma of mesenchymal origin that was first described by Gerald and Rosai in 1989.2 Etiology of DSRCT remains uncertain. The name DSRCT derives from its characteristic histologic findings of clusters of small round blue cells surrounded by abundant desmoplastic stroma. It is a disease of pediatric and young adult patients with peak incidence from 20 to 24 years and is more common in men.3 Patients usually present with nonspecific abdominal symptoms, such as distension, pain, discomfort, and constipation. Most patients have advanced disease at presentation evidenced by imaging findings of large abdominal masses and peritoneal implants with ascites.4
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Corresponding Author: Aung Myint Tun, MD, Department of Medicine, Division of Hematology and Oncology, The Brooklyn Hospital Center, 240 Willoughby St, Ste 5D, Brooklyn, NY 11201 (firstname.lastname@example.org).
Published Online: November 9, 2017. doi:10.1001/jamaoncol.2017.3432
Conflict of Interest Disclosures: None reported.
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