Desmoplastic small round cell tumor: the report of two cases and literature analysis review of the radiological findings
Wenying Chen, Haimei Chen, Chang Zhao, Sidong Xie, Jie Qin, Weimin Liu
Abstract
BackgroundDesmoplastic small round cell tumor (DSRCT) is a rare, unique, and highly malignant tumor that tends to occur in children and adolescents.There is an obvious gender preference, with a male to female ratio reaching 5 to 1 (1,2).It occurs predominantly in the abdominal pelvic cavity, spreads along the peritoneal surface, and very rarely appears in the lungs (3), salivary glands (4), heart (5), colon (6), bones (7) and gonads (8,9).DSRCT has an occult onset and rapid disease progression and is susceptible to implantable spread and blood and lymphatic metastasis, which most often occurs in the liver, followed by the lungs, lymph nodes, and bones (10).In this study, we retrospectively analyzed the two cases of patients with DSRCT admitted to our hospital from October 2016 to October 2021 and referred to the relevant literature in recent years, with the aim of improving the understanding of their clinical and imaging characteristics. Case presentation Clinical historyCase 1 A 9-year-old boy touched a mass in his abdomen 2 weeks prior to attending The Third Affiliated hospital of Sun Yat-sen University but complained of no other discomfort.Multiphase contrast-enhanced computed tomography (CT; Toshiba CT Aquilion; 64 slices; slice thickness, 3 mm) of the abdomen revealed a large mass in the left kidney, measuring approximately 129 mm × 95 mm × 161 mm, with heterogeneous density and multiple calcifications within it, showing significant heterogeneous enhancement.There were also multiple metastases in the abdominal lymph nodes and the liver (Figure 1).Thereafter, the patient also underwent whole-body fluorine 18 fluorodeoxyglucose positron emission tomography-computed tomography ( 18 F-FDG PET-CT), which showed a large left renal