Intra-abdominal desmoplastic small round cell tumors: CT and FDG-PET/CT findings with histopathological association

Abstract

Desmoplastic small round cell tumors (DSRCTs) are rare and aggressive malignant tumors. The aim of the present study was to analyze computed tomography (CT) and fluorodeoxyglucose positron emission tomography (FDG-PET)/CT imaging features of intra-abdominal desmoplastic DSRCT, and investigate the association of these features with histopathological results. The present study was a retrospective investigation of 4 patients with DSRCT. All patients underwent CT and dynamic CT, and 1 additionally underwent FDG-PET/CT scanning. Following a tumor resection, routine hematoxylin and eosin staining, and immunostaining, were performed and evaluated. Multiple large abdominopelvic masses were identified in all 4 patients; however, no indications of their site of origin were demonstrated. CT revealed soft-tissue masses with patchy foci of hypodense lesions. Contrast-enhanced CT revealed slightly or moderately heterogeneous enhancement of the lesions. Other observations from these patients included calcification (n=2), peritoneal seeding (n=3), hepatic metastasis (n=3), retroperitoneal lymphadenopathy (n=3) and ascites (n=2). FDG-PET/CT revealed multiple nodular increased FDG uptake in the abdominopelvic masses, and in the liver and peritoneum in 1 case. Intra-abdominal DSRCT demonstrated significant diagnostic characteristics on plain and contrast-enhanced CT. Multiple, bulky soft-tissue masses inside the peritoneal cavity, particularly in male adolescents and young adults, should be considered as potential cases of DSRCT. FDG-PET/CT techniques may be utilized to aid the staging of tumors.

Desmoplastic Small Round Blue Cell Tumor: A Review of Treatment and Potential Therapeutic Genomic Alterations

“…Characteristic findings in DSRCT include soft tissue masses which are often bulky (mean, 6 cm; range, 1–28 cm), lobulated, and heterogeneous with hypodense areas; these findings are present in up to three-quarters of patients [ 15 ], and a significant desmoplastic reaction differentiates DSRCTs from other small round cell tumors [ 16 ]. Adenopathies are present in approximately half of patients at the time of diagnosis (intraperitoneal, retroperitoneal, and pelvic) [ 15 , 17 ]. In a radiological review of 13 cases of abdominal DSRCT, the most common finding was the presence of several lobulated peritoneal soft tissue masses (mean number of masses/patient = 4).…”

Primary desmoplastic small round cell tumor of the tibia: PET/CT and MRI presentation of a rare case and review of the literature

“…However, this case is the first that reported the PET/CT imaging features of osseous DSRCT. The tumor mass appears with SUV max of 24.66 and SUV avg of 9.81, similar to the intra-abdominal masses [17,18], indicating that the mass is with high malignance.…”

“…DSRCT showed a higher rate of occurrence on serosal surfaces, where it appeared as single or multiple nodular masses on the surface of intra-abdominal organs. The characteristic imaging feature of DSRCT was multiple soft-tissue masses implicating the peritoneal cavity in the omentum or mesentery without a definite organ of origin as described in the literatures [16,17]. On CT images, tumor mass was usually patchy hypodense and mild heterogeneous enhancement on contrast enhanced images.…”

Tumor desmoplásico de células pequeñas y redondas. Presentación de caso y revisión de la literatura

“…La presentación clínica es inespecífica, se encuentra más frecuentemente dolor abdominal, distensión, cambios en el hábito intestinal y sensación de masa palpable en el abdomen (4,9). Las manifestaciones clínicas suelen estar relacionadas con el sitio anatómico comprometido, por lo que se encuentra en la literatura obstrucción ureteral e hidronefrosis secundaria, obstrucción intestinal, síndrome biliar obstructivo (10) e incluso aumento en la frecuencia miccional por compresión vesical (11).…”