Ajaykumar Chandralal Morani MD, Presenter: Nothing to Disclose

Andrea Hayes-Jordan, Abstract Co-Author: Nothing to Disclose

Venkateswar Rao Surabhi MD, Abstract Co-Author: Nothing to Disclose

Tharakeswara Kumar Bathala MD, Abstract Co-Author: Nothing to Disclose

Huh Winston MD, Abstract Co-Author: Nothing to Disclose

Tara Lynn Sagebiel MD, Abstract Co-Author: Nothing to Disclose

Srinivasa R. Prasad MD, Abstract Co-Author: Nothing to Disclose

To evaluate clinico-pathological and multi-modality, cross-sectional imaging features of a cohort of 94 cases of desmoplastic small round cell tumor (DSRCT).  

An IRB-approved, HIPAA-compliant, retrospective study of patients with DSRCT treated at a tertiary cancer center between 2001 & 2013 yielded 94 cases. Epidemiological, clinical and pathological data as well as imaging findings were recorded. Tumor size, location, morphology, and distribution pattern of metastases at the time of presentation, were analyzed.

DSRCT occurred in young patients with a median age of 21.5 years (age range 5-53 years) and a marked male predilection (M:F= 86:8). 89 were white (Caucasian or Hispanic), 4 were African-American, and 1 of Asian descent. Most patients were symptomatic with abdominal pain being the most common symptom. At initial presentaton, 85 patients showed multifocal, nodular &/or diffuse omental and peritoneal disease. 38 patients had diaphragmatic involvement. In addition, 32 patients had liver metastases, 2 cases had pancreatic involvement, and 1 had renal involvement. 49 patients had retroperitoneal involvement in the form of implants, tumoral extension or nodal involvement. In the thorax, 33 had nodal disease, 17 had pleural effusion, and only 2 cases had lung metastases at presentation. 1 patient had intracranial metastasis and 2 had bony metastases at presentation. 12 patients showed calcific lesions.

DSRCT is a rare, multifocal peritoneal malignancy with frequently disseminated abdominal disease at presentation. In the abdomen, the disease most commonly involves the omentum and peritoneum, followed by the retroperitoneum. Liver is the most common solid visceral metastatic site. A substantial number of patients have diaphragmatic involvement. In the thorax, nodal and pleural involvement is more common than lung parenchymal involvement. About 13% of cases showed calcifications.

Desmoplastic small round cell tumor (DSRCT) is a rare, biologically aggressive, multifocal primary peritoneal sarcoma that should be considered in the differential diagnosis of solitary or multiple peritoneal masses in a young white male. The characteristic t(11;22)(p13;q12) translocation involving fusion of EWS & WT1 genes and unique pathological findings are important diagnostic features of this tumor.

Morani, A, Hayes-Jordan, A, Surabhi, V, Bathala, T, Winston, H, Sagebiel, T, Prasad, S, Desmoplastic Small Round Cell Tumor: A Comprehensive, Single-institution Study of 94 Cases.  Radiological Society of North America 2014 Scientific Assembly and Annual Meeting, - ,Chicago IL. http://archive.rsna.org/2014/14016281.html