Intra-abdominal desmoplastic small round cell tumors: CT findings and clinicopathological correlations in 13 cases
Introduction
Desmoplastic small round cell tumor is a recently described rare malignant neoplasm. It is a member of the family of lesions classified as small round cell tumors (lymphoma, neuroblastoma, rhabdomyosarcoma, Ewing sarcoma, neurectodermic tumors) and it is characterized by a distinct immunohistochemical pattern and a recurrent, specific, chromosomal translocation.
This tumor has a predilection for involvement of the peritoneum mainly in young men. Other sites are rarely affected. It is an aggressive tumor associated with poor survival. Treatment consists in a combination of surgery, chemotherapy and radiotherapy.
CT features are peritoneal involvement with several bulky heterogeneous and necrotic soft tissue masses without any obvious organ base, which are sometimes associated with ascites, adenopathies and liver metastases.
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Materials and methods
Thirteen patients (12 men, 1 woman) with desmoplastic small round cell tumor and primary abdominal involvement were found in our hospital database between 1991 and 2003. Clinical, CT imaging and histological features were studied retrospectively. Initial CT studies were analyzed in all patients before treatment.
The technical performance was variable with conventional or spiral CT; slice thickness varied from 5 to 10 mm but all CT studies were performed after oral administration of contrast
Clinical findings
There were 12 men and 1 woman with a mean age of 24.8 years (range 12–34 years) at the time of the diagnosis (Fig. 1, Fig. 2).
The initial clinical manifestations were abdominal pain (n = 6), a palpable abdominal mass or abdominal distension (n = 6), constipation (n = 3), an inguinal mass (n = 1), a urinary disorder (n = 1), and a chronic cough associated with pulmonary carcinomatous lymphangitis (n = 1).
Follow-up was dismal with 10 deaths (mean duration of survival was 2.8 years (1–5 years); only three
Discussion
Desmoplastic small round cell tumor is a rare malignant peritoneal neoplasm. Less than 200 cases have been described since 1989 when it was first described by Gerald and Rosai [1].
It occurs typically in adolescents and young adult males (5 men for 1 woman in literature) and the mean age at diagnosis is 22 years (3–68 years) [2], [3], [4].
Desmoplastic small round cell tumor mainly involves the peritoneal cavity but other serous membranes have been described: pleura [5], para-testicular serosa [6]
Conclusion
Although CT features are nonspecific, the diagnosis of desmoplastic small round cell tumor may be suspected in young males with multiple bulky heterogeneous peritoneal soft tissue masses. Imaging is useful for staging and also to guide percutaneous biopsy.
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