Intra-Abdominal Desmoplastic Small Round Cell Tumor in Children: A Clinicopathologic Study

Abstract

Desmoplastic small round cell tumor (RCT) is a relatively newly recognized neoplasm. It has a very distinct morphologic and high-grade polyphenotypic expression with a very poor prognosis. It is commonly seen in adolescent boys. We describe two cases of intra-abdominal desmoplastic small RCT in young girls (5 and 11 years of age). In both cases, the exact origin of tumor in the abdomen could not be established. Histopathologic examination of the biopsy specimens showed distinct desmoplastic stroma and coexpression of various epithelial, neural, and muscle markers. These two patients were treated primarily by debulking surgery followed by adjuvant chemotherapy (RCT II protocol). One of the two had a stable disease while the other had a progressive disease while on treatment. Thus our findings support the diverse histogenesis of this tumor and its poor prognosis.

Intraabdominal desmoplastic small round cell tumour

“…Only 27 paediatric cases have been documented. Case 2 in this study is the second youngest patient documented so far [4,8,9].…”

“…The prognosis is also better in those patients who present with primary localisation at the paratesticular level without intraabdominal involvement [8]. It is proposed that new chemotherapy protocols be developed for treating this highly malignant disease.…”

Desmoplastic Small Round Cell Tumors in a Young Man

“…Characterized by a predilection for young men, it typically presents as an intra-abdominal mass with multiple intra-peritoneal implants (2,3). However, the site of origin may not be readily apparent (4). The diagnosis of DSRCT is established based on histology, immunochemistry and cytogenetics.…”

Treatment of Desmoplastic Small round Cell Tumor with Image-Guided Intensity Modulated Radiation Therapy as a Component of Multimodality Treatment

“…Mean survival is 20 months, however appreciably longer survival has been noted, particularly with aggressive therapy 10 -11 . DSRCT most frequently arises in the peritoneal cavity and may present with peritoneal "caking" of tumor implants 10. However, its site of origin may not be readily apparent 4 • Treatment failure may occur locally or via metastasis to the liver, lungs, lymph nodes, and less commonly to bone, thus the importance of multi-modality therapy was recognized early on 5 • 11 . Successful treatment approaches have generally emphasized aggressive, multi-agent chemotherapy regimens (cisplatin, etoposide, adriamycin, bleomycin, gemcitabine, ifosfamide, dacarbazine, methotrexate, taxol, cyclophosphamide, vincristine and others in varied combinations) 2 • 3 • 5 • 7 in addition to local therapy with surgery and radiation.…”