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Unusual Abdominal Tumors

CASE 2. INTRA-ABDOMINAL DESMOPLASTIC SMALL ROUND CELL TUMOR

University of Texas Medical Branch, Galveston, TX

A 19-year-old male presented with a 2-month history of abdominal fullness, abdominal discomfort, constipation, and weight loss of 20 pounds. Physical examination revealed a large lower abdominal mass. Computed tomography of the abdomen and pelvis revealed massive ascites with extensive nodularity in the peritoneal wall and the mesentery (Fig 1). A 15-cm heterogeneous mass with areas of low density occupied the pelvis and surrounded the rectosigmoid (Fig 2). The liver, spleen, adrenals, pancreas, and kidneys were normal. Microscopic examination of the core biopsy of the pelvic mass revealed tumor cells arranged in basaloid nests of varying size and shape surrounded by cellular desmoplastic stroma consistent with intra-abdominal desmoplastic small round cell tumor (Figs 3 and 4). Immunohistochemical stains revealed that the tumor cells were positive for cytokeratins, vimentin, and synaptophysin; weakly positive for epithelial membrane antigen, Wilms tumor gene product, and desmin; and negative for CD99.

View larger version (180K): [in this window] [in a new window]   Fig 1. Computed tomography of the abdomen and pelvis, showing massive ascites with extensive nodularity in the peritoneal wall and the mesentery.

View larger version (115K): [in this window] [in a new window]   Fig 2. A 15-cm heterogeneous man with areas of low density, located in the pelvis.

View larger version (114K): [in this window] [in a new window]   Fig 3. Tumor cells from the core biopsy of the pelvic mass.

View larger version (159K): [in this window] [in a new window]   Fig 4. Tumor cells from the core biopsy of the pelvic mass.

The overall survival is poor.^3,5,7 It has been shown to be sensitive to alkylating agents and is dose-responsive.² Multimodality treatment with high-dose chemotherapy, aggressive debulking surgery, radiotherapy, and myeloablative chemotherapy with stem cell rescue may improve progression-free survival.^2,4,5,7,8 Although such treatment offers good palliation and may prolong survival in isolated cases, it is not curative.

REFERENCES

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2. Kushner BH, LaQuaglia MP, Wollner N, et al: Desmoplastic small round cell tumor: Prolonged progression free survival with aggressive multimodality therapy. J Clin Oncol 14:1526–1531, 1996[Abstract/Free Full Text]

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4. Marina NM, Pappo AS, Parham MD, et al: Chemotherapy dose-intensification for pediatric patients with Ewing’s family of tumors and desmoplastic small round-cell tumors: A feasibility study at St. Jude Children’s Research Hospital. J Clin Oncol 17:180–190, 1999[Abstract/Free Full Text]

5. Gerald WL, Ladanyi M, Alava E, et al: Clinical, pathologic, and molecular spectrum of tumors associated with t(11;22)(p13;q12): Desmoplastic small round-cell tumor and its variants. J Clin Oncol 16:3028–3036, 1998[Abstract/Free Full Text]

6. Ali SZ, Nicol TL, Port J, et al: Intraabdominal desmoplastic small round cell tumor: Cytopathologic findings in two cases. Diagn Cytopathol 18:449–452, 1998[CrossRef][Medline]

7. Amato RJ, Ellenhorst JA, Ayala AG: Intraabdominal desmoplastic small cell tumor. Report and discussion of five cases. Cancer 78:845–851, 1996[CrossRef][Medline]

8. Schwartz RE, Gerald WL, Kushner BH, et al: Desmoplastic small round cell tumors: Prognostic indicators and results of surgical management. Ann Surg Oncol 5:416–422, 1998

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