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Kim, Jung, Kim, Lee, and Kim: Clinicopathologic Features and Clinical Outcomes of Desmoplastic Small Round Cell Tumors in Abdomen
Case Report
Journal of the Korean Surgical Society

Journal of the Korean Surgical Society 2011; 80(2): 151-156.

Published online: 7 February 2011

DOI: https://doi.org/10.4174/jkss.2011.80.2.151

Clinicopathologic Features and Clinical Outcomes of Desmoplastic Small Round Cell Tumors in Abdomen

So Hyun Kim, M.D., Sang Hun Jung, M.D., Min-Kyoung Kim, M.D.1, Kyung-Hee Lee, M.D.1, Jae Hwang Kim, M.D.

Department of Surgery, College of Medicine, Yeungnam University, Daegu, Korea.

1Department of Internal Medicine, College of Medicine, Yeungnam University, Daegu, Korea.

Corresponding author (jhkimgs@ynu.ac.kr)

Received 13 October 2010       Accepted 4 January 2011

Copyright © 2011 The Korean Surgical Society

Abstract

Desmoplastic small round cell tumor (DSRCT) is a rare malignant tumor that occurs mainly in the abdominal cavity and has a tendency to occur in young males. But it occurs in other primary sites including the central nerve system, lung, kidney and soft tissue like bone. Major symptoms include abdominal palpable mass, abdominal pain and distention. Useful studies for diagnosis are radiographic study including computerized tomogram and percutaneous pathologic study. DSRCT is an aggressive malignant neoplasm and its prognosis is poor. The overall survival at three years is about 30%. This report describes 5 cases of DSRCT since 2002. The median age of patients was 35 years. All patients are men that presented abdominal pain and abdominal mass. 3 patients underwent surgical resection of tumor and 4 patients received chemotherapy. The survival time of 2 expired patients was 39 and 28 months, individually. And the follow-up period of survival patients is 23~31 months.

Keywords: Desmoplastic small round cell tumor, Abdominal cavity, Prognosis

Figures and Tables

Fig. 1
(A) Abdominal CT shows a large, low-density, lobulated mass in pelvic cavity. (B) The tumor mass shows soild, firm and grey cut surface with necrosis and hemorrhage.
jkss-80-151-g001
Fig. 2
The histological appearance of DSRCT is small sized round cells with hyperchromic nuclei in desmoplastic stroma. (A) H&E stain (×40). (B) H&E stain (×400).
jkss-80-151-g002
Fig. 3
Iimmunohistochemical staining shows positive expression for desmin, cytokeratin. (A) Desmin (×200). (B) Cytokeratine (×200).
jkss-80-151-g003
Table 1
Clinical features and outcomes of desmoplastic small round cell tumors in abdomen (5 cases)
jkss-80-151-i001

*CTX = chemotherapy; †C = curative; ‡P = palliative; §Re-operation for recurrence (peritoneal seeding).

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