Open Access, Peer-reviewed
pISSN 2288-6575
eISSN 2288-6796
    J Korean Surg Soc. 2011 Feb;80(2):151-156. Korean.
    Published online Feb 07, 2011.
    https://doi.org/10.4174/jkss.2011.80.2.151
    Copyright © 2011 The Korean Surgical Society
    Case Report

    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 and 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 (Email: jhkimgs@ynu.ac.kr )
    Received October 13, 2010; Accepted January 04, 2011.

    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

    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.

    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).

    Fig. 3
    Iimmunohistochemical staining shows positive expression for desmin, cytokeratin. (A) Desmin (×200). (B) Cytokeratine (×200).

    Tables

    Table 1
    Clinical features and outcomes of desmoplastic small round cell tumors in abdomen (5 cases)

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    MeSH Terms
    Abdomen
    Abdominal Cavity
    Abdominal Pain
    Desmoplastic Small Round Cell Tumor
    Follow-Up Studies
    Humans
    Kidney
    Lung
    Male
    Prognosis
    Metrics
    Share
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