Case Report
Copyright ©2014 Baishideng Publishing Group Co., Limited. All rights reserved.
World J Gastroenterol. May 7, 2014; 20(17): 5157-5164
Published online May 7, 2014. doi: 10.3748/wjg.v20.i17.5157
Clinical and computed tomography features of adult abdominopelvic desmoplastic small round cell tumor
Xun-Ze Shen, Jian-Guo Zhao, Jian-Jun Wu, Fang Liu
Xun-Ze Shen, Department of Radiology, Shaoxing People’s Hospital, Shaoxing Hospital of Zhejiang University, Shaoxing 312000, Zhejiang Province, China
Jian-Guo Zhao, Department of Radiotherapy, Shaoxing People’s Hospital, Shaoxing Hospital of Zhejiang University, Shaoxing 312000, Zhejiang Province, China
Jian-Jun Wu, Department of Radiology, Second Affiliated Hospital of Medical School, Zhejiang University, Hangzhou 312009, Zhejiang Province, China
Fang Liu, Department of Pathology, Shaoxing People’s Hospital, Shaoxing Hospital of Zhejiang University, Shaoxing 312000, Zhejiang Province, China
Author contributions: Shen XZ designed the research and wrote the paper; Zhao JG and Wu JJ revised the paper; Liu F analyzed the pathological figures.
Correspondence to: Xun-Ze Shen, MD, Associate Chief Physician, Department of Radiology, Shaoxing People’s Hospital, Shaoxing Hospital of Zhejiang University, Yuecheng Downtown, Shaoxing 312000, Zhejiang Province, China. shenxunze@163.com
Telephone: +86-575-88228548 Fax: +86-575-88228899
Received: October 7, 2013
Revised: December 22, 2013
Accepted: January 20, 2014
Published online: May 7, 2014
Processing time: 211 Days and 23.2 Hours
Abstract

To investigate the clinical and computed tomography (CT) features of desmoplastic small round cell tumor (DSRCT), we retrospectively analyzed the clinical presentations, treatment and outcome, as well as CT manifestations of four cases of DSRCT confirmed by surgery and pathology. The CT manifestations of DSRCT were as follows: (1) multiple soft-tissue masses or diffuse peritoneal thickening in the abdomen and pelvis, with the dominant mass usually located in the pelvic cavity; (2) masses without an apparent organ-based primary site; (3) mild to moderate homogeneous or heterogeneous enhancement in solid area on enhanced CT; and (4) secondary manifestations, such as ascites, hepatic metastases, lymphadenopathy, hydronephrosis and hydroureter. The prognosis and overall survival rates were generally poor. Commonly used treatment strategies including aggressive tumor resection, polychemotherapy, and radiotherapy, showed various therapeutic effects. CT of DSRCT shows characteristic features that are helpful in diagnosis. Early discovery and complete resection, coupled with postoperative adjuvant chemotherapy, are important for prognosis of DSRCT. Whole abdominopelvic rather than locoregional radiotherapy is more effective for unresectable DSRCT.

Keywords: Desmoplastic small round cell tumor; Peritoneum; Pathology; Computed tomography; Clinical features

Core tip: Desmoplastic small round cell tumor (DSRCT) is a rare abdominopelvic malignancy with multicentric growth. The third decade may be a peak period of incidence, and the disease can also occur in older people. Computed tomography can display characteristic features that are helpful in diagnosis of DSRCT. Prognosis and overall survival rates are generally poor. Early discovery and complete resection, coupled with postoperative adjuvant chemotherapy, are important for prognosis of DSRCT. Whole abdominopelvic rather than locoregional radiotherapy is more effective for unresectable DSRCT.