Case Report
Copyright ©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Mar 28, 2017; 23(12): 2258-2265
Published online Mar 28, 2017. doi: 10.3748/wjg.v23.i12.2258
Multiple clear-cell sarcomas of small intestine with parotid gland metastasis: A case report
Hao Su, Wen-Sheng Liu, Wen-Hao Ren, Peng Wang, Lei Shi, Hai-Tao Zhou
Hao Su, Peng Wang, Lei Shi, Hai-Tao Zhou, Department of Colorectal Surgery, National Cancer Center/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100021, China
Wen-Sheng Liu, Department of Head and Neck Surgery, National Cancer Center/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100021, China
Wen-Hao Ren, Department of Pathology, National Cancer Center/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100021, China
Author contributions: Su H collected the data and drafted the manuscript; Zhou HT designed the study and helped revise the manuscript; Liu WS collected the surgical specimens; Ren WH participated in the discussions of the postoperative pathology; Shi L conceived the study and participated in the coordination; Wang P participated in the data interpretation; all authors have read and approved the final manuscript.
Supported by Basic Scientific Research Business of Chinese Academy of Medical Sciences, No. 2016ZX310020.
Institutional review board statement: This case report was exempt from the Institutional Review Board review at Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College.
Informed consent statement: The patient involved in this study gave her written informed consent authorizing the use and disclosure of her protected health information.
Conflict-of-interest statement: All the authors have no conflict of interest to declare.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Hai-Tao Zhou, MD, Professor, Department of Colorectal Surgery, National Cancer Center/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 17, Pan Jia Yuan Nan Li, Chaoyang District, Beijing 100021, China. zhouhaitao01745@163.com
Telephone: +86-10-67787110 Fax: +86-10-67787110
Received: December 6, 2016
Peer-review started: December 8, 2016
First decision: January 10, 2017
Revised: January 23, 2017
Accepted: February 16, 2017
Article in press: February 17, 2017
Published online: March 28, 2017
Abstract

Clear-cell sarcoma is a rare, malignant soft tissue tumor that displays melanocytic differentiation with a distinct molecular profile. It is rarely localized in the gastrointestinal tract. Herein we reported a case of multiple synchronous clear-cell sarcomas of the gastrointestinal tract with parotid gland metastasis. A 51-year-old male patient presented with a growing painless mass under the right ear. A preoperative positron emission tomography/computed tomography showed multiple intestinal masses and a mass in the right parotid with increased glucose uptake, and he underwent operative treatment with resection of three tumors in the jejunum and ileum and then received a right parotidectomy. Postoperative pathological examination showed that cells in the intestinal tumor were consistent with clear-cell sarcoma of the gastrointestinal tract, and the malignant cells in the parotid gland were similar to the intestinal tumor. Immunohistochemical studies revealed positive expression of HMB-45, Melan-A, and S-100. EWSR1 gene fusion transcripts were undetectable by fluorescence in situ hybridization.

Keywords: Clear-cell sarcomas, Clear-cell sarcomas of the gastrointestinal tract, Parotid gland metastasis, Immunohistochemistry

Core tip: Over the past 13 years, only 53 cases of clear-cell sarcomas of the gastrointestinal tract (CCS-GI) have been reported in the world. Most of the literature on CCS-GI describes a single tumor at diagnosis; our presentation is the third report of simultaneous tumors during the diagnosis to date and is the first case of CCS-GI with metastasis to the parotid gland. We also reviewed the literature on CCS-GI. Because of the high rarity, more cases need to be accumulated for further analysis.