PMID- 31949932 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200930 IS - 2078-6891 (Print) IS - 2219-679X (Electronic) IS - 2078-6891 (Linking) VI - 10 IP - 6 DP - 2019 Dec TI - Malignant gastrointestinal neuroectodermal tumor, presenting as a second malignancy after gastric adenocarcinoma: a case report and literature review. PG - 1144-1150 LID - 10.21037/jgo.2019.08.04 [doi] AB - Malignant gastrointestinal neuroectodermal tumor (GNET), is a rare soft tissue sarcoma. Here we report a case of GNET arising in the intestine of a 33-year-old female, who had been treated for gastric adenocarcinoma with surgery and chemotherapy at the age of 19, in 2001. Since then, she underwent follow-up care annually and kept disease free. Nevertheless, in 2015 she presented with vomiting and was found to have a mass in the small intestine. Surgical excision was performed. Histologically, the tumor was characterized by polygonal cells with clear or eosinophilic cytoplasm, and variably scattered osteoclast-like multinucleated giant cells. Immunohistochemically, the tumor cells showed diffuse and strong expression for S100, but AE1/AE3 cytokeratin, HMB-45 and Melan-A were negative. Genetically, EWSR1 gene rearrangement was detected by fluorescence in situ hybridization (FISH). All these alterations were different from primary gastric adenocarcinoma. Moreover, the tumor gave metastases to ileal mesentery and lung in 1 and 4 years later, respectively. In summary, this is the first report of primary intestinal GNET with multiple metastases in a young woman who had a known history of chemotherapy for gastric adenocarcinoma. In consistence with previous literature, which reported a secondary GNET following chemotherapy for hepatoblastoma, we speculate that the chemotherapy might trigger the rearrangement of EWSR1 and then promote the tumorigenesis of GNET. CI - 2019 Journal of Gastrointestinal Oncology. All rights reserved. FAU - Zhan, Meng-Na AU - Zhan MN AD - Department of Pathology, Zhongshan Hospital Affiliated to Fudan University, Shanghai 200023, China. FAU - Yu, Juan AU - Yu J AD - Department of Pathology, Zhongshan Hospital Affiliated to Fudan University, Shanghai 200023, China. FAU - Luo, Rong-Kui AU - Luo RK AD - Department of Pathology, Zhongshan Hospital Affiliated to Fudan University, Shanghai 200023, China. FAU - Hou, Ying-Yong AU - Hou YY AD - Department of Pathology, Zhongshan Hospital Affiliated to Fudan University, Shanghai 200023, China. AD - Department of Pathology, School of Basic Medical Sciences, Fudan University, Shanghai 200032, China. LA - eng PT - Case Reports PL - China TA - J Gastrointest Oncol JT - Journal of gastrointestinal oncology JID - 101557751 PMC - PMC6955011 OTO - NOTNLM OT - Gastrointestinal neuroectodermal tumor (GNET) OT - gastric neoplasms OT - secondary primary COIS- Conflicts of Interest: The authors have no conflicts of interest to declare. EDAT- 2020/01/18 06:00 MHDA- 2020/01/18 06:01 PMCR- 2019/12/01 CRDT- 2020/01/18 06:00 PHST- 2020/01/18 06:00 [entrez] PHST- 2020/01/18 06:00 [pubmed] PHST- 2020/01/18 06:01 [medline] PHST- 2019/12/01 00:00 [pmc-release] AID - jgo-10-06-1144 [pii] AID - 10.21037/jgo.2019.08.04 [doi] PST - ppublish SO - J Gastrointest Oncol. 2019 Dec;10(6):1144-1150. doi: 10.21037/jgo.2019.08.04.