Primary malignant gastrointestinal neuroectodermal tumor occurring in the ileum with intra‑abdominal granulomatous nodules: A case report and review of the literature

Huang,Gao‑Xiang; Chen,Qiu‑Yue; Zhong,Ling‑Ling; Chen,Hao; Zhang,Hai‑Peng; Liu,Xiao‑Fen; Tang,Fang

doi:10.3892/ol.2019.10060

Primary malignant gastrointestinal neuroectodermal tumor occurring in the ileum with intra‑abdominal granulomatous nodules: A case report and review of the literature

Authors:
- Gao‑Xiang Huang
- Qiu‑Yue Chen
- Ling‑Ling Zhong
- Hao Chen
- Hai‑Peng Zhang
- Xiao‑Fen Liu
- Fang Tang
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Affiliations: Department of Pathology, 924th (181st) Hospital of The People's Liberation Army, Guangxi Key Laboratory of Metabolic Diseases Research, Guilin, Guangxi 541002, P.R. China
Published online on: February 21, 2019 https://doi.org/10.3892/ol.2019.10060
Pages: 3899-3909
Copyright: © Huang et al. This is an open access article distributed under the terms of Creative Commons Attribution License.

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Abstract

Malignant gastrointestinal neuroectodermal tumors (GNETs) are rare aggressive malignant neoplasms that exclusively occur within the wall of the gastrointestinal tract. The GNET was first described as an ‘osteoclast‑rich tumor of the gastrointestinal tract with features resembling clear cell sarcoma (CCS) of soft parts’ in 2003. Although the GNET shares certain histological features with CCS, it is characterized by a lack of melanocytic differentiation and the presence of non‑tumoral osteoclast‑like giant cells (OLGCs). The present study reports a case of a GNET of the ileum with intra‑abdominal granulomatous nodules, an uncommon accompanying finding, and summarizes the current literature. A 30‑year‑old woman presented with the symptoms of intestinal obstruction, and a mass was found within the ileum wall. Multiple grey‑white nodules were found adhering to the omentum and serosa of the ileum. Histologically, the tumor was located in the muscularis propria and infiltrated the mucosa and the serosa. Tumor cells presented with oval or polygonal nuclei and prominent nucleoli, and were predominantly arranged in nested and pseudopapillary patterns, with the presence of cluster of differentiation (CD)68‑positive, scattered OLGC. Immunohistochemically, it was determined that the tumor cells expressed Vimentin, CD56, S‑100 and transcription factor SOX‑10, while being negative for pan‑cytokeratin, cytokeratin (CK)7, CK20, synaptophysin, chromogranin‑A, CD117, anoctamin‑1, CD34, human melanoma black‑45, Melan‑A, smooth muscle actin, CD3 and CD20 expression. Ewing sarcoma breakpoint region 1 gene rearrangement was identified by fluorescence in situ hybridization analysis. Ultrastructurally, no typical melanosomes were identified. In addition, the intra‑abdominal grey‑white nodules were microscopically identified as chronic granulomatous inflammation. The patient received four cycles of adjuvant chemotherapy following routine tumor resection. Due to its rarity and histological similarity with other neoplasms, unfamiliarity with the features of GNETs by surgical pathologists can easily lead to a misdiagnosis. Therefore, comprehensive assessments, including morphology and ancillary studies, are required for an accurate diagnosis of GNET.

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