Malignant gastrointestinal neuroectodermal tumor presenting with small intestinal obstruction: A case report

Malignant gastrointestinal neuroectodermal tumors (GNETs) are rare malignant mesenchymal neoplasms. To our knowledge, only 99 cases have been reported worldwide. The tumor has an aggressive malignancy, with a rapid progression. The histological features of GNET overlap with those of clear cell sarco...

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Bibliographic Details
Published in:DEN open 2022-04, Vol.2 (1), p.n/a
Main Authors: Sasaki, Makiko, Tanaka, Mamoru, Asukai, Koki, Koguchi, Hiroki, Inoue, Yusuke, Moriyama, Mizuki, Tsukahara, Tetsuo, Kawahara, Takeo, Hayashi, Eiji, Hattori, Yukinori, Hasegawa, Izumi, Kataoka, Hiromi
Format: Article
Language:English
Subjects:
Abdomen
Antigens
balloon enteroscopy
Cancer
Case Report
Case Reports
clear cell sarcoma
Conflicts of interest
Cytokeratin
Dissection
Emergency medical care
Endoscopy
Ewings sarcoma
Intestinal obstruction
Laparoscopy
Lymphatic system
Lymphoma
Microscopy
neuroectodermal tumor
Patients
Small intestine
Surgery
Tumors
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Summary:Malignant gastrointestinal neuroectodermal tumors (GNETs) are rare malignant mesenchymal neoplasms. To our knowledge, only 99 cases have been reported worldwide. The tumor has an aggressive malignancy, with a rapid progression. The histological features of GNET overlap with those of clear cell sarcoma, which contain Ewing sarcoma breakpoint region 1 mutation. GNETs lack melanocyte‐specific markers, while clear cell sarcoma exhibits melanocytic differentiation. Various symptoms have been reported previously, and the most reported lesion is in the small bowel. The patient was a 69‐year‐old man who presented with abdominal pain and vomiting. Computed tomography revealed a nodule in the small bowel, which induced small intestinal obstruction. Enteroscopic images revealed a submucosal tumor. Surgery was performed, and the patient was diagnosed with GNET. Only two patients whose primary lesions were in the small intestine, including the patient in this report, have undergone enteroscopy before surgery. This is a rare case of GNET in which a patient underwent enteroscopy before surgical treatment.
ISSN:2692-4609
2692-4609
DOI:10.1002/deo2.119