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

Abstract

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.

Clear Cell Sarcoma-Like Tumor of the Gastrointestinal Tract with Peritoneal Metastasis in a Young Adult: A Case Report with Literature Review

“…To the best of our knowledge, only seven papers have described the radiological findings of a CCSLTGT in the small intestine ( Table 1 ) ( 1 5 6 7 8 9 ). The patients’ ages ranged from 16 to 69 years, and the mean and median ages were 35.5 and 31.0 years, respectively.…”

“…Half of the cases involved the ileum and the remaining ones involved the jejunum. There were six reports describing tumor morphology, among them, four patients showed bowel wall thickening ( 1 5 6 7 ) and the others presented with an exophytic appearance or intraluminal protruding tumor ( 8 9 ). Four reports illustrated the enhancement pattern of the tumor, and three patients showed mild to moderate progressive enhancement ( 5 8 9 ).…”