Malignant gastrointestinal neuroectodermal tumor-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 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.

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

Sasaki

Tanaka

Asukai

et al. 2022

DEN Open

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Malignant gastrointestinal neuroectodermal tumor: a case report and literature review

Su,

Yang,

Zhao

et al. 2023

Annals of Medicine &Amp; Surgery

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Introduction and importance: Malignant gastrointestinal neuroectodermal tumor (GNET) is an extremely rare primary malignant mesenchymal tumor of the gastrointestinal tract characterized by EWSR1 gene rearrangement. An optimal systemic treatment strategy for advanced/recurrent GNET has not yet been identified. Case presentation: A 24-year-old male patient was hospitalized with abdominal pain and underwent two operations for tumor in his small intestine. Immunohistochemistry showed strong expression of S-100 protein and SOX 10. Fluorescence in situ hybridization analysis and next-generation sequencing analysis indicated that there were EWSR gene rearrangements and the presence of EWSR-ATP1 gene fusions, respectively. The diagnosis of GNET in the small intestine was confirmed by pathology. The young patient received the fifth line of apatinib mesylate and the sixth line of apatinib combined with temozolomide. The two apatinib-containing regimens showed stable disease and progression-free survival of 4.7 months and 3.1 months with single agent apatinib or apatinib combined with temozolomide, respectively. Clinical discussion: To our best knowledge, this is the first report of malignant GNET treated with apatinib and temozolomide. Apatinib containing regimens might has antineoplastic activity against GNET. We reviewed the relevant reports of previous GNET treatment, summarized the clinicopathological characteristics of GNET, and found that there are no reports of apatinib for backline treatment of GNET. Conclusion: Containing apatinib may provide an additional treatment option for patients with chemotherapy-resistant GNET tumors.

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

Park

Seo

2023

J Korean Soc Radiol

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Clear cell sarcoma-like tumor of the gastrointestinal tract (CCSLTGT) is a rare malignant mesenchymal tumor of the gastrointestinal (GI) tract with a high probability of local recurrence and distant metastasis in young adults. To the best of our knowledge, only seven case reports have described the imaging findings of a CCSLTGT originating from in the small intestine in English literatures so far. Therefore, we report the imaging findings of a CCSLTGT that occurred in the small intestine of a 22-year-old female and summarize the imaging findings of the previous reports.

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Malignant gastrointestinal neuroectodermal tumor-A case report

Cited by 5 publications

References 10 publications

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

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

Malignant gastrointestinal neuroectodermal tumor: a case report and literature review

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

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