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DEN open 2022 Apr 10;2(1):e119. Epub 2022 Apr 10.

Department of Gastroenterology and Metabolism Nagoya City University Graduate School of Medical Science Aichi Japan.

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. Read More

BMJ Case Rep 2022 Jul 20;15(7). Epub 2022 Jul 20.

Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA

Malignant gastrointestinal neuroectodermal tumour (GNET) is a rare, aggressive neoplasm with fewer than 100 cases reported in the literature. Most cases arise in the tubular gastrointestinal tract. We reported a unique case of GNET arising in the extrahepatic bile ducts and reviewed the literature of GNETs. Read More

Front Endocrinol (Lausanne) 2022 28;13:925632. Epub 2022 Jun 28.

Department of Hepatology and Gastroenterology, Aarhus University Hospital, Aarhus, Denmark.

Introduction: The European Neuroendocrine Tumor Society, ENETS, reports variables of prognostic significance in pancreatic neuroendocrine tumors (PNET). However, studies have short follow-ups, and the optimal treatment remains controversial. We aimed to determine overall survival (OS), progression-free survival (PFS) after conservative treatment, and recurrence-free survival (RFS) after surgery and further to find predictors of aggressive PNET behavior to support treatment decisions. Read More

J Gastrointest Oncol 2022 Jun;13(3):1489-1498

Zhuhai Interventional Medical Center, Zhuhai Hospital Affiliated with Jinan University, Jinan University (Zhuhai People's Hospital), Zhuhai, China.

Background: Malignant gastrointestinal neuroectodermal tumor (GNET) is extremely rare in soft tissue sarcoma and occurs mainly in the small intestine of young adults, without sex predilection. Local recurrence and metastasis are common in GNET, resulting in a poor prognosis. GNETs are histologically and immunohistochemically similar to many sarcomas, especially clear cell sarcoma (CCS), making their identification difficult. Read More

J Comput Assist Tomogr 2022 Jun 28. Epub 2022 Jun 28.

From the Department of Abdominal Radiology, The University of Texas MD Anderson Cancer Center.

Abstract: Also referred to as "osteoclast-rich, clear cell sarcoma-like tumor of the gastrointestinal tract (CCSLGT)," malignant gastrointestinal neuroectodermal tumor is a newly described, rare, aggressive sarcoma that commonly arises in the small bowel, stomach, and colon. Histogenesis is likely from an autonomous nervous system-related primitive cell of neural crest origin. The hallmark genetic finding of EWS-CREB1 or EWS-ATF1 fusion transcripts clinches the diagnosis. Read More