Malignant duodenal gastrointestinal neuroectodermal tumor (GNET): Case report and review of the literature

Malignant gastrointestinal neuroectodermal tumor (GNET) is a rare malignancy primarily affecting the gastrointestinal tract. Upon cross-sectional imaging, it can be easily confused with other mesenchymal tumors. This article presents a case of duodenal GNET and reviews the current literature on this rare entity.

A 73-year-old female patient presented with a 4 cm duodenal mass on CT scan. With a presumptive diagnosis of GIST, a D3-D4 duodenectomy with cholecystectomy were performed. Subsequent pathological analysis of the surgical specimen revealed a 4.5 cm malignant gastrointestinal neuroectodermal tumor (GNET), also known as clear cell sarcoma-like gastrointestinal tumor (CCSLGT).

While there are less than 115 cases of GNET reported worldwide, prognosis is usually poor with a 50 % survival at 3 years, and mortality rate described is as high as 75 %. To the authors' knowledge, this duodenal GNET case represents the first one ever described for this location.

Early recognition of GNET is essential due to its poor prognosis and its ability to metastasize. Awareness of its existence and diagnostic criteria by every member of the medical team is key to obtain optimal patient care.