MULTIPLE NEUROENDOCRINE TUMORS OF DUODENUM ...ENDOSCOPIC SURPRISE AND SURGICAL DILEMMA.

Background: The manifold population of neuroendocrine neoplasms (NENs) arising in the duodenal mucosa and ampullary region has been investigated in several papers, leading to the characterization of various tumor entities according to histological structure, hormonal expression, clinical profile, genetic background, and/or patient outcome [1-3) The duodenal NETs are mainly located in the second part of the duodenum and are usually Single. Even though it is reported in various case series, Multiple neuroendocrine tumour of the first, second and Third part of duodenum is extremely rare and very difficult for a pre op diagnosis and planning. This case is presented to highlight the endoscopic features and therapeutic challenge in the unusual presentation. Clinical Presentation: A 65/M patient was admitted with vague upper abdominal pain and dyspepsia and Vomiting. On OGD he was found to have Unusual multiple umbilicated lesions involving the first, second part of duodenum. Endoscopic diagnosis was Lymphoma / Duodenal carcinoma. The endoscopy biopsy was suggested it to be duodenal carcinoma with neuroendocrine differentiation. In view of existing CKD Patient was investigated with MRI scan and MRI showed multiple polypoidal lesion duodenum first and second part. Patient was worked up and had planned Whipple resection and Histopathology revealed it as neuroendocrine tumour with low grade differentiation. The case is discussed with the stress on unusual endoscopic appearance and rarity of multiplicity. Results: This entity of NETs of duodenum are rare tumours predominantly seen on the second part and single tumour and endoscopic appearance was very unusual. Most were low-grade tumors, ranging from reportedly self-limiting gangliocytic paragangliomas (GPs) to a variety of well-differentiated epithelial neuroendocrine tumors (NETs). This latter category comprised clinically silent or endocrinally active (such as gastrinomas) neoplasms of the duodenum as well as nonfunctioning, somatostatin cell tumors (often called 'somatostatinomas' despite their usual lack of clinically relevant signs of endocrine hyperfunction), commonly localized in the ampullary region and often causing biliary or pancreatic duct obstruction and regional lymph node (LN) metastases. A minority of tumors arose in a genetic background, such as gastrinomas in multiple endocrine neoplasia type 1 (MEN1) syndrome and somatostatin cell tumors in type 1 neurofibromatosis [4-6]. In addition to these differentiated, grade 1 or 2, NETs, a few high-grade (grade 3) neuroendocrine carcinomas (NECs) have also been reported [7,8, 9]. Conclusion: Duodenal neuroendocrine tumours are relatively very rare mesenchymal tumour with a unique histological appearance, and it needs to be distinguished from GIST and other gastrointestinal mesenchymal tumours. But this paper highlights an unusual presentation in view of its endoscopic appearance and Multiplicity. [ABSTRACT FROM AUTHOR]