Neuroendocrine tumours of the duodenum

Abstract

Non-ampullary duodenal NTs differ clinically, histologically and immunohistochemically as well as with respect to the extent of resection from NTs of the ampulla of Vater.

Well-Differentiated Duodenal Tumor/Carcinoma (Excluding Gastrinomas)

“…In older studies reporting on the 5 types of duodenal NETs, duodenal gastrinomas were the most frequent (mean 48.3% of all duodenal NETs, range 27–58%, 9 series) [4, 6, 10, 11,21,22,23,24,25,26,27]; followed by somatostatinomas (mean 43 ± 6%, range 23–75%, 9 series) [4]; nonfunctional serotonin-containing tumors (mean 27.6 ± 7.2%, 6 series) [4]; nonfunctional calcitonin-containing NETs (mean 9 ± 2.5%, 4 series) [4], and finally rare gangliocytic paragangliomas or neuroendocrine carcinomas.…”

“…This has been well studied for duodenal gastrinomas [5, 6, 10, 21, 22, 24, 25, 27, 28] where 58% arise in D1, 33% arise in D2, 5% in D3 and 4% in D4 [29,30,31,32,33]. Approximately 20% (mean 18 ± 5%, 6 series) of duodenal NETs occur in the periampullary region [4].…”

“…Many studies also differentiated ampulla of Vater or periampullary NETs because numerous studies demonstrated they differed from other duodenal NETs clinically, histologically and in their growth behaviors [10,11,12,13,14,15]. Ampullary NETs are frequently associated with von Recklinghausen’s disease and often show somatostatin immunoreactivity, but almost never produce the clinical features of the somatostatinoma syndrome [4, 6, 10, 13,16,17,18,19,20]. …”

A Rare Cause of Melaena-Duodenal Gangliocytic Paraganglioma

“…Tumour size does not correlate with LN metastases [10,11]. Metastatic potential rises with increased rate of mitosis and spread into the muscularis propria [11].…”

“…Tumour size does not correlate with LN metastases [10,11]. Metastatic potential rises with increased rate of mitosis and spread into the muscularis propria [11]. It is of significance to note that most cases of duodenal GP with lymph node involvement are manifested by spread of the epithelioid component only [12].…”