Neuroendocrine neoplasms of the jejunum: a heterogeneous group with distinctive proximal and distal subsets

Abstract

Neuroendocrine tumors (NETs) of the jejunum are rare and usually grouped with either duodenal or ileal NETs. We aimed at better evaluating their characteristics by studying 116 cases of small-bowel NETs for which a precise anatomical location was available. Thirty-four cases were duodenal. Eighty-two were located after the duodenojejunal ligament, including ten cases in the first 50 cm, four cases between 50 and 100 cm, and six cases between 100 and 250 cm. All tumors located after 50 cm from the duodenojejunal ligament were enterochromaffin neoplasms. In contrast, the ten tumors located before this point formed a heterogeneous group. They included two cases of gastrin-expressing tumors in the first 10 cm and one case of enterochromaffin tumor located at 45 cm. The seven remaining cases were large tumors, located between 10 and 50 cm, of intermediate or high histological grade (four out of seven G2 or G3), locally invasive and usually metastatic (five out of seven with liver metastases); their survival was comparable to that of duodenal NETs. Patients with tumors located in the duodenum or the first 50 cm of the jejunum had longer survivals than those with lower jejunal and ileal tumors (p = 0.024). In conclusion, our study underlines the heterogeneity of jejunal NETs and supports the distinction between "upper" and "lower" jejunal tumors, which, for prognostic purposes, might be grouped with, respectively, duodenal and ileal NETs. Our data suggest that the arbitrary limit between upper and lower jejunal tumors might be fixed at 50 cm from the duodenojejunal ligament.

Pattern and risk factors for distant metastases in gastrointestinal neuroendocrine neoplasms: a population‐based study

“…Therefore, we suppose that small intestine NENs should be divided into duodenal and jejunal/ileal subtypes, which have different embryonic origin and showed different biological behaviors in our study. This conclusion is consistent with some retrospective studies [22][23][24]. The risk factors for metastasis presented in our study suggested that tumor grade, histology, T-stage, and N-stage do not affect the metastatic status in some primary sites, such as the esophagus and stomach.…”

Breast Metastasis as the First Clinical Manifestation of Ileal Neuroendocrine Tumor. A Challenging Diagnosis with Relevant Clinical Implications

“…Although most ileal NETs are grade 1 (NET G1), they are frequently metastatic to regional lymph nodes and the liver [1,4]. In addition, a few cases of ileal NETs that are metastatic to the breast have been reported in the English literature [5][6][7][8][9][10][11][12][13][14][15][16].…”

Rare diseases in clinical endocrinology: a taxonomic classification system

“…REMD (main disorders, with all variants and subtypes) have been subdivided as follows: (Table 1) [184] J Endocrinol Invest Childhood/ adulthood [194] 1-9/1,000,000 3-5 years [196] tumors of the upper jejunum usually share the same general behavior than their duodenal counterparts Jejunal entero chromaffin tumors share the same presentation and general clinical behavior than their ileal counterparts, including a strong tendency to local invasion, regional lymph node involvement, and metastatic dissemination ''Duodenal-like'' tumors of the upper jejunum usually synthesize gastrin Unknown Unknown Unknown [252] 48 rare mineral and bone diseases, (Table 4) 41 rare diseases of the PTH/PTHr1 and PTHrp receptors, (Table 5) 69 rare metabolic diseases of glucose, lipids and mucopolysaccharides, (Table 6) 6 rare water metabolism diseases, ( Table 7) 49 rare adrenal glands and paraganglia diseases, (Table 8) 20 rare ovarian diseases, (Table 9) 28 rare testicular diseases, (Table 10) 14 rare diseases with possible multiple endocrine involvement, and (Table 11) 23 rare neuroendocrine tumors. This report provides a complete taxonomy for the classification of REMDs of the adult.…”