Consensus Guidelines for the Management of Patients with Digestive Neuroendocrine Tumors – Well-Differentiated Jejunal-Ileal Tumor/Carcinoma

Abstract

age peak in the 6th and 7th decades of life [1] . The frequency of multicentricity lies between 26 and 30% [3,4] and 15-29% of tumors are associated with other non-carcinoid malignancies [5][6][7] . The WHO classification subdivides jejunal-ileal tumors, similar to other gastroenteropancreatic endocrine tumors, into three general categories [8] : (1) well-differentiated endocrine tumor (carcinoid) of benign behavior (confined to the mucosasubmucosa, non-angioinvasive, ! 1 cm in size) or uncertain behavior (non-functioning, confined to mucosasubmucosa, 1 1 cm in size, or angioinvasive); (2) well-differentiated endocrine carcinoma (malignant carcinoma) with low-grade malignant behavior, deeply invasive (muscularis propria or beyond) or with metastases, and (3) poorly-differentiated endocrine carcinoma (small-cell carcinoma), high-grade malignant.

Diagnosis and Treatment of Gastroenteropancreatic Neuroendocrine Tumors: Current Data on a Prospectively Collected, Retrospectively Analyzed Clinical Multicenter Investigation

“…On reviewing single-center reports from referral centers [13] and recently established national registries [14,15], functioning tumors were reported in up to 40% of patients. Up to 49% localized in the small intestine [14,16,17] and up to 75% localized in the pancreas [9,18] were functioning. In our study, the lack of more patients with functioning tumors and endocrine tumor syndromes in various sites (pancreas, ileum/jejunum) is surprising but reflects the "general" situation.…”

“…Curative surgery is always recommended for jejunal/ileal tumors whenever feasible after careful symptomatic control of the clinical syndrome with somatostatin analogs [16]. Surgery of the primary should be performed as segmental resection, ileocecal resection, or right hemicolectomy with wide lymphadenectomy depending on the localization of the tumor [16].…”

GEP–NETS UPDATE: A review on surgery of gastro-entero-pancreatic neuroendocrine tumors

“…Transduodenal local excision with or without lymph node sampling and pancreaticoduodenectomy are other options for primary treatment of non-metastatic duodenal NETs O2 cm. Nevertheless, the optimal approach remains unclear as the biological behavior of these NETs remains largely unknown (30,31). Although ileum/jejunum NETs often present with metastases at diagnosis, surgical treatment has become increasingly important for their management (32).…”

“…Although ileum/jejunum NETs often present with metastases at diagnosis, surgical treatment has become increasingly important for their management (32). For patients presenting with tumors in the jejunum/ileum, surgical resection of the bowel with regional lymphadenectomy is recommended (30). Mesenteric lymph node metastases are common also with small tumors (33).…”

“…As tumor spread to the lymph nodes and liver can occur also in patients with small primary tumors, surgery of the primary tumors should adhere to oncological principles. This involves clearance of lymph node metastases by dissection around the mesentery (30). The surgical procedure should include careful examination of the entire bowel, because multiple synchronous lesions may be present.…”