Management of Gastrointestinal Neuroendocrine Tumors

Abstract

Neuroendocrine neoplasms (NENs) are derived from neuroendocrine cell system and can have benign or malignant characteristics. They are rare tumors, but have been increasing in incidence over the past 40 years. Patients with NENs may develop symptoms due to primary tumor invasion, metastasis, or from secretion of hormonally active tumor substances. Multiple imaging modalities are used for diagnosis and staging, including specialty scans such as 111In pentetreotide (Octreoscan) and 68Gallium-DOTATATE, along with endoscopy, endoscopic ultrasound, and biochemical marker testing. Treatment involves both surgical approach, for both primary and metastatic lesions, as well as medical management for symptom management and disease progression. This article will review the current clinical knowledge regarding the diagnosis, treatment, and prognosis of these fascinating neoplasms and the associated hormonal syndromes.

Gastrointestinal neuroendocrine tumors in 2020

“…Most NETs are slow growing with a small percentage of NETs being rapidly growing[ 6 ]. About 20% of NETs are associated with hereditary genetic syndromes like multiple endocrine neoplasia type 1 (MEN1) and neurofibromatosis type 1 (NF-1)[ 7 ]. We will review the epidemiology, classification, biology, clinical aspects, and management of GI-NETs in this article.…”

Neuroendocrine tumor in the presacral region: a case report

“…On the other hand, there are also non-functioning neuroendocrine tumors, which, as in the case of our patient, usually cause compression symptoms. 13,14 Neuroendocrine tumors are usually more common in the gastrointestinal tract, where authors such as Arvind Dasari et al, 15 recorded an increase in incidence by 6.4 times, being from 1.09 per 100,000 people in 1973 to 6.98 per 100,000 people in 2012, also noting a prevalence of 0.006% in 1993 to 0.048% in 2012. Despite this, the percentage of presentation of these lesions in the pre-sacral space is not known exactly.…”

“…2,5,15 These lesions have a variable clinic that is not only related to the release of hormones but also to their location, which is why symptoms such as digestive bleeding, jaundice, symptoms of intestinal obstruction, weight loss, among others, are usually described; on the other hand, since the root of the sacral nerves is located in the pre-sacral space, it is not strange to suppose that the symptoms of an injury in this location are usually referred to as radiculopathies or rectum anal or sexual dysfunction. 2,13 The world health organization, last updated in 2017, classifies neuroendocrine tumors into three groups that are the differentiated goods, poorly differentiated and the mixed or adenoneuroendocrine. In turn, the well-differentiated are subdivided according to their proliferative activity given by the mitotic index and / or Ki67 index, which has a strong prognostic value (Figure 4).…”

“…Other treatments described for the management of these tumors are Everolimus, Bevacizumab, Sunitinib, as well as cytotoxic therapy (Streptozocin, Dacarbazine, 5-Fluoracil, Capecitabine, Doxorubicin, among others) in progressive or metastatic disease. 13,14 The prognosis of pre-sacral tumors generally depends on the histological nature of the lesion and on complete surgical resection. When malignant degeneration occurs, it usually occurs in males.…”

Differential Diagnosis and Management of Diarrhea in Patients with Neuroendocrine Tumors

“…Carcinoid syndrome (CS) is a paraneoplastic syndrome occurring in 30-40% of patients with well-differentiated GI NETs [20]. It is determined by endogenous secretion of peptide hormones (serotonin and kallikrein).…”