Medical Treatment of Gastrointestinal Neuroendocrine Neoplasms

Abstract

Neuroendocrine neoplasms (NENs) are rare tumours that arise mainly in the gastrointestinal or pulmonary system. Most NENs are well-differentiated and may obtain prolonged survival besides the presence of metastatic disease; however, a subset (poorly differentiated NENs) may display a truly aggressive behaviour exhibiting a poor prognosis. The recently developed classification systems along with advances in functional imaging have helped stratify patients to the administration of appropriate therapeutic options. Surgery is the mainstay of treatment of NENs, but in recent decades there has been a considerable evolution of medical treatments that are used for locally advanced or metastatic disease not amenable to surgical resection. Long acting somatostatin analogues are the main therapeutic modality for patients with functioning and well-differentiated low grade NENs exhibiting symptomatic control and mainly stabilisation of tumour growth. Other systemic treatments include chemotherapy, molecular targeted agents, interferon-α, peptide receptor radionuclide therapy (PRRT), and immunotherapy. In addition, new agents such as telotristat may be used for the control of symptoms of carcinoid syndrome. The choice and/or sequence of therapeutic agents should be individualized according to tumour origin and differentiation, disease burden, presence of clinical symptoms and patients’ performance status in the context of a multidisciplinary approach. Recent advances in the molecular pathogenesis of NENs set the field for a more personalised treatment approach.

Pancreatic metastases in patients with neuroendocrine neoplasms: A multi‐centre cohort study

“…A number of parameters including the primary origin, the grade, the metastatic extent, the functionality and the patient's performance status need to be considered in order to select the most appropriate modality of systemic treatment in the context of a multidisciplinary approach. [22] This retrospective analysis of patients with PMs identified in six tertiary referral centres suggests that the presence of PMs does not shorten survival while no predictors of survival have been identified.…”

“…A number of parameters including the primary origin, the grade, the metastatic extent, the functionality and the patient's performance status need to be considered in order to select the most appropriate modality of systemic treatment in the context of a multidisciplinary approach. [22]…”

Changing biological behaviour of NETs during the evolution of the disease: progress on progression

“…On progression, interferon-α, targeted-therapy, either molecular or radionuclide (PRRT), or chemotherapy may follow and in a higher-grade NEN, immunotherapy may be used depending on the progression of the disease, imaging or histological features. Therapeutic agents are used in sequential order or in combination depending on tumour origin and differentiation, disease burden, clinical symptoms, and the patient's performance status (Tsoli et al 2020). As tumours move along a path of progression, the appropriate therapy may change, and it is, therefore, important that the varying biochemical, pathological, and imaging modalities are considered.…”

The Role of Somatostatin Analogues in the Control of Diarrhea and Flushing as Markers of Carcinoid Syndrome: A Systematic Review and Meta-Analysis

“…Surgical resection is the treatment of choice for local, locoregional disease and oligometastatic hepatic disease and as a means for cytoreduction in severe and uncontrolled CS [ 12 , 13 ]. However, somatostatin analogues (SSAs) remain the standard medical care for the hormonal symptoms in CS, even in a presurgical setting, in order to avoid a carcinoid crisis [ 14 , 15 ]. Somatostatin (SST-14), originally discovered in 1973, was considered at the time as a hypothalamic neuropeptide inhibiting the secretion of growth hormone but subsequently was shown to also inhibit gastrointestinal cell motility, secretion, and growth [ 16 ].…”