The management of neuro-endocrine neoplasms

Neuroendocrine neoplasms arise in tissues of the endocrine system, including pituitary, thyroid, parathyroid, pancreas, respiratory system, gastrointestinal tract, and adrenal glands and rarely in other unusual sites. Surgery is the first line of treatment in many of these cases and can include a complete removal of the organ (i.e., total thyroidectomy or adrenalectomy), partial removal (i.e., hemithyroidectomy or distal pancreatectomy), or removal of only the tumor (i.e., pituitary tumor). Medical treatment is frequently used, and while this is usually reserved as adjuvant treatment to control symptoms or tumor growth in cases of residual disease following surgical intervention, it is sometimes used as a treatment of choice (i.e., prolactinoma), sometimes as neoadjuvant before surgery to improve outcomes (i.e., treatment to control growth hormone or cortisol excess before surgery), or as the sole primary treatment in patients who are poor candidates for surgery. Other treatment options can include radiotherapy and peptide receptor radionuclide therapy (PRRT); in some patients, additional therapies are intended for symptom control. Additional data on the molecular basis of neuroendocrine neoplasms may lead to providing treatment sequences personalized for each patient, based on the tumor biology and molecular and genetic patterns. Immunotherapy with immune checkpoint inhibitors may prove to be a successful option for selected neuroendocrine neoplasms.