The North American Neuroendocrine Tumor Society Consensus Guideline for the Diagnosis and Management of Neuroendocrine Tumors

TLDR

Neuroendocrine tumors such as pheochromocytomas, paragangliomas, and medullary thyroid cancer arise from adrenal chromaffin or thyroid C cells, cause significant morbidity through catecholamine release, and are diagnosed and monitored using markers like calcitonin, CEA, and chromogranin A, with disease aggressiveness ranging from indolent to highly lethal. Management requires preoperative adrenergic blockade, laparoscopic removal of primary tumors when feasible, and complete surgical resection as the sole curative option, with diagnosis and surveillance guided by elevated markers such as calcitonin, CEA, and chromogranin A. Genetic testing for RET mutations has allowed identification of familial cases and prophylactic thyroidectomy for cure.

Abstract

Pheochromocytomas, intra-adrenal paraganglioma, and extra-adrenal sympathetic and parasympathetic paragangliomas are neuroendocrine tumors derived from adrenal chromaffin cells or similar cells in extra-adrenal sympathetic and parasympathetic paraganglia, respectively. Serious morbidity and mortality rates associated with these tumors are related to the potent effects of catecholamines on various organs, especially those of the cardiovascular system. Before any surgical procedure is done, preoperative blockade is necessary to protect the patient against significant release of catecholamines due to anesthesia and surgical manipulation of the tumor. Treatment options vary with the extent of the disease, with laparoscopic surgery being the preferred treatment for removal of primary tumors. Medullary thyroid cancer (MTC) is a malignancy of the thyroid C cells or parafollicular cells. Thyroid C cells elaborate a number of peptides and hormones, such as calcitonin, carcinoembryonic antigen, and chromogranin A. Some or all of these markers are elevated in patients with MTC and can be used to confirm the diagnosis as well as to follow patients longitudinally for recurrence. Medullary thyroid cancer consists of a spectrum of diseases that ranges from extremely indolent tumors that are stable for many years to aggressive types associated with a high mortality rate. Genetic testing for RET mutations has allowed identification of familial cases and prophylactic thyroidectomy for cure. The only curative treatment is complete surgical resection.