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

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, pre-operative 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, CEA, 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. MTC consists of a spectrum of disease 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.

A 92-gene cancer classifier predicts the site of origin for neuroendocrine tumors

“…Clinical situations that may cause diagnostic subtyping challenges include: multiple possible primary tumors (in the context of Multiple Endocrine Neoplasia syndromes), widespread metastatic disease, lack of tumor-expressing site-specific serological markers and small biopsies with insufficient clinical data. The site of origin for neuroendocrine carcinoma has become increasingly important for grading/staging purposes, 1,2 for new clinical management guidelines, [3][4][5][6][7] and for primary site-specific targeted therapy. [8][9][10] When a primary site cannot be identified, tumors generally are treated according to the presumed aggressiveness of the tumor, as determined by a combination of the tumor grade [11][12][13][14] and available clinical and radiographic information about tumor metabolism.…”

Efficacy and safety of metyrosine in pheochromocytoma/paraganglioma: a multi-center trial in Japan

“…Although most of the patients with PPGL show benign lesions, a considerable portion of these patients show metastatic lesions [2,3].…”

Continuous renal replacement therapy for haemodynamic collapse and rhabdomyolysis induced by pheochromocytoma crisis

“…The definitive therapy for pheochromocytoma is complete surgical resection of the tumour. For patients in pheochromocytoma crisis, the effects of excessive release of catecholamines must be counteracted to result in haemodynamic stabilization prior to surgery and improve outcomes 5, 6, 7, 8. Administration of α with or without β blockade is part of the standard of care.…”