[Literature review in the diagnosis and treatment of malignant pheochromocytomas and paragangliomas.].

OBJECTIVE: Perform a review on the diagnosis and treatment of pheochromocytomas and malignant paragangliomas. MATERIAL AND METHOD: A search was conducted in PubMed and Google Scholar of articles or clinical guides that referred to the diagnosis and treatment of these tumors. RESULTS: For the diagnosis of malignancy, a histological confirmation of a pheochromocytoma or paraganglioma should be provided, plus the presence of metastasis confirmed by images. Methanephrines are recommended over other biochemical determinations. For staging, PET-CT with 18F-FDG or 18F-DOPA is preferred because of its greater sensitivity than conventional images. The 123I-MIBG scan should be requested when radiotherapy with 131I-MIBG is planned.For treatment, control of adrenergic symptoms through the use of α-blockers is recommended. Active surveillance was an option in selected patients with slowly progressive tumors. Surgical treatment improved OS (148 months vs 36 months p=<0.01). Therapy with 131I-MIBG was indicated in patients with positive scintigraphy, reporting a global survival of 50% at 5 years with variable tumor responses. Chemotherapy was proposed in rapidly progressive disease, reporting a median overall survival of 6 years. Ablative therapies should be considered when there is a limited number of lesions, to achieve local tumor control and reduce the symptoms of excess catecholamines. External radiation therapy at high doses would be effective for patients with local symptoms due to their tumor burden. Prospective multi-institutional clinical trials are needed to determine the true benefits of molecular therapies in these patients.

CONCLUSIONS: We recommend a multidisciplinary approach in centers of high complexity to be able to offer the entire diagnostic - therapeutic arsenal available so far that they improve the survival and quality of life of these patients.