Abstract

HEVIA COSTA, Manuel Ernesto. Update on diagnostic and therapeutic management of paraganglioma. Rev Cubana Endocrinol [online]. 2014, vol.25, n.3, pp.149-162. ISSN 1561-2953.

Paraganglioma is a rare neuroendocrine tumor that may occur in several parts of the body. Roughly 97 % of these tumors are benign and can be excised through surgery whereas 3 % of them are malignant and cause distant metastasis. Almost 75 % are sporadic and the remaining 25 % are hereditary (more likely to be multiple and developed at early ages). They may be genetically predisposed and associated to family tumor syndromes such as type 2 multiple endocrine neoplasia, Von Hippel-Lindau syndrome and type 1 neurofibromatosis or specific mutations related to development of paragangliomas. When compared to the sporadic occurrence, the family presentation tends to appear at younger ages, with multiple locations. Owing to the low resource availability and the high cost of research, these tumors are difficult to be diagnosed in our conditions, so there may be failures in registering the real number of cases; this is the reason why it is always important to think on possible diagnostic confirmation when clinical suspicion of paraganglioma arises. Imaging studies and measurement of the non-physiological production of catecholamines may help in the diagnosis of this disease. The main therapeutical modalities are surgery, embolization and radiotherapy. The present review was intended to update this topic and to submit a treatment guideline.

Keywords : paraganglioma; neuroendocrine tumor; catecholamines; surgery.

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