Pheochromocytomas: a single centre experience
S. Belo, A. Magalhães, C. Nogueira & D. Carvalho
Introduction: Catecholamine-secreting tumors (pheochromocytomas and paragangliomas) are rare intra and extra-adrenal neoplasms, probably occurring in less than 0.2% of patients with hypertension. Serious morbidity and mortality rates are associated with these tumors which are related to the effects of catecholamines on various organs, especially those of the cardiovascular system.
Methods: We reviewed the cases of pheochromocytomas and paragangliomas that were diagnosed, treated and/or followed in our institution since 1983 till 2011. Statistical analyses was made using SPSS 17. Data is presented in frequencies and mean ± standard deviation.
RESULTS: In this review were included 88 patients (58% women; 42% men). The mean age of diagnosis was 49.2±15.2 years and only 6.8% of the patients presented known familial history of the disease. The diagnosis of pheochromocytoma was made in 59% of the patients.
The most frequent form of presentation was as incidentaloma (36.4%) followed by persistent hypertension (27.3%) and other causes (ex: cervical mass: 26.1%).
Urinary metanephrines were determined in 51% of the patients (normetanephrine 3042.7±4702.0 μg/24 h; metanephrine 1991.8±3741.0 μg/24 h). An imaging examination was done in 60% of the cases, being a MIBG scintigraphy in 68% of them.
Surgery preparation was made in 45.5% of the patients (34.1% only α blocking; 11.4% α and β blocking) for a mean time of 25.3±44.9 days. Major surgery complications were observed in 8% of the patients, 43% of them didnt perform preoperative preparation according to clinical standards.
Patients were followed for a mean period of 48.5±52.8 months, 10.5% were not cured after surgery, in 4.7% disease recurrence was observed and 29.1% were lost to following.
Discussion: Pheochromocytoma is a rare entity but with serious consequences if not diagnosed or properly managed. Judicious ad eternum follow up is necessary to ensure early detection of recurrences.
Declaration of interest: The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project.
Funding: This research did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector.