Endocrine Abstracts

Background: Typical presentation of adrenal pheochromocytomas (PHEOs) is a combination of variable hypertension with paroxysmal symptoms. However, recent improvements in diagnostic imaging techniques and increasing availability of genetic testing have facilitated presymptomatic diagnosis of PHEOs.

Objetive: To analyse the clinical features at presentation of PHEOs in a multicentric study population.

Design and patients: Seven Spanish endocrine centres participated in this study. Medical records of 69 patients who were diagnosed of PHEO between 1991 and 2011 were reviewed.

Results: 34 were male and 35 were female. Mean age at diagnosis was 52.11 years (17–78). Typical triad of symptoms was found only in 18.6% of cases. Nine patients (12.9%) were diagnosed during hypertension study. Hypertensive crises during diagnostic or surgical procedures occurred in 10.1% of cases. Thirty two patients (45.7%) were incidentally discovered. 5 cases were diagnosed before 2000, and 27 after this year. These patients were significantly older than patients in whom the diagnosis was suspected on clinical grounds. (57.6±12.2 vs 46.8±17.3 years; P<0.01) The mean size of incidental PHEOs were 4.79±2 cm, smaller than the mean size of the whole study group PHEOs (5.35±3.29 cm). Seventeen genetic testing were done (seven negative and ten positive: nine MEN2 and one Von Hippel Lindau). Three patients (4,3%) was discovered by this way. Patients with positive genetic study were significantly younger (32.1±11.4 vs 52.1±16 years; P< 0.001). Six patients (8.7%) had bilateral tumors; four of these were found to have MEN2. Only one patient had metastasic disease at the time of diagnosis.

Conclusions: In our study group, presymptomatic diagnosis were done in a half of the patients with PHEOs. Frecuency of incidentally discovered PHEOs seemed to be increasing over time and familial PHEO was found in a significant proportion of the patients. Every adrenal incidentaloma should be investigated for the presence of PHEO and genetic testing should be considered in patients with a family history, young age, or multifocal, bilateral, extra-adrenal, or malignant tumors.

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.