DIFFERENCES BETWEEN SPORADIC AND MULTIPLE ENDOCRINE NEOPLASIA TYPE 2APHEOCHROMOCYTOMA

OBJECTIVES Phaeochromocytoma may be either sporadic or part of a famil ial cancer syndrome, We have investigated whether there are difference s between sporadic and MEN 2A phaeochromocytomas. DESIGN A retrospecti ve study, We analysed age at presentation, sex, mode of presentation, clinical data, laboratory and imaging techniques, treatment, pathology and follow-up. PATIENTS Forty-six patients diagnosed with phaeochromo cytoma between 1979 and 1995 (23 sporadic and 23 familial) at the Hosp ital Universitario Virgen de la Arrixaca (Murcia, Spain), a tertiary r eferral centre, RESULTS Mean age at presentation was 47 +/- 16 years f or sporadic and 38 +/- 11 years for familiar phaeochromocytoma (P < 0. 05). The most common clinical feature and the presenting feature in ca ses of isolated phaeochromocytoma was hypertension, Of the MEN 2A pati ents, 52% were asymptomatic at diagnosis and only 35% presented with h ypertension, Levels of adrenaline, noradrenaline, dopamine and metanep hrines in a 24-hour urine connection confirmed the diagnosis in 95% of the isolated and 91% of the familiar phaeochromocytomas. Computed tom ography located 100% of the sporadic and 76% of the familiar phaeochro mocytomas (P < 0.02). All the MEN 2A phaeochromocytomas were bilateral compared with none of the sporadic phaeochromocytomas (P < 0.001). Al l the patients with familial phaeochromocytoma underwent bilateral adr enalectomy, The patients with sporadic phaeochromocytoma had the affec ted gland or the tumour, if extraadrenal, removed, CONCLUSIONS In comp aring sporadic and MEN 2A phaeochromocytoma we found differences in ag e at presentation, mode of presentation, clinical data, duration of cl inical features, imaging findings bilaterality and type of surgical tr eatment required.