Incidental adrenal pheochromocytoma. Report on 5 operated patients and update of the literature

Abstract

Introduction: Adrenal masses discovered by imaging techniques for reasons unrelated to adrenal diseases are called incidentalomas with pheochromocytomas accounting for about 20%. The aim of this study was to report on our experience of 5 patients operated for incidentally discovered adrenal pheochromocytoma and update the literature concerning this subject.

Patients and methods: From September 1976 to December 2000 we operated on 28 for adrenal incidentaloma, and pheochromocytoma was detected in 5 patients (19%). Average age was 61.4 years (range 54-65). All patients underwent adrenal hormonal as well as imaging investigation. Preoperative care for pheochromocytoma was performed in 3 patients. All patients were followed up.

Results: Average tumor diameter, as detected by CT, was 5.5 cm (range 3-10). Urinary 24/h excretion of VMA resulted elevated in 2 cases (40%). Two patients during adrenalectomy developed paroxysmal hypertension which was readily controlled by intravenous administration of nitroprussiate and trandate. Postoperative outcome was uneventful. Average tumor size, as detected by pathology, resulted 8.9 cm (range 3-12). Mean follow-up was 60.4 months (range 32-122) and overall survival rate was 60%. Three patients are alive and disease-free and 2 died after a follow-up of 32 and 36 months for reasons unrelated to the primary disease.

Conclusions: Every incidentally discovered adrenal mass should be investigated for pheochromocytoma since accounting for about 20%. Undetected pheochromocytomas undergoing surgery without preoperative care for avoiding cardiovascular disturbances confer a high risk for morbidity and mortality, especially in pregnancy and childhood. Nonfunctional and small tumors sized 3 cm or lesser may be closely followed up by imaging and hormonal investigation. Surgery is indicated when tumors are functional or larger than 3 cm. Actually, laparoscopy is the new gold-standard in treating adrenal pheochromocytomas. Adrenal sparing surgery as well as autotransplantation of adrenal tissue are both effective and safe in treating patients with bilateral pheochromocytomas, thus improving quality of life since avoiding lifelong steroid dependency. When managed appropriately, the outlook for pheochromocytoma is excellent with a free survival rate of 92% and 80% at 5 and 10 years respectively. Unfortunately, malignant pheochromocytomas are still difficult to treat.