Diagnosis and treatment of pheochromocytoma

Early detection of a pheochromocytoma is necessary to avoid the cardiovascular complications of hypertension and to recognize the 10% of malignant neoplasms. Hypertensive changes, particularly hypertensive spikes and stable hypertension are the most common clinical features. A pheochromocytoma must be suspected in presence of various symptoms of which cephalea, tachycardia, diaphoresis are the most frequently encountered. Plasma catecholamines and 24-hours urine metabolites measurements have 60-100% and 72-99% sensitivity respectively. Radiologic procedures commonly used to locate a pheochromocytoma include CT and RM: they achieve 100% sensitivity for unilateral adrenal lesions and respectively 64% and 88% sensitivity for extraadrenal lesions. Scintigraphic localization with MIBG provides functional informations and is recommended for follow-up of the recurrent or metastatic pheochromocytoma. Preoperative medical treatment using alpha and β blocking agents or calcium channel blockers has contributed to reduce perioperative morbidity and mortality. Anterior transabdominal approach is advocated as the standard operative procedure; this approach allows ready access to any site where tumors are obviously present and permits thorough exploration of the abdominal cavity for additional contralateral adrenal or extraadrenal lesions. An accurate preoperative localization of a sporadic, unilateral, of less than 7-10 cm, benign neoplasm constitutes the indication for the laparoscopic adrenalectomy. Pheochromocytoma-free survival were 92% and 80% at 5 and 10 years respectively; in living patients without recurrence, hypertension-free survival was 74% and 45% at 5 and 10 years respectively. Extraadrenal pheochromocytomas are frequently malignant (36%) and are associated with a high incidence of persistent or recurrent disease (32%).