Phaeochromocytomas in 72 patients: Clinical and diagnostic features, treatment and long term results

Abstract

Summary Phaeochromocytomas were diagnosed in 72 patients in Hammersmith, Belfast and Newcastle between 1955 and 1976. Fourteen were found unexpectedly at autopsy while 58 were diagnosed in life. Associated lesions included neurofibromatosis (7 patients), medullary carcinoma of the thyroid (3), renal artery stenosis (2) and cerebellar haemangioblastoma (1). The clinical features were similar to those reported by others. Hypertension was found in all but one of the patients and was sustained only in 17 per cent, paroxysmal only in 48 per cent and sustained with superadded paroxysms in 33 per cent. Hypertension was significantly greater in the women than in the men. Diagnosis was confirmed chemically in all patients and urinary vanylmandelic acid (VMA) was elevated in all but one. Tumours were localized correctly by various radiographic techniques and by selective venous sampling in over 80 per cent of patients. The tumours were in adrenals in 59 patients (bilateral in 6) and at extra-adrenal, intra-abdominal sites in 13. The tumours were removed surgically from those in whom they were diagnosed in life. Before 1967 3 of 17 patients died after operation (18 per cent). Since then patients have been prepared with alpha blockade and have received infusions of plasma, blood or dextran after removal of the tumours. One of 41 patients so treated has died after operation (2 per cent). Phaeochromocytomas were diagnosed and treated in pregnancy in 4 patients, all of whom survived. Two of the fetuses died. Five tumours diagnosed in life were malignant and 2 recurred (40 per cent). Fifty-three were benign and 2 of these recurred locally (4 per cent). All but 2 patients (who could not be traced) were followed completely from 1 to 20 years after operation and 3 died during this period. Two-thirds of the patients were alive after 10 years. Paroxysmal hypertension was abolished by operation, but sustained hypertension often persisted and required drug therapy. It is concluded that at least one-third of phaeochromocytomas are not diagnosed in life and that a high index of suspicion is required to bring them to light. If they are diagnosed and treated by present methods, the prognosis is good.