Abstract

Pheochromocytoma is a rare neuroendocrine tumour. The prognosis after metastasis is poor, but there have been rare cases of prolonged survival of up to 26 years. Treatments for malignant phaeochromocytoma include surgical debulking, pharmacological control of hormone-mediated symptoms, 131I-metaiodobenzylguanidine (MIBG) therapy, chemotherapy and hormone therapy such as somatostatin analogues. We report the case of a patient, treated only with 131IMIBG therapy, who has survived 28 years since her diagnosis in 1981. The primary lesion was removed in 1982 by adrenectomy. In 1987, urinary catecholamines began to rise, heralding the onset of multiple bone and lung metastases. Her only treatment has been six doses of 131I-MIBG therapy between 1988 and 2003, (total of 22,804MBq). These treatments have relieved pain and led to the resolution of some of the metastatic lesions. She is now 81 years old and is currently very well and asymptomatic, despite evidence of residual thoracic and lumbar vertebral metastases.