Langdurige controle aangewezen na chirurgische behandeling wegens een feochromocytoom

Klinische les
R.R.H. Nap, J.R. Meinardi, G. van den Berg, R.P.F. Dullaart, J. de Vries en B.H.R. Wolffenbuttel

Long-term follow-up is indicated after surgery for a phaeochromocytoma.

- Two patients presented with symptoms that suggested a recurrence of phaeochromocytoma, 17 and 14 years after a first manifestation of this disease for which they had undergone successful surgery. The first patient, a 57-year-old man, had typical symptoms of phaeochromocytoma and had recently experienced deterioration of the glycaemic control of his diabetes mellitus type 2. After initial evaluation and treatment with alpha-adrenergic blockade and propranolol, he again underwent surgery. In addition to peritoneal metastases confirmed to be caused by malignant phaeochromocytoma he also had a carcinoma of the caecum. The second patient, a 32-year-old woman, complained of headache and palpitations and had liver-enzyme disorders. Ultrasound examination revealed a tumour in the right upper abdomen and a recurrence of phaeochromocytoma was diagnosed; an intact pregnancy was also found. After a healthy son had been born by caesarian section, she underwent debulking of the large intra-abdominal lymph nodes and of two metastases in the lung. At the last follow-up she was under treatment with radioactive 123I-meta-iodobenzylguanidine (123I-MIBG) because of residual metastases. Studies report recurrence of phaeochromocytoma in 8-20 of cases, and such a recurrence may be either benign or malignant. Factors predictive for recurrence are older age, a malignant primary tumour, tumour weight above 60 g, extra-adrenal origin of the tumour and peroperative tumour spill. Long-term follow-up of patients is indicated, even after successful initial surgery.

Ned Tijdschr Geneeskd. 2006;150:1045-9