PS10 PHAEOCHROMOCYTOMA IN CHILDREN

Purpose  Phaeochromocytomas are rare tumours of the sympathoadrenal neuroendocrine system. It is known that these tumours are associated with Von Hippel Lindau (VHL) disease, multiple endocrine neoplasia type II and neurofibromatosis type I. Recently an association with succinate dehydrogenase type B and D gene mutations has been described. Discovery of a SDHB mutation in a girl at our hospital prompted us to review our experience with phaeochromocytoma in children. Methodology  Retrospective review of case notes of all children treated for phaeochromocytoma over the period 1997–2006 at Starship Children’s Hospital, NZ. Results  Seven patients with 8 tumours were identified with average age of 10 years and average systolic BP of 161. The tumours were adrenal in location in 37.5% and 62.5% extra‐adrenal. Our malignancy rate was 12.5%. Familial disease was noted in two patients with one girl having VHL disease and the other found to carry a mutation in the SDHB gene. All children received preop antihypertensives and were treated operatively with seven open resections and one laparoscopic converted to open resection. One child received adjuvant radiotherapy. There were no recurrences or deaths. Conclusions  Phaeochromocytoma in children varies from the adult form. It is more likely to be extra‐adrenal and have a greater chance of a familial genetic syndrome. The discovery of SDH mutations has altered the diagnosis of familial disease and carries implications for family screening, prognosis and surveillance. We emphasize the importance of taking a good family history and suggest screening for SDH mutations in all patients with phaeochromocytoma.