Successful high-dose ¹³¹I-MIBG therapy with autologous hematopoietic stem cell rescue for malignant pheochromocytoma

Abstract

A 6-year-old girl with von Hippel–Lindau disease was diagnosed as having pheochromocytoma on the basis of histological examination of the totally resected right adrenal tumor. At the age of 13, interaortocaval tumor and multiple bone metastases were detected. Seven courses of cyclophosphamide–vincristine–dacarbazine therapy after resection of the interaortocaval tumor were not effective; therefore, the patient was administered two courses of high-dose ¹³¹I-MIBG therapy with autologous hematopoietic stem cell rescue. The blood catecholamine level normalized and the uptake of MIBG in bone metastases decreased on ¹²³I-MIBG scintigraphy following the high-dose ¹³¹I-MIBG therapy. The patient is alive and progression-free 3 years after the treatment. High-dose ¹³¹I-MIBG therapy is feasible as a safe and effective treatment for unresectable malignant pheochromocytoma.