title: Familial phaeochromocytoma: successful treatment with 131I-MIBG.
abstract: We report the case of a family in which the mother died of hypertensive encephalopathy following the relapse of a phaeochromocytoma. Two of her children are still alive. Both children had malignant phaeochromocytomas that have been treated by surgery and 131I-MIBG. The first child presented with phaeochromocytoma of the right suprarenal gland at the age of 7 years. Surgery was performed. At the age of 14 years, he developed a tumour of the left suprarenal gland and two pulmonary metastases demonstrated by 131I-MIBG. The three tumours were removed, but new lesions occurred. The boy then was treated with 200 mCi (7,400 MBq) of 131I-MIBG given twice, and is now free of disease more than 2 years after treatment. His sister presented at the age of 12 years with phaeochromocytoma of the left suprarenal gland, the only lesion recognized by 131I-MIBG. The tumour was removed, but 5 months later, she developed phaeochromocytoma in the right suprarenal gland. She was treated with 200 mCi (7,400 MBq) of 131I-MIBG and surgery was performed 6 months later. Histology of the suprarenal gland could not demonstrate the persistence of phaeochromocytoma cells. The child is now free of disease more than 2 years after treatment.
authors.affiliation: Department of Paediatrics, Cliniques Universitaires Saint Luc, Brussels, Belgium.
authors.forename: E
authors.initials: E
authors.lastname: Goncalves
authors.forename: J
authors.initials: J
authors.lastname: Ninane
authors.forename: F X
authors.initials: FX
authors.lastname: Wese
authors.forename: J
authors.initials: J
authors.lastname: Leonet
authors.forename: L
authors.initials: L
authors.lastname: Piret
authors.forename: G
authors.initials: G
authors.lastname: Cornu
authors.forename: R
authors.initials: R
authors.lastname: De Meyer
mesh_terms: D019797:3-Iodobenzylguanidine; D000310:Adrenal Gland Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000235:genetics*; D000328:Adult; D002648:Child; D003287:Contrast Media; D005260:Female; D006801:Humans; D007457:Iodine Radioisotopes; D007462:Iodobenzenes / Q000627:therapeutic use*; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000235:genetics*; D010673:Pheochromocytoma / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000235:genetics*; D011877:Radionuclide Imaging; D013565:Sympatholytics / Q000627:therapeutic use*
chemical_list: D003287:Contrast Media; D007457:Iodine Radioisotopes; D007462:Iodobenzenes; D013565:Sympatholytics; D019797:3-Iodobenzylguanidine