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Familial phaeochromocytoma: successful treatment with 131I-MIBG.

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Goncalves, E Ninane, J. Wese, François-Xavier [UCL] Léonet, Jacqueline Piret, L. Cornu, Guy [UCL] De Meyer, R

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.

Document type Article de périodique (Journal article) – Case Reports, Journal Article
Publication date 1990
Journal information "Medical and pediatric oncology" - Vol. 18, no. 2, p. 126-30 (1990)
issn 0098-1532
Publication status Publié
Affiliation UCL - MD/CHIR - Département de chirurgie
MESH Subject 3-Iodobenzylguanidine ; Adrenal Gland Neoplasms - drug therapy - genetics - radionuclide imaging ; Adult ; Child ; Contrast Media ; Female ; Humans ; Iodine Radioisotopes - diagnostic use ; Iodobenzenes - therapeutic use ; Male ; Neoplasms, Multiple Primary - drug therapy - genetics - radionuclide imaging ; Pheochromocytoma - drug therapy - genetics - radionuclide imaging ; Sympatholytics - therapeutic use
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Bibliographic reference Goncalves, E ; Ninane, J. ; Wese, François-Xavier ; Léonet, Jacqueline ; Piret, L. ; et. al. Familial phaeochromocytoma: successful treatment with 131I-MIBG.. In: Medical and pediatric oncology, Vol. 18, no. 2, p. 126-30 (1990)
Permanent URL http://hdl.handle.net/2078.1/27212