Carotid body tumors and adrenal pheochromocytomas in siblings of a Turkish family.
OBJECTIVE
This is a report of 2 hypertensive siblings with a history of carotid body tumors and subsequent benign adrenal pheochromocytomas (pheos) in a family where the mother had died of possible adrenal carcinoma.


METHODS
The first case was a 35-year-old woman with paroxysmal hypertensive attacks and a right adrenal mass. She had earlier undergone surgery to remove bilateral carotid body tumors. Investigation revealed excessive excretion of catecholamines and their metabolites in the urine. Abdominal MRI and (131)I-MIBG scintigraphy revealed a right adrenal tumor. Right adrenalectomy was performed. The second case, the 45-year-old brother of the first case, was found to have a left adrenal mass on abdominal MRI. Catecholamines and their metabolites in the urine were found to be increased. He had also had surgery to remove bilateral carotid body tumors of the neck. Left adrenalectomy was performed. Both siblings showed no evidence of other familial syndromes, such as multiple neoplasia type 2, von Hippel-Lindau disease or neurofibromatosis type 1.


CONCLUSIONS
Although the combination of familial carotid body tumors and pheo is rare, a patient who remains hypertensive after removal of a carotid body tumor deserves a careful evaluation to exclude pheo. Such tumors may be extra-adrenal or multifocal.
Erem
Cihangir
C
Department of Internal Medicine, Division of Endocrinology and Metabolism, Karadeniz Technical University Faculty of Medicine, Trabzon, Turkey. cihangirerem@hotmail.com
Hacihasanoglu
Arif
A
Cinel
Akif
A
Isik
A Cemal Umit
AC
Reis
Abdulkadir
A
Sari
Ahmet
A
Ersoz
Halil Onder
HO
Ukinç
Kubilay
K
D000310:Adrenal Gland Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D000315:Adrenalectomy; D000328:Adult; D002345:Carotid Body Tumor / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D010673:Pheochromocytoma / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D014421:Turkey