title: Carotid body tumors and adrenal pheochromocytomas in siblings of a Turkish family.
abstract: 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.
authors.affiliation: Department of Internal Medicine, Division of Endocrinology and Metabolism, Karadeniz Technical University Faculty of Medicine, Trabzon, Turkey. cihangirerem@hotmail.com
authors.forename: Cihangir
authors.initials: C
authors.lastname: Erem
authors.forename: Arif
authors.initials: A
authors.lastname: Hacihasanoglu
authors.forename: Akif
authors.initials: A
authors.lastname: Cinel
authors.forename: A Cemal Umit
authors.initials: AC
authors.lastname: Isik
authors.forename: Abdulkadir
authors.initials: A
authors.lastname: Reis
authors.forename: Ahmet
authors.initials: A
authors.lastname: Sari
authors.forename: Halil Onder
authors.initials: HO
authors.lastname: Ersoz
authors.forename: Kubilay
authors.initials: K
authors.lastname: Ukinç
mesh_terms: 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