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Med Princ Pract 2006;15:396–400

Carotid Body Tumors and Adrenal Pheochromocytomas in Siblings of a Turkish Family

Erem C.a · Hacihasanoglu A.a · Cinel A.b · Isik A.C.U.c · Reis A.d · Sari A.e · Ersöz H.Ö.a · Ukinç K.a
aDivision of Endocrinology and Metabolism, Department of Internal Medicine, and Departments of bGeneral Surgery, cOtorhinolaryngology, dPathology and eRadiology, Karadeniz Technical University Faculty of Medicine, Trabzon, Turkey
email Corresponding Author

 goto top of outline Key Words

  • Familial pheochromocytoma
  • Carotid body tumor
  • Turkish family

 goto top of outline 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. Clinical Presentation and Intervention: 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 131I-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. Conclusion: 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.

Copyright © 2006 S. Karger AG, Basel

 goto top of outline References
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    External Resources

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 goto top of outline Author Contacts

Dr. Cihangir Erem
Karadeniz Technical University Tıp Fakültesi
İç Hastalıkları Anabilim Dalı
TR–61080 Trabzon (Turkey)
Tel. +90 462 377 5449, Fax +90 462 325 2270, E-Mail

 goto top of outline Article Information

Received: March 21, 2005
Revised: November 28, 2005
Number of Print Pages : 5
Number of Figures : 3, Number of Tables : 0, Number of References : 12

 goto top of outline Publication Details

Medical Principles and Practice (International Journal of the Kuwait University Health Sciences Centre)

Vol. 15, No. 5, Year 2006 (Cover Date: August 2006)

Journal Editor: Al-Zaid, N.S. (Kuwait)
ISSN: 1011–7571 (print), 1423–0151 (Online)

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