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.

Keywords:
Familial pheochromocytoma, Carotid body tumor, Turkish family
This content is only available via PDF.
© 2006 S. Karger AG, Basel
2006
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.