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Increased prevalence of catecholamine excess and phaeochromocytomas in a well-defined Dutch population with SDHD-linked head and neck paragangliomas

Leiden University Medical Center,¹ Departments of Endocrinology and Metabolic Diseases, ² Otorhinolaryngology, ³ Center of Human and Clinical Genetics ⁴ Clinical Chemistry, ⁵ Nuclear Medicine and ⁶ Pathology, Leiden, The Netherlands

(Correspondence should be addressed to E P M Corssmit, Leiden University Medical Center, Department of Endocrinology and Metabolic Diseases, Albinusdreef 2, PO Box 9600, 2300 RC, Leiden, The Netherlands; Email: e.p.m.corssmit{at}lumc.nl)

Objective: The aim of this study was to identify the prevalence of catecholamine excess and phaeochromocytomas in a well-defined population of people with hereditary head and neck paragangliomas.

Methods: We studied in a prospective follow-up protocol all consecutive patients referred to the Department of Endocrinology, Leiden University Medical Center, Leiden, The Netherlands with documented head and neck paragangliomas and either a positive family history for paragangliomas or a proven SDHD gene mutation. Initial analysis included medical history, physical examination and the measurement of excretion of catecholamines in two 24-h urine collections. In the case of documented catecholamine excess iodinated meta-iodobenzylguanidine (¹²³I-MIBG) scintigraphy and magnetic resonance imaging were done.

Results: Between 1988 and 2003, 40 consecutive patients (20 male and 20 female) with documented head and neck paragangliomas were screened. Biochemical screening revealed urinary catechol-amine excess in 15 patients (37.5%). In nine of these 15 patients a lesion was found by ¹²³I-MIBG scintigraphy. Exact localization by magnetic resonance imaging revealed phaeochromocytomas in seven of the 15 patients. One of the nine patients had an extra-adrenal paraganglioma. Histopathological examination in a subset of tumors displayed loss of heterozygosity of the wild-type SDHD allele in all cases.

Conclusions: The prevalence of catecholamine excess (37.5%) and phaeochromocytomas (20.0%) is high in patients with familial head and neck paragangliomas. Therefore, patients with hereditary head and neck paragangliomas require lifelong follow up by biochemical testing for catecholamine excess.

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