Artikel
Paragangliomas of the head and neck: a report on 163 patients (1989–2009)
Suche in Medline nach
Autoren
Veröffentlicht: | 6. Juli 2010 |
---|
Gliederung
Text
Introduction: Paragangliomas (PGL) of the head and neck are rare, mostly benign tumors. They can arise multifocally and show in 1–3% clinical signs of hormonal activity. 10% to 15% of paragangliomas are caused by mutations in the succinate dehydrogenase (SDH) genes B, C, or D.
Methods: Retrospective study of 163 patients with 206 head and neck PGL, including 59 carotid body, 23 vagal, 83 jugulotympanic, 28 tympanic tumors and 13 PGLs in other locations in the head and neck area, 28 PGLs outside the head and neck area and two papillary thyroid carcinomas. Genetic analysis could be performed on 69 patients.
Results: One patient was found positive for a mutation of von Hippel Lindau (VHL) gene and 30 for succinate dehydrogenase (SDH) gene mutations (19 in SDHD, 7 in SDHC and 4 in SDHB), 16 of the latter carrying a novel mutation. Thirty-three patients (20%) had multiple PGLs and ten (6%) a malignant paraganglioma. Patients with familial mutations had multiple tumors in 61% and malignant tumors in 16%.
Conclusion: Multifocal occurrence, malignancy, hormone production, potential coincidence of thyroid and renal carcinoma and genetic background have to be considered in patients with PGLs of the head and neck.