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RESEARCH PRODUCT
Head and neck paragangliomas: Report of 175 patients (1989-2010)
Torsten MewesOliver BartschKarl J. LacknerKonstantinos PapaspyrouRonald G. AmedeeHeidi RossmannBrigitte Schneider-raetzkeWolf J. MannMathias SchreckenbergerChristian Fottnersubject
MaleOncologyHeterozygotemedicine.medical_specialtyPathologyMEDLINEMalignancyNeoplasms Multiple PrimaryParagangliomaInternal medicineCarcinomamedicineHumansHead and neckRetrospective Studiesbusiness.industryRetrospective cohort studyHeterozygote advantagemedicine.diseaseCarcinoma PapillarySuccinate DehydrogenaseOtorhinolaryngologyHead and Neck NeoplasmsVon Hippel-Lindau Tumor Suppressor ProteinMutationMutation (genetic algorithm)Femalebusinessdescription
Attention of the otorhinolaryngologist needs to be drawn to the versatile aspects of head and neck paragangliomas (PGLs).This study is a retrospective, nonrandomized clinical study of all 175 individuals with PGLs treated in our department between 1989 and 2010. A genetic analysis was performed on 86 patients.The 175 patients presented 224 head and neck PGLs as well as 2 thyroid papillary carcinomas. Genetic analysis resulted in 1 patient positive for a von Hippel-Lindau (VHL) gene mutation and 34 for succinate dehydrogenase (SDH) gene mutations (22 SDHD, 7 SDHC, and 5 SDHB), 12 of the latter carrying a novel mutation. Thirty-three patients (18.9%) had multiple PGLs and 11 patients (6.3%) had a malignant paraganglioma. SDH-mutation carriers had multiple tumors in 64.7% and malignant paragangliomas in 20.6%.Multifocal occurrence, potential malignancy, genetic aspects, possible coincidence of thyroid carcinoma, and hormone production have to be considered in patients with head and neck PGLs.
year | journal | country | edition | language |
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2011-02-21 | Head & Neck |