Malignant head/neck paragangliomas. Comparative Study
| Autor: | J.-D. Laredo, M. Duet, Michel Wassef, P. Tran Ba Huy, S. Ammari, Anne-Paule Gimenez-Roqueplo, A. Mediouni, N. Oker |
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| Rok vydání: | 2014 |
| Předmět: |
Male
18FDG-PET Lung Neoplasms medicine.medical_treatment Metastases Neoplasms Multiple Primary chemistry.chemical_compound Paraganglioma Epidemiology Young adult Family history Malignant head and neck paraganglioma Liver Neoplasms Middle Aged 3. Good health SDHx mutation Succinate Dehydrogenase Octreoscan Head and Neck Neoplasms Lymphatic Metastasis Neck Dissection Female Radiology Adult Diagnostic Imaging medicine.medical_specialty Adolescent Bone Neoplasms Malignancy Young Adult medicine Humans Genetic Testing Thyroid Neoplasms Sex Distribution Metanephrine Aged Retrospective Studies business.industry Neck dissection Retrospective cohort study medicine.disease Surgery Otorhinolaryngology chemistry Mutation Neoplasm Recurrence Local business |
| Zdroj: | European Annals of Otorhinolaryngology, Head and Neck Diseases. 131:159-166 |
| ISSN: | 1879-7296 |
| Popis: | Summary Background The objective of this study was to report 11 cases of malignant head and neck paraganglioma and to compare their epidemiological, clinical, and genetic characteristics, their natural history and their treatment with those of a series of 131 benign paragangliomas. Patients and methods Retrospective analysis of 142 patients with head and neck paraganglioma managed between 2001 and 2008. Age at the time of diagnosis, gender, primary tumour site, presence of other non-head/neck paragangliomas and/or metastases diagnosed by imaging (CT, MRI, Octreoscan or 18F-FDG PET), histology, urinary catecholamine and metanephrine levels, family history, and genetic test results were recorded. Results This series comprised 131 benign head and neck paragangliomas, mostly observed in women with a mean age at diagnosis of 45 years and a predominance of tympanojugular sites (followed by carotid and vagal sites) with 5% of secreting tumours and 20% of multifocal tumours. Eleven patients (7.7%) with a 1:1 sex ratio presented criteria of malignancy. These patients, with a lower mean age (38 years), predominantly presented carotid lesions with a higher rate of secreting and multifocal tumours, 27% and 46% respectively. The main sites of metastases were bone and lymph nodes. No tympanic paragangliomas were observed. Conclusions Malignant paragangliomas are mainly observed in young patients with multifocal tumours, particularly carotid tumours, and are predominantly related to subunit SDH-B mutation. The work-up in these high-risk patients must include whole body scintigraphy and spine MRI. Malignancy is not necessarily associated with a poor short-term prognosis due to the slow course of the disease. |
| Databáze: | OpenAIRE |
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