Malignant head/neck paragangliomas. Comparative study.
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.


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 (18)F-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.
Service de médecine nucléaire, hôpital Lariboisière (Assistance Publique-Hôpitaux de Paris), 2, rue Ambroise-Paré, 75010 Paris, France.
A
A
Mediouni
Service de médecine nucléaire, hôpital Lariboisière (Assistance Publique-Hôpitaux de Paris), 2, rue Ambroise-Paré, 75010 Paris, France.
S
S
Ammari
Service de cytologie et d'anatomie pathologique, hôpital Lariboisière (Assistance Publique-Hôpitaux de Paris), 2, rue Ambroise-Paré, 75010 Paris, France; Université Paris-Diderot Paris-7, 75205 Paris cedex 13, France.
M
M
Wassef
Service de génétique, hôpital européen Georges-Pompidou (Assistance Publique-Hôpitaux de Paris), 20, rue Leblanc, 75015 Paris, France.
A-P
AP
Gimenez-Roqueplo
Service de radiologie, hôpital Lariboisière (Assistance Publique-Hôpitaux de Paris), 2, rue Ambroise-Paré, 75010 Paris, France; Université Paris-Diderot Paris-7, 75205 Paris cedex 13, France.
J-D
JD
Laredo
Service de médecine nucléaire, hôpital Lariboisière (Assistance Publique-Hôpitaux de Paris), 2, rue Ambroise-Paré, 75010 Paris, France; Université Paris-Diderot Paris-7, 75205 Paris cedex 13, France.
M
M
Duet
2, rue Saint-Petersbourg, 75008 Paris, France.
P
P
Tran Ba Huy
Service d'otorhinolaryngologie, hôpital Lariboisière (Assistance Publique-Hôpitaux de Paris), 2, rue Ambroise-Paré, 75010 Paris, France; Université Paris-Diderot Paris-7, 75205 Paris cedex 13, France. Electronic address: n.oker@hotmail.fr.
N
N
Oker
D000293:Adolescent; D000328:Adult; D000368:Aged; D001859:Bone Neoplasms / Q000556:secondary / Q000628:therapy; D003952:Diagnostic Imaging; D005260:Female; D005820:Genetic Testing; D006258:Head and Neck Neoplasms / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D006801:Humans; D008113:Liver Neoplasms / Q000556:secondary / Q000628:therapy; D008175:Lung Neoplasms / Q000556:secondary / Q000628:therapy; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D009154:Mutation; D037981:Neck Dissection; D009364:Neoplasm Recurrence, Local; D009378:Neoplasms, Multiple Primary / Q000473:pathology; D010235:Paraganglioma / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D012189:Retrospective Studies; D017678:Sex Distribution; D013385:Succinate Dehydrogenase / Q000235:genetics; D013964:Thyroid Neoplasms / Q000556:secondary / Q000628:therapy; D055815:Young Adult
C491545:SDHD protein, human; C108095:SDHB protein, human; D013385:Succinate Dehydrogenase
(18)FDG-PET; Malignant head and neck paraganglioma; Metastases; Octreoscan; SDHx mutation