RESUMO
INTRODUCTION AND OBJECTIVE: To report a single center experience with carotid body paraganglioma cases that were treated by the same surgeon in a city with high prevalence of paragangliomas due to high altitude. METHODS: We retrospectively investigated the demographic, clinicopathological and radiological data of 104 patients diagnosed with cervical paragangliomas between 2003 and 2017. The patients were classified according to the Shamblin classification. RESULTS: In this study a total of 104 patients (33 male and 71 female, with a mean age of 54.6±13 years) diagnosed with cervical paragangliomas located on carotid bifurcation between 2003 and 2017 were included. Among those patients, 10 presented with bilateral tumors and in total, 114 paragangliomas were managed in this period. The mean diameter of the tumors was 5.12±1.45cm. Malignant tumor was determined in only one (0.9%) patient. All patients were operated. In 12 patients with the tumor diameter larger than 5cm, preoperative coil embolization was achieved. In 14 patients, preoperative angiographic embolization was employed and in 4 patients intraoperative sclerosing agent injections were performed. Facial paralysis was observed in 2 patients and dysphagia was present in 1 patient, Horner syndrome was seen in 1 patient and hoarseness was reported in 7 patients after operation. All those complications improved during follow-up. Mortality was not reported in any cases. CONCLUSION: Surgery is the definitive treatment for patients with cervical paragangliomas. Although, it may be difficult in patients with the advanced Shamblin types, in experienced hands, complication rates are very low.
Assuntos
Tumor do Corpo Carotídeo , Embolização Terapêutica , Paraganglioma , Adulto , Idoso , Tumor do Corpo Carotídeo/diagnóstico por imagem , Tumor do Corpo Carotídeo/cirurgia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Pescoço , Paraganglioma/diagnóstico por imagem , Paraganglioma/cirurgia , Estudos Retrospectivos , Resultado do TratamentoRESUMO
Head and neck paragangliomas are tumors arising from specialized neural crest cells. Prominent locations are the carotid body along with the vagal, jugular, and tympanic glomus. Head and neck paragangliomas are slowly growing tumors, with some carotid body tumors being reported to exist for many years as a painless lateral mass on the neck. Symptoms depend on the specific locations. In contrast to paraganglial tumors of the adrenals, abdomen and thorax, head and neck paragangliomas seldom release catecholamines and are hence rarely vasoactive. Petrous bone, jugular, and tympanic head and neck paragangliomas may cause hearing loss. The internationally accepted clinical classifications for carotid body tumors are based on the Shamblin Class I-III stages, which correspond to postoperative permanent side effects. For petrous-bone paragangliomas in the head and neck, the Fisch classification is used. Regarding the molecular genetics, head and neck paragangliomas have been associated with nine susceptibility genes: NF1, RET, VHL, SDHA, SDHB, SDHC, SDHD, SDHAF2 (SDH5), and TMEM127. Hereditary HNPs are mostly caused by mutations of the SDHD gene, but SDHB and SDHC mutations are not uncommon in such patients. Head and neck paragangliomas are rarely associated with mutations of VHL, RET, or NF1. The research on SDHA, SDHAF2 and TMEM127 is ongoing. Multiple head and neck paragangliomas are common in patients with SDHD mutations, while malignant head and neck paraganglioma is mostly seen in patients with SDHB mutations. The treatment of choice is surgical resection. Good postoperative results can be expected in carotid body tumors of Shamblin Class I and II, whereas operations on other carotid body tumors and other head and neck paragangliomas frequently result in deficits of the cranial nerves adjacent to the tumors. Slow growth and the tendency of hereditary head and neck paragangliomas to be multifocal may justify less aggressive treatment strategies.