Surgical treatment of paragangliomas of the carotid body and other rare localisations

Background. Cervical paragangliomas are uncommon benign or malignant neoplasms, deriving from stem cells of the neural crest. Compared to all the tumors of the head and neck, they occur less frequently. They can be found in any part of the body where there are sympathetic ganglia including chemoreceptors, suprarenal medulla, retroperitoneal ganglia and the extreme branches of the vagus nerves. It is not easy nowadays to define properly their biological behaviour, the possible multicentric location and the association with Multiple Endocrine Neoplasms; this is considered particularly important and occurs in 42 per cent of the cases of familial neoplasms of the paraganglion system. Methods. After a review of recent diagnostic, pathological and clinical findings, the authors report their experience, between 1970-1995, of 10 patients affected by sporadic paraganglioma and 1 by familial multicentric neoplasm localised in the carotid bodies of both sides, left vagus nerve and left hypoglossus nerve. All patients but one were treated by a curative resection of the neoplasm, In one case only an explorative laparatomy was possible because of visceral and vascular involvement. Results, There is no mortality. There are no modifications in arterial blood pressure and catecholamine values in all patients. The complications were a recurrential palsy in a patient operated on for vagal paraganglioma; a recurrential palsy and temporary dysarthria in the patient affected by multiple familial paraganglioma; another patient operated on for carotid body paraganglioma showed a cerebral ischemic lesion which caused a slightly transitory facial-brachial motor deficit on the right side and speech impairment. Conclusions. We can venture to say that any type of cervical, mediastinal or retroperitoneal swelling in persons belonging to a genetically prone family must be first of all considered a possible paraganglioma, For this reason the patient with more than one growth of this type, whether synchronous or not, must undergo genetic investigation, along with the rest of his family.