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

Background. Cervical paragangliomas are uncommon benign or malignant neopla sms, deriving from stem cells of the neural crest. Compared to all the tumo rs of the head and neck, they occur less frequently. They can be found in a ny part of the body where there are sympathetic ganglia including chemorece ptors, suprarenal medulla, retroperitoneal ganglia and the extreme branches of the vagus nerves. It is not easy nowadays to define properly their biol ogical behaviour, the possible multicentric location and the association wi th Multiple Endocrine Neoplasms; this is considered particularly important and occurs in 42 per cent of the cases of familial neoplasms of the paragan glion system. Methods. After a review of recent diagnostic, pathological and clinical fin dings, the authors report their experience, between 1970-1995, of 10 patien ts affected by sporadic paraganglioma and 1 by familial multicentric neopla sm localised in the carotid bodies of both sides, left vagus nerve and left hypoglossus nerve. All patients but one were treated by a curative resecti on 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 bloo d pressure and catecholamine values in all patients. The complications were a recurrential palsy in a patient operated on for vagal paraganglioma; a r ecurrential palsy and temporary dysarthria in the patient affected by multi ple familial paraganglioma; another patient operated on for carotid body pa raganglioma showed a cerebral ischemic lesion which caused a slightly trans itory facial-brachial motor deficit on the right side and speech impairment . Conclusions. We can venture to say that any type of cervical, mediastinal o r retroperitoneal swelling in persons belonging to a genetically prone fami ly must be first of all considered a possible paraganglioma, For this reaso n the patient with more than one growth of this type, whether synchronous o r not, must undergo genetic investigation, along with the rest of his famil y.