Carotid body paraganglioma: review and surgical management

The term paraganglia best defines the spread in the body of clusters of cel ls with histological and cytochemical characteristics of neuroendocrine cel ls originating from the neural crest. with either sympathetic or parasympat hetic function. Carotid body hyperplasia is associated with long-standing h ypoxia as in native inhabitants in high altitude or in patients with chroni c pneumonopathies. while carotid body paraganglioma is a rare parasympathet ic tumor with significant morbidity. Tumor extension per se, associated cra nial nerve involvement, and the estimated 3-10% malignant potential. partic ularly at a young: age, make early diagnosis and treatment of carotid body paraganglioma mandatory. Biopsy should be avoided, because of the accompany ing incidence of hemorrhage, while it is essential in all cases to investig ate both sides of the neck to exclude bilateral tumors. The modalities of t herapy include preoperative embolization, preoperative and postoperative ra diation with ambiguous results of both, and complete surgical removal which constitutes the definite therapy. The current stroke rate is less than 5%. The histological appearance of a carotid body paraganglioma is not a relia ble guide to its propensity for malignant behavior and recurrences are most likely to appear in patients who have multiple paragangliomas or a family history of carotid body paraganglioma.