Carotid body paraganglioma: review and surgical management
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The term paraganglia best defines the spread in the body of clusters of cells with histological and cytochemical characteristics of neuroendocrine cells originating from the neural crest, with either sympathetic or parasympathetic function. Carotid body hyperplasia is associated with long-standing hypoxia as in native inhabitants in high altitude or in patients with chronic pneumonopathies, while carotid body paraganglioma is a rare parasympathetic tumor with significant morbidity. Tumor extension per se, associated cranial nerve involvement, and the estimated 3–10% malignant potential, particularly at a young age, make early diagnosis and treatment of carotid body paraganglioma mandatory. Biopsy should be avoided, because of the accompanying incidence of hemorrhage, while it is essential in all cases to investigate both sides of the neck to exclude bilateral tumors. The modalities of therapy include preoperative embolization, preoperative and postoperative radiation 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 reliable 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.
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