Paragangliomas-- a decade of clinical experience.

BACKGROUND AND OBJECTIVES: This study is a review of 14 patients with paragangliomas between 1986 and 1996. The purpose was to determine the sites of origin, clinical manifestations and analyze the benefits of different treatment modalities.
METHODS: There were 20 tumors in 14 patients. Three (21.0%) of the patients had familial history. There were 7 (50%) females and 7 (50%) males. Anatomically 14 (70%) tumors were in head and neck, 5 (25%) were in the retroperitoneum, one (5%) was in the heart. Of the head and neck tumors 9 (64.25%) were in the carotid body, 3 (21.42%) were found in the vagus, and 2(14.33%) were found in the middle ear. The tumor found in the heart was in the atrial septum. The clinical behavior of paragangliomas is determined by cellular characteristics, secreting capabilities and tumor location. The symptoms and signs depend on the site of origin and the stage at which it presents. The clinically functioning tumors were 3 (17%) in our experience and they typically present with uncontrolled hypertension. The carotid body and mediastinal tumors usually manifested as asymptomatic masses. The intravagal tumors presented with paresis of the nerve. Malignancy rarely occurs and is defined by the existence of metastasis rather than by histology. In our series 2 (10%) of the patients presented with metastasis to lymph nodes, and the vertebrae. The diagnoses in our patients were established by CT and MRI scanning. Angiography was performed in 5 patients with carotid body tumor, two of whom underwent therapeutic embolization to reduce the tumor size. The mainstay of treatment was surgical removal, though radiation has been advocated for patients who cannot undergo surgery.
RESULTS: All patients underwent successful surgical resection of the tumor after appropriate preoperative preparation. Late mortality occurred in two (12.5%) patients at 3 and 5 years from unrelated etiology. Four (25%) patients were lost to follow-up. Three (18.7%) patients developed new primaries, two of them at two years and one after 8 years. One (6%) patient developed recurrent paraganglioma after remaining disease free for 20 years.
CONCLUSION: In conclusion, paragangliomas are rare with multicentricity being more common in patients with familial history. The malignant potential of the tumor is determined by metastasis as there are no characteristic cellular change. Aggressive surgery is mandatory to obtain disease free survival with low morbidity and mortality. Recurrences can also be successfully operated with low morbidity. Copyright 2000 Wiley-Liss, Inc.