Craniocervical paragangliomas - literature review and review of 39 patients

Abstract

INTRODUCTION. Craniocervical paragangliomas are rare tumors. These paragangliomas are most commonly found in the carotid-body, middle ear and in the course of n.vagus. The overwhelming majority of these tumors are benign.

MATERIALS AND METHODS. The patient material consists of a total of 39 patients. During the period 1985-2009 36 patients were treated for craniocervical paragangliomas at Rikshospitalet. In addition, three patients in the patient material were treated at Ullevål University Hospital. The patients had a total of 46 tumors. In the patient material there were 27 women and 12 men, sex: ratio 2,25:1. There were 19 carotid body paragangliomas, 12 jugular paragangliomas, nine tympanic paragangliomas, two vagal paragangliomas, two paragangliomas in gl.thyroidea, one paraganglioma in gl.parotis and one laryngeal paraganglioma.

OBJECTIVE. The objective of this retrospective study and review of the literature is evaluation of diagnosis, radiological evalutation and treatment of paragangliomas in the head and neck area.

RESULTS. Median age at the time of diagnosis was 52 years. Five patients (12.8 percent) had multiple tumors, while three patients (7.7 percent) had familial paraganglioma. Three of the total of 46 tumors were malignant (6.5 percent). Preoperatively, there were eight patients in the patient material that had affection of the cranial nerves (20.5 percent), with clear predominance concerned the patients with jugular paragangliomas. Postoperatively, there were among ten of the total 25 patients (40.0 percent) who were subjected to surgery affection of the cranial nerves. Eight patients had postoperative affection of n.vagus, which was the most frequent postoperative cranial nerve affection. Of the 46 tumors in the patient material 25 were subjected to surgery (54.3 percent), 18 were irradiated (39.1 percent) and six were initially found suitable for expectant management (13.0 percent).

CONCLUSION. The treatment modality is determined by the number of paragangliomas each patient has; localization; risk of cranial nerve damage; comorbidity and age. The aim is to avoid neurovascular morbidity and limiting tumor growth. Radiation may be equal to surgery, but surgery is preferred when there are solitary paragangliomas, absence of severe comorbidity and low probability of affection of cranial nerves during surgery. Genetic testing should be considered in patients with head and neck paragangliomas. Fluorodopa-PET should be considered in the diagnosing process.