[Endocrine surgery for neck paraganglioma: operation, radiation therapy or wait and scan?].

BACKGROUND: Head and neck paraganglioma (HNP) represent rare endocrine tumors. Therapy is decided on genetic findings, tumor characteristics (e.g. tumor size, localization and dignity), age of patient and symptoms. In terms of local control radiation therapy is as equally effective as surgery but surgical morbidity rates secondary to cranial nerve injuries remain high. PATIENTS: Based on 6 patients with 11 solitary (4 patients) and multiple (2 patients) HNP (8 carotid body tumors, 1 vagal, 1 jugular and 1 jugulotympanic paraganglioma) the specific characteristics of the need for surgery as well as correct choice of treatment in cases of sporadic succinate dehydrogenase (SDH) negative and hereditary SDH positive HNP will be exemplarily demonstrated. RESULTS: A total of 6 carotid body tumors (four sporadic, two hereditary) were resected in 4 patients, five as primary surgery and one as a revision procedure. In one case a preoperative embolization was performed 24 h before surgery. Malignancy could not be proven in any patient. The 30-day mortality was zero. In the patient with bilateral hereditary carotid body tumors, unilateral local recurrent disease occurred. After resection of the recurrent tumor permanent unilateral paralysis of the laryngeal nerve, glossopharyngeal nerve and hypoglossal nerve occurred. All patients were followed-up postoperatively for a mean of 64 months (range 23-78 months) with a local tumor control rate of 100%. The overall survival rate after 5 years was 100%. CONCLUSIONS: Given a very strict indication with awareness of surgical risks selective surgery has a key position with low postoperative morbidity in the treatment of HNPs. We prefer surgery for small unilateral paraganglioma, malignant or functioning tumors.