Paraganglioma as a systemic syndrome: pitfalls and strategies

Tumours of the neuroendocrine system in the head and neck region are mostly paragangliomas of the glomus tympanicum or jugulare, or of the carotid bod y. The majority of these tumours are benign, and the coexistence of multipl e paragangliomas seems to be rare. Pre-operative embolization and surgery a re regarded as primary therapy for these tumours. The treatment regimen in any patient depends on age, general health, hearing status and the function of the lower cranial nerves. Several presentations are possible in which p aragangliomas occur as systemic disease. 1. Paragangliomas may occur bilaterally, or, in rare cases, in multiple are as. Pre-operative bilateral angiography is of utmost importance. In case of multicentricity, it might be necessary to proceed without, or just with, u nilateral surgery for preservation of adjacent structures. In surgery of ju gular vein paraganglioma, we usually perform a modified transmastoidal and transcervical approach with preservation of middle-ear structures and the o ssicles. As an alternative or supplement to surgery, radiotherapy or defini tive embolization may be used in the treatment of paragangliomas. 2. Paragangliomas may occur as multiple endocrine neoplasia (MEN) syndrome combined with medullary thyroid gland carcinoma, and, facultatively, pheoch romocytoma In these cases, endocrinological examination and magnetic resona nce imaging (MRI) of the adrenal region, the thorax and the neck are requir ed for an adequate therapeutic strategy. As MEN may be inherited, family hi story should be evaluated. 3. Paragangliomas can became malignant and metastasize. Thus, cervical lymp h node metastases or distant metastases may occur. We recommend the removal of all ipsilateral lymph nodes and their histological examination.