caa a22 4500 388085584 CHVBK 20180307125229.0 cr unu---uuuuu 161130e199911 xx s 000 0 eng 10.1017/S0022215100145761 doi S0022215100145761 pii (NATIONALLICENCE)cambridge-10.1017/S0022215100145761 Paraganglioma as a systemic syndrome: pitfalls and strategies [Elektronische Daten] Tumours of the neuroendocrine system in the head and neck region are mostly paragangliomas of the glomus tympanicum or jugulare, or of the carotid body. The majority of these tumours are benign, and the coexistence of multiple paragangliomas seems to be rare. Pre-operative embolization and surgery are 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 paragangliomas occur as systemic disease. 1. Paragangliomas may occur bilaterally, or, in rare cases, in multiple areas. Pre-operative bilateral angiography is of utmost importance. In case of multicentricity, it might be necessary to proceed without, or just with, unilateral surgery for preservation of adjacent structures. In surgery of jugular vein paraganglioma, we usually perform a modified transmastoidal and transcervical approach with preservation of middle-ear structures and the ossicles. As an alternative or supplement to surgery, radiotherapy or definitive 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, pheochromocytoma In these cases, endocrinological examination and magnetic resonance imaging (MRI) of the adrenal region, the thorax and the neck are required for an adequate therapeutic strategy. As MEN may be inherited, family history should be evaluated. 3. Paragangliomas can became malignant and metastasize. Thus, cervical lymph node metastases or distant metastases may occur. We recommend the removal of all ipsilateral lymph nodes and their histological examination. Copyright © JLO (1984) Limited 1999 Paraganglioma nationallicence Thyroid gland neoplasms nationallicence Endocrine gland neoplasms nationallicence Maier W. Marangos N. Laszig R. The Journal of Laryngology & Otology Cambridge University Press 113/11(1999-11), 978-982 0022-2151 113:11<978 1999 113 JLO https://doi.org/10.1017/S0022215100145761 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1017/S0022215100145761 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Maier W. NATIONALLICENCE 700 1- Marangos N. NATIONALLICENCE 700 1- Laszig R. NATIONALLICENCE 773 0- The Journal of Laryngology & Otology Cambridge University Press 113/11(1999-11), 978-982 0022-2151 113:11<978 1999 113 JLO CC0 http://creativecommons.org/publicdomain/zero/1.0 nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-cambridge