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Paraganglioma as a systemic syndrome: pitfalls and strategies

Published online by Cambridge University Press:  29 June 2007

W. Maier ,
N. Marangos  and
R. Laszig
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Abstract

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.

Keywords

ParagangliomaThyroid gland neoplasmsEndocrine gland neoplasms
Type
Main Articles
Information
The Journal of Laryngology & Otology , Volume 113 , Issue 11 , November 1999 , pp. 978 - 982
Copyright
Copyright © JLO (1984) Limited 1999

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Footnotes

Presented in part on the 4th European Skull Base Society Congress, Nuernberg, Germany, May 19-22, 1999. Dedicated to Prof. C. Beck, former head of the Department of Oto-Rhino-Laryngology, University of Freiburg, and to Prof. E. Lehnhardt, former head of the Department of Oto-Rhino-Laryngology, Medical School Hannover.

References

Balatsouras, D. G., Eliopoulos, P. N., Economou, C. N. (1992) Multiple glomus tumours. Journal of Laryngology and Otology 106: 538–543.CrossRefGoogle ScholarPubMed
Fisch, U., Mattox, D. (1988) Microsurgery of the Skull Base. 1st Edition. Thieme, Stuttgart – New York.Google Scholar
Fuchshuber, P. R., Loree, T. R., Hicks, W. L., Cheney, R. T., Shedd, D. P. (1998) Medullary carcinoma of the thyroid: prognostic factors and treatment recommendations. Annals of Surgical Oncology 5: 81–86.CrossRefGoogle ScholarPubMed
Gimm, O., Dralle, H. (1997) Reoperation in metastasizing medullary thyroid carcinoma: is a tumour stage-oriented approach justified? Surgery 122: 1124–1130.CrossRefGoogle ScholarPubMed
Giuffrida, D., Gharib, H. (1998) Current diagnosis and management of medullary thyroid carcinoma. Annals of Oncology 9: 685–701.CrossRefGoogle ScholarPubMed
Gosepath, J., Welkoborsky, H. J., Mann, W. (1998) Studies on the tumour biology and growth rate in jugulotympanic and carotic glomus tumours. Laryngo-Rhino-Otologie 77: 429–433.CrossRefGoogle Scholar
Held, P., Breit, A. (1994) Methodenvergleich von CT und MRT in der Diagnostik von Tumoren des Para- und Retropharyngealraumes und des Schläfenbeines. Bildgebung 61: 263–271.Google Scholar
Herdman, R. C. D., Gillespie, J. E., Ramsden, R. T. (1993) Facial palsy after glomus tumour embolization. Journal of Laryngology and Otology 107: 963–966.CrossRefGoogle ScholarPubMed
Hirano, S., Shoji, K., Kojima, H., Omori, K. (1998) Dopaminesecreting carotid body tumour. American Journal of Otolaryngology 19: 412–416.CrossRefGoogle Scholar
Kennedy, D. W., Nager, G. T. (1986) Glomus tumour and multiple endocrine neoplasia. Otolaryngology – Head and Neck Surgery 94: 644–648.CrossRefGoogle ScholarPubMed
Kim, J. A., Elkon, D., Lim, M. L., Constable, W. C. (1980) Optimum dose of radiotherapy for chemodectomas of the middle ear. International Journal of Radiation Oncology Biology Physics 6: 815–819.CrossRefGoogle ScholarPubMed
Marangos, N., Schumacher, W. (1998) Facial palsy after glomus jugulare tumour embolization. Journal of Laryngology and Otology 113: 268–270.CrossRefGoogle Scholar
Milewski, C. (1993) Morphological and clinical aspects of head and neck paraganglioma. HNO 41: 526–531.Google Scholar
Milewski, C., Eimannsberger, K., Pflughaupt, K. W. (1993) Does surgical manipulation cause hormone release in paraganglioma of head and neck? – A serum NSE study. Laryngo-Rhino-Otologie 72: 252–255.CrossRefGoogle Scholar
Parkin, J. L. (1981) Familial multiple glomus tumours and pheochromocytomas. Journal of Otology 90: 60–63.Google ScholarPubMed
Schick, B., Draf, W., Kahle, G. (1998) Development of therapy concepts of jugulotympanic paragangliomas. Laryngo-Rhino-Otologie 77: 434–443.CrossRefGoogle Scholar
Schwaber, M. K., Glasscock, M. E., Nissen, A. J., Jackson, C. G., Smith, P. J. (1984) Diagnosis and management of catecholamine secreting glomus tumours. Laryngoscope 94: 1008–1015.CrossRefGoogle Scholar
Sillars, H. A., Fagan, P. A. (1993) The management of multiple paraganglioma of the head and neck. Journal of Laryngology and Otology 107: 538–543.CrossRefGoogle ScholarPubMed
Strauss, M., Nicholas, G. G., Abt, A. B., Harrison, T. S., Seaton, J. F. (1983) Malignant catecholamine-secreting carotid body paraganglioma. Otolaryngology – Head and Neck Surgery 91: 315–321.CrossRefGoogle ScholarPubMed
Valavanis, A. (1986) Preoperative embolization of the head and neck: Indication, patient selection, goals and precautions. American Journal of Neuroradiology 7: 943–952.Google ScholarPubMed
Walsh, R. M., Leen, E. J., Path, M. R. C., Gleeson, M. J., Shaheen, O. H. (1997) Malignant vagal paraganglioma. Journal of Laryngology and Otology 111: 83–88.CrossRefGoogle ScholarPubMed
Zbaren, P., Lehmann, W. (1985) Carotid body paraganglioma with metastases. Laryngoscope 95: 450–454.CrossRefGoogle ScholarPubMed