Familial Paraganglioma of Head and Neck

Abstract

Paragangliomas are tumors originated from non-chromaffin paranganglia cells derived from the neural crest. These highly vascular tumors are located in the region which extends from skull base to arcus aorta. Head and neck paragangliomas have rare occurence constituting 0.012% of all the body tumors and 0.6% of head and neck tumors. Head and neck paragangliomas occur in sporadic and familial forms. The ratio of familial head and neck paragangliomas is about 10%. Familial form has a characteristic of autosomal dominant inheritance. In this article, four cases having first and second degree of affinity that we followed for paraganglioma diagnosis are presented.

Keywords

• Familial, paraganglioma, head, neck

Familial Paraganglioma of Head and Neck

Öz

Paragangliyomalar nöral krest kökenli non-kromafin paragangliyon hücrelerinden kaynaklanan tümörlerdir. İleri derecede vasküler olan bu tümörler kafa tabanından arkus aortaya kadar uzanan alanda bulunur. Baş boyun paragangliyomaları nadir görülür ve tüm vücut tümörlerinin %0.012’sini, baş-boyunda görülen tümörlerin ise %0.6’sını oluşturur. Baş boyun paragangliyomaları sporadik ve ailesel olmak üzere iki formda görülür. Ailesel baş boyun paragangliyomalarının oranı yaklaşık %10’dur. Ailesel form otozomal dominant geçiş özelliğine sahiptir. Bu yazıda aralarında birinci veya ikinci derece akrabalık bağı olan paraganglioma tanısı ile takip ettiğimiz 4 olgu sunulmuştur

Anahtar Kelimeler

• Ailesel, paragnaglioma, baş, boyun

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