副神经节瘤综合征合并恶性副神经节瘤的罕见病例
[Uncommon case of paraganglioma syndrome in combination with malignant paraganglioma].
作者信息
Schmahl K, Atamna N, Schönijahn T, Lülsdorf P, Göller T, Jacob R
机构信息
Abt. XIII, Pathologie, Bundeswehrzentralkrankenhaus Koblenz.
出版信息
Laryngorhinootologie. 2008 Feb;87(2):112-7. doi: 10.1055/s-2007-966892.
BACKGROUND
Combination of paraganglioma syndrome and malignant paraganglioma is an uncommon disease worldwide.
PATIENT
We report the case of a 69-year old man with a jugular-tympanal paraganglioma, who underwent surgery. Histopathological examination revealed a malignant paraganglioma. An other contralateral carotid-body and a tumor in the thyroid gland had been discovered during staging. The molecular results confirmed the existence of a paraganglioma syndrome.
DISCUSSION
A malignant paraganglioma based on a hereditary paraganglioma syndrome is a rare described case in literature. On the background of the literature we discuss the pathology, pathogenesis, diagnostic and therapy of this disease.
背景
副神经节瘤综合征与恶性副神经节瘤并存的情况在全球范围内均属罕见疾病。
患者
我们报告了一例69岁患有颈静脉鼓室副神经节瘤的男性患者，该患者接受了手术治疗。组织病理学检查显示为恶性副神经节瘤。在分期检查过程中还发现了对侧的另一个颈动脉体瘤以及甲状腺内的一个肿瘤。分子检测结果证实存在副神经节瘤综合征。
讨论
基于遗传性副神经节瘤综合征的恶性副神经节瘤在文献中鲜有报道。基于文献背景，我们对该疾病的病理学、发病机制、诊断及治疗进行了讨论。
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