Ruiz-Ortiz M, Azcarate-Diaz F J, Galindo-Rodriguez D, Torres-Calcines N, Calleja-Castano P
Hospital Universitario 12 de Octubre, Madrid, Espana.
Rev Neurol. 2019 Sep 16;69(6):249-254. doi: 10.33588/rn.6906.2019191.
The cavernous sinus is a structure in the base of the skull that houses several nerve and vascular structures. Its compromise leads to cavernous sinus syndrome, which is a combination of oculomotor disorders and others affecting the first two trigeminal branches, often accompanied by pain or proptosis. Infiltration due to Burkitt's lymphoma is a rare cause of this syndrome.
A 43-year-old male, carrier of human immunodeficiency virus, with good control of the disease, who developed a clinical picture consisting of progressive painful ophthalmoplegia in the presence of a laterocervical adenopathy. Complementary tests allowed a diagnosis of Burkitt's lymphoma with extranodal extension to the cavernous sinus. A review of the cases published in Medline was also carried out: a total of 15 cases were detected and their epidemiological characteristics, form of presentation, extracranial involvement at the time of diagnosis and clinical progression were described.
Burkitt's lymphoma is a high-grade lymphoproliferative syndrome. Its form associated with immunodeficiency is an important cause of morbidity and mortality in this subgroup of patients. In the cases analysed in the literature, the age of presentation varied and the form of onset was a progressive painful ophthalmoplegia or numb chin syndrome. Exclusive involvement of the cavernous sinus was infrequent, but in that case it entailed a poor prognosis. It is important to rule out a primary extracranial origin and not to confuse it with an idiopathic Tolosa-Hunt syndrome that would delay the beginning of antitumour treatment.
海绵窦是颅底的一个结构,容纳多个神经和血管结构。其受损会导致海绵窦综合征,这是一种动眼神经障碍与影响三叉神经前两支的其他症状的组合,常伴有疼痛或眼球突出。伯基特淋巴瘤浸润是该综合征的罕见病因。
一名43岁男性,人类免疫缺陷病毒携带者,病情控制良好,出现了伴有颈侧淋巴结病的进行性疼痛性眼肌麻痹的临床表现。辅助检查确诊为伯基特淋巴瘤并伴有海绵窦的结外扩展。还对Medline上发表的病例进行了回顾:共检测到15例,并描述了其流行病学特征、表现形式、诊断时的颅外受累情况及临床进展。
伯基特淋巴瘤是一种高度恶性的淋巴增殖综合征。其与免疫缺陷相关的形式是该亚组患者发病和死亡的重要原因。在文献分析的病例中,发病年龄各异,发病形式为进行性疼痛性眼肌麻痹或颏部麻木综合征。单纯海绵窦受累并不常见,但这种情况下预后较差。重要的是要排除原发性颅外起源,且不要将其与特发性托洛萨 - 亨特综合征混淆,以免延误抗肿瘤治疗的开始。