Sakakibara Y, Matsuzawa M, Taguchi Y, Sekino H, Shinagawa T, Abe M, Tadokoro M
Division of Neurosurgery, St. Marianna University Yokohama City Seibu Hospital, Japan.
No Shinkei Geka. 1998 Jan;26(1):53-8.
A case of primary T cell type malignant lymphoma of the sellar region was reported. This 53-year-old male was admitted to our neurosurgical service because of slowly progressive occipitalgia and diplopia over the previous 5 months. On admission neurological examination revealed a slight limitation of the lateral movement of the left eye. Endocrinological examination showed no abnormalities. Despite a mild neurological deficit, a CT scan revealed an extensive bony destruction around the sellar region including the dorsum sellae, the bilateral petrous apices, and the upper two thirds of the clivus. Magnetic resonance imaging (MRI) disclosed that the tumor was slightly hypointense on T1-weighted image, isointense on T2-weighted image, and faintly and homogeneously enhanced after administration of Gd-DTPA. Since the tumor was enhanced faintly on MRI, it was diagnosed as an invasive pituitary adenoma, but it was thought that chordoma and germinoma should be considered. The patient underwent a transsphenoidal surgery. A firm, grayish and avascular tumor was partially removed. The histopathological examination using monoclonal surface marker of the specimen revealed the tumor was stained with UCHL-1 and CD3 antibodies directed against T cells, and diagnosed as T cell type malignant lymphoma of the sellar region (diffuse, large cell type; LSG). Postoperatively he received radiation therapy. His symptoms much improved and no regrowth of the residual tumor has been found in follow-up studies to the present. Primary malignant lymphomas very rarely occur in the skull base. As far as we are aware, this is the only second case of primary T-cell lymphoma in the sellar region reported so far in the medical literature. Although preoperative diagnosis is extremely difficult because of a paucity of data, malignant lymphomas should be kept in mind in the differential diagnosis of sellar tumors, especially if neuroimaging studies show an extensive bony destruction in the skull base.
报告了1例鞍区原发性T细胞型恶性淋巴瘤。该53岁男性因在过去5个月中缓慢进展的枕部疼痛和复视而入住我院神经外科。入院时神经检查发现左眼外展轻度受限。内分泌检查未发现异常。尽管存在轻度神经功能缺损，但CT扫描显示鞍区周围包括鞍背、双侧岩尖和斜坡上三分之二有广泛的骨质破坏。磁共振成像（MRI）显示肿瘤在T1加权图像上略呈低信号，在T2加权图像上呈等信号，静脉注射钆喷酸葡胺（Gd-DTPA）后呈轻微均匀强化。由于肿瘤在MRI上强化轻微，最初诊断为侵袭性垂体腺瘤，但认为应考虑脊索瘤和生殖细胞瘤。患者接受了经蝶窦手术。部分切除了一个质地硬、灰白色且无血管的肿瘤。对标本使用单克隆表面标志物进行组织病理学检查，发现肿瘤被针对T细胞的UCHL-1和CD3抗体染色，诊断为鞍区T细胞型恶性淋巴瘤（弥漫性大细胞型；LSG）。术后他接受了放射治疗。他的症状明显改善，随访至今未发现残留肿瘤复发。原发性恶性淋巴瘤很少发生在颅底。据我们所知，这是医学文献中迄今为止报道的第二例鞍区原发性T细胞淋巴瘤。尽管由于数据有限术前诊断极其困难，但在鞍区肿瘤的鉴别诊断中应考虑恶性淋巴瘤，尤其是当神经影像学检查显示颅底有广泛骨质破坏时。
