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Neurological Surgery No Shinkei Geka Volume 22, Issue 5 （May 1994）

Neurological Surgery 脳神経外科 22巻5号（1994年5月発行）

Japanese English

症例

小脳天幕に腫瘤を形成したDural Type Histiocytosis Xの1例 A Case of Dural Type of Histiocytosis X Presenting as a Mass Lesion in the Tentorium Cerebelli 馬場雄大 ¹ , 伊林至洋 ¹ , 森本繁文 ¹ , 丹羽潤 ¹ , 田辺純嘉 ¹ , 端和夫 ¹ Takeo BABA ¹ , Yukihiro IBAYASHI ¹ , Shigefumi MORIMOTO ¹ , Jun NIWA ¹ , Sumiyoshi TANABE ¹ , Kazuo HASHI ¹ ¹札幌医科大学脳神経外科 ¹Department of Neurosurgery, Sapporo Medical University School of Medicine キーワード： Dural type extension , Eosinophilic granuloma , Histiocytosis X , MRI , Tentorial tumor Keyword: Dural type extension , Eosinophilic granuloma , Histiocytosis X , MRI , Tentorial tumor pp.471-476

発行日 1994年5月10日 Published Date 1994/5/10

DOI https://doi.org/10.11477/mf.1436900840

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Abstract 文献概要
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I．はじめに

　Histiocytosis Xは細網組織性肉芽腫の増殖を病変とする比較的稀な疾患であり，病因，本態は未だ明らかではない^21）．histiocytosis Xの全身病態の一部として中枢神経系へ浸潤することは知られているが^11），その大半は間脳下垂体系に集中している．

　今回，われわれは左頬骨部から頭蓋内に浸潤し，硬膜を介して海綿静脈洞から天幕切痕部へと進展し小脳天幕硬膜に腫瘤を形成したhistiocytosis Xの1症例を経験したので，文献的考察を加え報告する．

Histiocytosis X is a disease of unknown etiology, characterized by a mass of proliferating histiocytes, plasma cells and inflammatory cells foaming a granulo-ma within the reticuloendothelial elements of any organ in the body. In the central nervous system (CNS), hypothalamic disorder of histiocytosis X is often found, but histiocytosis X in other regions is quite rare. We re-port a case of a 5-year-old girl with histiocytosis X of the zygoma presenting as a mass lesion in the tentor-ium cerebelli. A computed tomographic (CT) scan de-monstrated a tumor at the left tentorial region, extend-ing along the dura mater of the tentorium cerebelli.Magnetic resonance imaging (MRI) revealed a low sig-nal intensity region on both T1 and T2-weighted im-ages. MRI with Gd-DTPA showed a homogeneous en-hanced mass extending to right and inferior sites with a thickened tentorium. As the thickened dura matter con-tinued from the left middle fossa to the mass lesion, the tumor was considered to arise from the left zygoma and extend to the tentorium cerebelli.

CNS extension of histiocytosis X is manifested either as (1) the cerebral type or (2) the dural type. Many cases of cerebral type histiocytosis X including hypothalamic disorder have been reported. Only 6 cases of the dural type of histiocytosis X have been de-scribed. Although the lesions of the cerebral type of histiocytosis X show prolonged T1 and T2 values on MRI, the MRI findings of the dural type have not been reported. The present case is the first report of the appearance of the lesion on MRI. The sagittal and coronal sections of MRI with Gd-DTPA provided addi-tional topographic dimensions, which were valuable for the assessment of the extension of the lesion, and for planning the appropriate operation.