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[起源于蝶骨嵴内侧、额叶的原发性颅内卵黄囊瘤]

[Primary intracranial yolk sac tumor developing in the frontal lobe from the inside of the sphenoidal ridge].

作者信息

Sugawara T, Tsurumi Y, Kuwahara K, Katakura R, Suzuki J

出版信息

No Shinkei Geka. 1984 Mar;12(3 Suppl):401-6.

PMID:6462349
Abstract

A case of primary intracranial yolk sac tumor, the first known case growing in the frontal lobe, is compared with similar cases of suprasellar region. The case, 18-year-old female, suffered from headache, vomiting and visual disturbance for one month prior to the hospitalization. Plain CT scan demonstrated suppressed left anterior horn and normal density area in front of it. After injection of contrast medium, the area was enhanced distinctly. The left carotid angiography displayed a hypervascular mass in the suprasellar region and tumor stain was also seen in the capillary phase. Bilateral frontal craniectomy was performed and the tumor was almost totally removed macroscopically. The tumor situated in the left frontal lobe infiltrated into the optic nerve and a part of anterior cerebral artery. Histologically the tumor was diagnosed as yolk sac tumor according to Teilum's classification. There were stellate cells arranged in loose vacuolated network which formed cystic cavities and a complicated network of honeycomb with communicating cavities and extracellular PAS-positive hyaline globules. Glomerular-like structures (Schiller-Duval body) was also seen. Immunoperoxidase study clearly demonstrated the presence of intracytoplasmic alphafetoprotein granules in the tumor tissue. In radioimmunoassay, the level of the serum alphafetoprotein measured was two folds higher than that of the normal range, postoperatively. Although irradiation (local 3000 rads, whole 3000 rads) combined with chemotherapy (ACNU, Futraful), PSK had almost no effect. The effect of other chemotherapy (Cis-platin, VBL, Bleomycin) was indicated by the diminish size of the tumor. Five months after the onset, she was discharged with almost no neurological findings other than left visual loss. Pathological findings and clinical treatments were also discussed in detail.

摘要

本文报告一例原发性颅内卵黄囊瘤,这是首例已知生长于额叶的病例,并与鞍上区类似病例进行了比较。该病例为一名18岁女性,住院前一个月出现头痛、呕吐及视力障碍。平扫CT显示左侧前角受压,其前方密度正常区域。注入造影剂后,该区域明显强化。左侧颈动脉血管造影显示鞍上区有一高血运肿块,毛细血管期可见肿瘤染色。行双侧额部开颅手术,肿瘤大体上几乎完全切除。肿瘤位于左侧额叶,侵犯视神经及部分大脑前动脉。组织学上,根据Teilum分类,该肿瘤被诊断为卵黄囊瘤。可见星状细胞排列成疏松的空泡状网络,形成囊腔以及复杂的蜂窝状相互连通的腔隙,还有细胞外PAS阳性透明小球。还可见肾小球样结构(Schiller-Duval小体)。免疫过氧化物酶研究清楚地显示肿瘤组织中存在胞浆内甲胎蛋白颗粒。放射免疫测定显示,术后血清甲胎蛋白水平比正常范围高两倍。尽管放疗(局部3000拉德,全脑3000拉德)联合化疗(ACNU、氟尿嘧啶)及PSK几乎无效,但其他化疗(顺铂、长春碱、博来霉素)显示肿瘤体积缩小。发病五个月后,她出院时除左侧视力丧失外几乎没有神经功能异常。文中还详细讨论了病理结果及临床治疗情况。

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