颅骨黄色瘤病1例

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患者男,18岁,以多饮、多尿、消瘦半年于1995年3月28日入院。既往无头部外伤史。近半年出现烦渴,24h饮水量约4000~6000ml,同时出现多尿,约20次/日;伴烦躁不安,肌肉、关节酸痛及失眠,并逐渐出现消瘦。检查:发育营养中等,消瘦,皮肤干燥,弹性尚可,突眼征(一),右顶后部肿胀约3cm×2cm,触之软,扪及搏动感,压痛(+),神经系统无阳性体征。24h总尿量约3500~5400m1,尿比重1.002;血红蛋白130g/L,血钠153 mmol/L,T_3 0.53 nmol/L,T_4 122 nmol/L,TSH 7.0mU/L。ECG为窦性心律,61次/min。头颅MRI检查见右顶骨处有结节状肿块,约2.2cm× 2.4cm大小,颅骨内板受侵,涉及脑膜。Gd—DTPA增强扫描见肿物强化,脑膜未见强化。脑垂体形态、信号及大小均正常,双大脑半球、脑干、小脑亦未见异常。术中发现右顶骨后部全层缺损,大小约2.5cm× 3cm,有一隆起肉芽肿块,侵入骨膜及硬脑膜外层。切除后检查肿块中心呈脂性坏死,病理检查见泡沫细胞及胆固醇结晶,诊断为颅骨黄色瘤病。因颅骨缺损不大,未予修补。术后24h总尿量渐趋减少,烦渴减轻;第6日创口拆线,24h总尿量为2500ml,转上级医院进一步放疗。 The patient, male, 18 years old, admitted to hospital on March 28, 1995 for more than half a year to drink, polyuria, and weight loss. No previous history of head injury. In the past six months, there was polydipsia, and the amount of drinking water was about 4000 to 6000 ml in 24 hours. At the same time, there was polyuria in about 20 times per day; with irritability, muscle and joint aches and insomnia, and gradual weight loss. Check: moderate developmental nutrition, weight loss, dry skin, fair elasticity, exophthalmos sign (1), swelling of the right top posterior part of about 3cm × 2cm, soft touch, palpitation and pulsation, tenderness (+), no positive neurological system Signs. The 24h total urine volume was about 3500-5400m1, urine specific gravity 1.002, hemoglobin 130g/L, blood sodium 153 mmol/L, T_3 0.53 nmol/L, T_4 122 nmol/L, and TSH 7.0 mU/L. ECG is sinus rhythm, 61 beats/min. The cranial MRI showed a nodular mass at the right parietal bone, approximately 2.2 cm × 2.4 cm in size, and the inner skull plate was invaded, involving the meninges. Gd-DTPA enhanced scan showed tumor enhancement and no enhancement of the meninges. Pituitary morphology, signal and size were normal, but no abnormalities were found in the two hemispheres, brainstem and cerebellum. The full-thickness defect of the posterior parietal bone was found during the operation. The size of the posterior parietal bone was about 2.5cm×3cm. There was a raised granuloma mass that invaded the periosteum and the outer layer of the dura mater. After excision, the center of the mass was examined for fatty necrosis. The pathological examination showed foam cells and cholesterol crystals. The diagnosis was skull angioma. Due to the small skull defect, no repairs were made. After 24 hours, the total urine volume gradually decreased, and the thirst decreased. On the 6th day, the wounds were sutured and the total urine output was 2500 ml in 24 hours. The patients were transferred to a higher hospital for further radiotherapy.
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