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家族性嗜铬细胞瘤比较罕见,国内报导较少,合并小脑星状细胞瘤未见报导,我们遇到一例报告如下。 例1,女性32岁,住院号9999,因多饮多食多尿6年,1个月来上述症状加重伴体重下降入院。高血压病史16年,平素血压22.0～24.0/13.0～160.KPa。查体:BP22.0/13.0KPa,表情焦虑,目光有神,肢端皮肤潮红,甲状腺不肿大,心肺未见异常,行走呈醉汉步态。血电解质肝肾功能,甲状腺功能,血皮质醇,血醛固酮,尿17-OH,尿17-酮均正常。血RBC6.15×10~(12)/LHb181g/L,空腹血糖15.0mmol/L,尿糖+++,尿VMA152.2μmnol/24h,尿儿茶酚胺665μmol/24h。眼底动静脉交叉压迫征阳性。B超右肾上腺可见9.2×4.9×4.4cm不规则强回声团,有包膜,内有不规则低回声区;MRI右肾上腺区可见8.8×4.6×4.0cm中等长T_1中等长T_2信号;左小脑半球可见3.0×5.0×6.0cm中等长T_1,中等长T_2信号,边界清楚,第四脑室及脑桥区受压,小脑扁桃体下缘变尖,向下突出枕大孔9.0mm。1995年4月行小脑肿瘤切除术,病理诊断:小脑星状细胞瘤Ⅱ级。病理号:98163。
Familial pheochromocytoma is relatively rare, less reported in the country, combined cerebellar stellate cell tumor has not been reported, we encountered a case report as follows. Example 1, female 32 years old, hospital number 9999, due to drink plenty of polyuria 6 years, 1 month to aggravate the above symptoms accompanied by weight loss to hospital. Hypertensive history of 16 years, usually blood pressure 22.0 ~ 24.0 / 13.0 ~ 160. KPa. Physical examination: BP22.0 / 13.0KPa, anxiety, eyes look, acne flushing of the extremities, thyroid enlargement, heart and lung no abnormalities, walking was drunk gait. Blood electrolytes liver and kidney function, thyroid function, blood cortisol, blood aldosterone, urine 17-OH, urine 17-ketone were normal. Blood RBC6.15 × 10-12 / LHb181g / L, fasting blood glucose 15.0mmol / L, urine sugar + + +, urine VMA152.2μmnol / 24h, urinary catecholamines 665μmol / 24h. Cross-ophthalmic ophthalmic positive sign. B super-right adrenal can be seen 9.2 × 4.9 × 4.4cm irregular strong echo group, there are envelopes, there are irregular hypoechoic area; MRI right adrenal area can be seen 8.8 × 4.6 × 4.0cm medium T_1 medium T_2 signal; left cerebellum Hemisphere visible 3.0 × 5.0 × 6.0cm medium T_1, medium long T_2 signal, the boundary is clear, the fourth ventricle and pons area compression, cerebellar tonsils lower edge sharp, downward prominent occipital 9.0mm. 1995 April cerebellar tumor resection, pathological diagnosis: cerebellar astrocytoma grade Ⅱ. Pathology: 98163.