患者,男,61岁.因左侧腹股沟坠痛1周,查体发现马蹄肾合并左肾占位5d于2012年9月24日入院.查体及实验室检查未见异常.B超检查:双肾下极向中线靠拢,于主动脉前融合,左肾中上部可见5.0 cm×3.6 cm中等回声结构,提示为马蹄肾合并左肾占位性病变.CT检查:双肾位置较低,下极可见融合,左肾中上部可见一不规则混杂密度灶,边缘欠清,向内推挤肾实质,最大断面5.0 cm×3.8 cm,平扫CT值30～ 34 HU,强化扫描后肿物呈不均匀强化,皮质期CT值60～ 100 HU,分泌期CT值72～ 80 HU;下腔静脉及腹主动脉周围未见淋巴结肿大.CT血管造影检查:肾段腹主动脉从L1上缘平面起向前、向右扭曲下行,到L2下缘平面再向后、向左扭曲下行,发出两支动脉支配左肾,肾肿物血供主要来自优势动脉,血供丰富.临床诊断为马蹄肾合并肾癌。
目的 总结肾上腺皮质腺癌(ACC)的诊治经验,提高肾上腺皮质癌的诊治水平.方法 对5例ACC患者的临床表现、实验室及影像学检查、治疗和预后进行回顾性分析.结果 本组B超示肾上腺实质性中等回声,内部回声不均匀,可见点状血流反射;CT均示肿瘤边缘模糊,CT值32 ～ 65 HU,强化不均匀;MRI示肿瘤边界欠清,T2加权片上2例呈高信号,1例呈高低混杂信号,病灶中心坏死区无明显强化.5例均行开放性手术完全切除肿物,术后应用米托坦2例、放疗1例;术后随访6 ～ 36个月,平均生存23个月.结论 B超、CT等检查有利于ACC的早期诊断,手术切除是治疗ACC的有效方法.
Objective To investigate the genetic and clinical characteristics of non-syndromic familial pheochromocytoma. Methods We retrospectively analyzed the clinical data of a case of non-syndromic familial pheochromocytoma in an adolescent combined with relevant literature review. Then we investigated the incidence of his kindred and drew a dendrogram. Results There were three members with pheochromocytoma in the big family of three generations. All tumors occurred in adolescence which were planted on the adrenal body only. All three patients received operation of adrenalectomy.And one of them was recurred on the opposite side after unilateral operation. The pathologic results showed benign adrenal pheochromocytoma. The follow-up lasted for one year to twenty-nine years without recurrence. Conclusions The characteristic of non-syndromic familial pheochromocytoma is accordance with that of autosomal dominant inheritance disease. The tumor occurs mostly in adolescence. Mutation detection for candidate genes in adolescent patients with clinically suspected pheochromocytoma is conducive to the early diagnosis of this disease.
