Role of miR-101 in pheochromocytoma patients with SDHD mutation

This study aimed to screen the potential diagnostic biomarkers for distinguishing the malignant pheochromocytoma (PCC) from benign PCC. A total of 59 patients with PCC (benign and malignant) were enrolled in this study. The expression level of miRNAs in patients with different kind PCCs (healthy control, benign, malignant, malignant with or without SDHD mutation, adrenal and extra-adrenal) was analyzed using the qRT-PCR analysis. Besides, the diagnosis accuracy of miRNA in PCC samples was analyzed using the ROC analysis. Moreover, level of miR-101 in serum was detected by qRT-PCR analysis and serum VEGF level in patients with PCC was detected using the ELISA kit. Compared with benign PCC, miR-101 level was higher in patients with malignant PCC (P < 0.05), while the level of miR-513-5p and miR-26b showed no difference between malignant PCC and benign PCC (P > 0.05). miR-101 expression was significantly increased in malignant tumor tissue with SDHD mutation (P < 0.05) and in extra-adrenal tissues (P < 0.05), respectively. Besides, AUCs for miR-101 in PCC samples was 0.79 and for which in PCC samples with non-SDHD mutation was 0.77. Besides, serum miR-101 in malignant PCC was high but showed no difference among groups (P > 0.05). Moreover, serum VEGF level in malignant tumors was significantly high compared with benign tumor, as well as that in malignant PCC with SDHD mutation (P < 0.05). Our study suggested that SDHD mutation may enhance the overexpression of miR-101 in malignant tumors and miR-101 may be a potential diagnostic biomarker for malignant PCC and benign PCC.     

肾上腺嗜铬细胞瘤致异位ACTH综合征的临诊应对

肾上腺嗜铬细胞瘤引起的异位ACTH综合征非常罕见.本文详细介绍1例肾上腺嗜铬细胞瘤引起异位ACTH综合征临床特点并结合国内外研究进展,讨论其诊治.提出正确的诊断应结合临床、生化、激素分泌、影像学、病理学、肿瘤免疫组化染色进行最终的综合判断.     

腹腔镜下切除无症状、生化正常的肾上腺嗜铬细胞瘤12例报告

目的:探讨无症状、生化检查正常的肾上腺嗜铬细胞瘤的诊治原则。方法:回顾分析12例无症状、生化检查正常的肾上腺嗜铬细胞瘤患者的临床资料,术前口服酚苄明1～2周,充分扩容,行腹腔镜肾上腺肿瘤切除术,其中经腰2例,经腹10例。结果:12例患者术中挤压肿瘤时血压均波动明显,手术一期完成,未发生大出血、心脑血管意外等严重并发症,无一例中转开放手术。术后病理均诊断为肾上腺嗜铬细胞瘤。随访6～36个月,血压正常,无复发。结论:无症状、生化检查正常的肾上腺肿瘤临床多见,术前按嗜铬细胞瘤进行准备,充分扩容后行腹腔镜手术是安全、有效的。     

膀胱非上皮性肿瘤的MDCT表现

目的 探讨膀胱非上皮性肿瘤的MDCT表现特征.方法 搜集经病理证实的19例膀胱非上皮性肿瘤患者的临床、膀胱镜和MDCT平扫及四期增强检查资料,结合相关文献分析MDCT表现特征.结果 19例膀胱非上皮性肿瘤中,良性肿瘤11例,其中平滑肌瘤6例,嗜铬细胞瘤4例,炎性肌纤维母细胞瘤1例;恶性肿瘤8例,其中原发性淋巴瘤3例,胚胎性横纹肌肉瘤2例,纤维肉瘤、低度恶性间质瘤及神经内分泌癌各1例.MDCT对膀胱非上皮性肿瘤的良恶性诊断准确率为89.47％ (17/19),病理类型诊断准确率为63.16％ (12/19).5例膀胱镜提示膀胱腔内肿瘤,诊断为膀胱癌,14例膀胱镜提示膀胱黏膜光滑,诊断为黏膜下病变.结论 膀胱非上皮性肿瘤各自有一定的MDCT表现特征,仔细分析大部分可正确诊断,有效地指导临床选择合适的治疗方案.     

Prevalence and progression of carbohydrate disorders in patients with pheochromocytoma/paraganglioma: retrospective single-center study

BackgroundCarbohydrate disorders are the most frequent metabolic disorders, affecting a significant proportion of patients with pheochromocytoma.ObjectiveA retrospective study assessed the prevalence and progression of carbohydrate disorders in 204 patients (92 men, 112 women) with histologically proven pheochromocytoma diagnosed in a single specialized tertiary center during a 40-year period (1978–2017). One hundred were followed-up after tumor removal.ResultsCarbohydrate disorders were diagnosed in 49.5% of cases: 30.4% with diabetes and, 19.1% prediabetes. Subjects with carbohydrate disorders had significantly greater age at diagnosis and higher 24-hour urine metanephrine and normetanephrine concentrations than those with normal glucose tolerance. One-third of patients with diabetes achieved good glycemic control under oral treatment (54% on metformin monotherapy). One-third of patients overall required preoperative insulin treatment. Postoperative follow-up (100 patients; 5-year mean duration) showed reduced prevalence of diabetes (13% vs. 33%; P = 0.0007) and prediabetes (12% vs. 24%; P = 0.027). Almost 60% of subjects initially diagnosed with carbohydrate disorders recovered normal glucose tolerance after surgery; these subjects had significantly higher preoperative urine metanephrine/normetanephrine levels than those with persistent diabetes/prediabetes. Correlation analysis revealed a moderate negative relationship between urine metanephrine/normetanephrine concentration and the outcome of the carbohydrate disorders (Spearmen's Rho = − 0.507; P = 0.013). There was no significant difference according to pre- or postoperative prevalence of obesity (15% vs. 16%; P = 0.845) or dyslipidemia (46% vs. 39%; P = 0.316).ConclusionsCarbohydrate disorders affect approximately 50% of pheochromocytoma patients; 30% develop overt diabetes, which may be the only clinical manifestation in some rare cases. Pheochromocytoma-related diabetes is more likely to affect patients with predominant adrenaline secretion. It is often easy to control and usually requires oral antidiabetic treatment. Reversibility of carbohydrate disorders depend on severity, preoperative metanephrine level, age and weight.     

甲襞微循环监测在嗜铬细胞瘤术前准备中的应用

目的:探讨甲襞微循环监测在嗜铬细胞瘤术前药物准备中的临床意义。方法:采取前瞻性自身对照研究,对20例嗜铬细胞瘤患者术前用竹林胺药物准备前、后,以及术后2~3个月,通过WXT-4型微循环图象计算机分析系统监测甲襞微循环,在微循环形态、血管流态、血管周围的情况三方面共22项指标进行定量分析与t检验。结果:20例患者经药物准备前后,在血管襻清晰度、管襻数、输入支管径、输出支管径、管襻长度、红细胞聚集程度、血色、形态积分、流态积分、总积分这10项微循环指标的统计学检验显示差异均有统计学意义(P<0.05或P>0.01);而手术前后微循环各项指标间差异无统计学意义。结论:甲襞微循环可以较好地反映全身微循环的状况,甲襞微循环监测可作为嗜铬细胞瘤术前准备的一项重要指标应用于临床。     

腹腔镜腹腔入路嗜铬细胞瘤切除

目的 回顾分析腹腔镜腹腔入路肾上腺嗜铬细胞瘤切除的经验,探讨腹腔镜腹腔入路切除嗜铬细胞瘤的效果.方法 22例患者中男9例,女13例,年龄42岁(18～67岁),双侧3例.所有患者均行腹腔入路肾上腺手术.对手术方法、临床效果进行分析.结果 手术时间60～240 min,平均90min,术中失血量20～150 ml,平均80ml,无需输血者.中转开放手术1例(4.5%),其余手术均成功,无血管及胃肠道并发症.患者术后第1d下床活动,术后平均住院时间7d(6～9 d).结论 腹腔镜肾上腺嗜铬细胞瘤切除术适用于直径＜6cm的肾上腺嗜铬细胞瘤,具有安全、微创、出血少、恢复快等优点.     

严重发作性高血压-伪嗜铬细胞瘤的治疗

目的报道严重发作性高血压-伪嗜铬细胞瘤的治疗。方法总结一组酷似嗜铬细胞瘤的严重发作性高血压：伪嗜铬细胞瘤,男女各两例,平均年龄42.50±8.06岁,归纳其临床特点。结果伪嗜铬细胞瘤具有嗜铬细胞瘤的类似症状：①突然血压升高、②伴随躯体不适症状。伪嗜铬细胞瘤区别于嗜铬细胞瘤及其他高血压之处：①无儿茶酚胺代谢物指标升高。②并非由情感因素直接引起,多数患者具有创伤史或消极的对应方式,③排除嗜铬细胞瘤及酷似嗜铬细胞瘤症状体征的疾患。④β、α1受体阻滞剂、心理治疗必要时联合抗抑郁药、抗焦虑药有效。结论伪嗜铬细胞瘤有别于嗜铬细胞瘤及一系列酷似嗜铬细胞瘤的临床情况,β、α1受体阻滞剂、心理治疗必要时联合抗抑郁药、抗焦虑药有效。     

嗜铬细胞瘤的CT诊断

目的:探讨嗜铬细胞瘤的CT表现,提高CT诊断的准确性。方法:搜集经手术和病理证实的17例嗜铬细胞瘤患者的资料,所有病例术前均作超声、CT检查。结果:瘤体呈较大(平均瘤径6.1cm)的类圆形肿块,瘤界清晰,平扫密度多不均匀,10例出现坏死和囊变,肿瘤实体部分多明显强化。结论:嗜铬细胞瘤CT表现具有一定特征,CT可清楚显示肿瘤的部位、形态、大小、瘤内成份及与周围结构的关系,结合临床表现多可明确诊断。     

嗜铬细胞瘤的诊治

一例34岁男性因波动性高血压疑为嗜铬细胞瘤而转至本科就诊。该患者临床表现典型,人院查血浆甲氧基肾上腺素(MN)及甲氧基去甲肾上腺素(NMN)均明显升高,CT和PET-CT检查均发现左侧肾上腺占位,诊断为嗜铬细胞瘤。术前予以甲磺酸多沙唑嗪控释片4 mg/d口服2周,血压平稳,在腹腔镜下行左侧肾上腺肿瘤切除术。术后病理提示嗜铬细胞瘤,MN、NMN、血压恢复正常,症状缓解。该患者术后随访3年,血压、血浆MN、NMN水平均正常,肾上腺CT未见肿瘤复发。