CLINICAL DIAGNOSIS AND TREATMENT OF NONFUNCTIONING PHEOCHROMOCYTOMAS IN 14 PATIENTS

Objective To analyze the clinical characteristics of nonfunctioning pheochromocytoma, and to evaluate the efficacy of 131I-metaiodobenzylguanidine (MIBG) scan in the diagnosis and perioperative treatment of nonfunctioning pheochromocy- toma. Methods The clinical data of 14 patients with nonfunctioning pheochromocytoma were analyzed retrospectively. Plasma free corticoid, renin, aldosterone, and urine catecholamines levels were estimated. B-mode ultrasonography, computed tomo- graphy scan, thoracic X-ray and 131I-MIBG were used. Results All patients with nonfunctioning pheochromocytoma had no hypertension and the tumors were found inciden- tally. The 24 hours urine catecholamines levels in 80% (8/10) patients were normal. The positive rate of 131I-MIBG was 80% (8/10) and the specificity was 100%. All patients underwent surgical operation of tumor resection. No preoperative volume expansion was given to all patients. All tumors were resected completely, and no death accident happened. There was no recurrence and metastasis after operation by long-term follow-up. Conclusion 131I-MIBG scan is the first choice technique for the diagnosis of nonfunctioning pheochromocytoma. Blood volume expansion is unnecessary before resection of pheochromocytoma.no recurrence and metastasis})

Twenty-six patients with nonfunctional pheochromocytomas

Nonfunctional pheochromocytoma is a particular type of pheochromocytoma without typical clinical features of catecholaminism. It is difficult to diagnose or differentially diagnose. This type of pheochromocytoma is rarely reported in the literature. From January 1, 1990 to June 1, 2004, we performed operations for 197 patients with pheochromocytomas, including 26 patients with nonfunctional pheochromocytoma. We analyzed the clinical data of the 26 patients.

超声在肾上腺占位性病变中的诊断价值

目的:探讨超声在肾上腺占位性病变中的诊断价值及其超声影像学特点。方法:回顾分析12例肾上腺占位的二维及彩色多普勒超声的声像图特点。结果:12例病例中有6例为嗜铬细胞瘤,2例为肾上腺囊肿,2例为肾上腺转移癌,1例为无功能皮质腺瘤,1例为髓样脂肪瘤。结论:结合病史资料,根据不同的超声声像图表现,可提高对于肾上腺占位性病变的诊断。

伴儿茶酚胺心肌病的嗜铬细胞瘤／副神经节瘤的围手术期处理经验

目的 总结伴儿茶酚胺心肌病的嗜铬细胞瘤/副神经节瘤（PHEO/PGL）患者的心功能变化、诊断及围手术期治疗经验。方法 回顾性分析2008年1月至2018年3月收治的15例PHEO/PGL伴儿茶酚胺心肌病患者的临床资料，男8例，女7例。年龄13-64岁，平均32.8岁。左侧PHEO 4例，右侧PHEO 4例，双侧PHEO 3例（其中VHL病2例），颈静脉体PGL 1例，左腹膜后PGL 2例，右腹膜后PGL 1例。肿瘤直径2.3-7.2 cm。14例术前确诊为PHEO/PGL伴儿茶酚胺心肌病，有头痛、心悸、大汗等典型PHEO/PGL临床表现；24 h尿儿茶酚胺检查示肾上腺素平均24.87 μg/24 h，去甲肾上腺素平均551.70μg/24 h，多巴胺平均395.41μg/24 h；7例奥曲肽检查及131I-间碘苄胍显像均阳性，6例奥曲肽检查阳性，1例肾上腺髓质全身131I-间碘苄胍显像阳性。增强CT检查示腹膜后肾上腺区或肾上腺外明显不均匀强化肿物，内部可见缺血坏死区，部分瘤体血运极丰富，可见肿瘤周边呈蔓状分布的不规则血管网。超声心动图检查示儿茶酚胺心肌病发作时左室射血分数（LVEF）为（42.0±7.1）%（32%-54%），其中轻度异常（LVEF 45%-54%）5例，中度异常（LVEF 30%-44%）9例，伴左心室肥厚、心肌回声增强、左心扩大、心肌运动减弱等儿茶酚胺心肌病表现。9例严重心功能不全患者伴急性左心衰，表现为肺水肿、咳大量粉红色泡沫痰。此14例予规律α受体阻滞剂、β受体阻滞剂及钙离子拮抗剂治疗1-3个月，怀疑肺部感染者加用敏感抗生素，并予强心、利尿等治疗，待心功能显著改善后手术。1例为隐匿型PHEO，缺乏典型临床表现及影像学改变，24 h尿儿茶酚胺检查及奥曲肽显像均无异常；术前超声心动图检查示心功能正常，LVEF为73%；术前诊断为右肾上腺无功能腺瘤，未行药物准备。本组15例，行腹腔镜手术13例，其中1例中转开放手术；开放手术2例（均为PGL患者）。结果 本组15例手术均完整切除肿瘤，1例隐匿型PHEO术中触碰瘤体时出现血压剧烈波动，血压190/130-80/50 mmHg（1 mmHg＝0.133 kPa），心率150次/分，术后于ICU病房出现儿茶酚胺心肌病表现，超声心动图检查示LVEF为37%，左心扩大且心肌普遍收缩不良；纤维支气管镜检查示双肺粉红色泡沫痰；胸部X线片检查示双侧肺水肿、左侧为著。确诊为儿茶酚胺心肌病，经治疗后心功能恢复。14例术前确诊病例，药物治疗后LVEF为（55.9±7.6）%，与治疗前比较差异有统计学意义（P＝0.041）；术后LVEF为（66.1±8.5）%，与药物治疗后比较差异有统计学意义（P＝0.013）。本组15例术后随访1个月-10年，均无复发。结论 PHEO/PGL伴儿茶酚胺心肌病患者如诊断明确，需行充分药物准备，发生心功能异常需积极对症治疗，心功能异常可在肿瘤切除后有效改善。隐匿型PHEO围手术期风险大，对于疑似隐匿型PHEO需积极药物准备后再行手术治疗。

13例无功能型肾上腺嗜铬细胞瘤临床分析

目的探讨无功能型肾上腺嗜铬细胞瘤的诊断和治疗方法。方法回顾性分析13例无功能型肾上腺嗜铬细胞瘤的临床资料。患者均为偶然发现，无高血压和典型的嗜铬细胞瘤临床表现，4例为体检时发现，8例出现腰区、上腹部不适或疼痛，2例出现发热。血浆肾上腺素升高1例（7．7％），去甲肾上腺素升高2例（15．4％）。24h尿儿茶酚胺（CA）及尿香草基扁桃酸（VMA）测定均正常。12例术前给予哌唑嗪降压或全血扩容治疗，经腰第十一肋切口肿瘤切除术12例，腹腔镜肾上腺及肿瘤切除术1例，术中血压平稳无波动。结果术后病理检查均证实为嗜铬细胞瘤。随访11个月至6．5年，患者血压正常，无肿瘤复发及转移。结论无功能型肾上腺嗜铬细胞瘤患者无高血压等典型症状，多为体检时发现或表现为上腹部或腰区疼痛，由于其分泌CA及其代谢产物水平较低，为定性诊断带来困难。为防止隐匿功能型嗜铬细胞瘤所致围手术期严重并发症，对于具有典型影像学表现的无功能型肾上腺嗜铬细胞瘤患者术前也应进行常规的降压及扩容治疗，降低手术危险和并发症发生率。