北京大学学报(医学版) ›› 2019, Vol. 51 ›› Issue (2): 298-301. doi: 10.19723/j.issn.1671-167X.2019.02.019

• 论著 • 上一篇    下一篇

肾上腺皮质癌的综合治疗

邓建华,李汉忠(),纪志刚,张玉石,刘广华   

  1. 中国医学科学院北京协和医学院,北京协和医院泌尿外科, 北京 100730
  • 收稿日期:2017-03-14 出版日期:2019-04-18 发布日期:2019-04-26
  • 通讯作者: 李汉忠 E-mail:lihzhpumch@163.com

Comprehensive treatment of adrenal cortical carcinoma

Jian-hua DENG,Han-zhong LI(),Zhi-gang JI,Yu-shi ZHANG,Guang-hua LIU   

  1. Department of Urology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China
  • Received:2017-03-14 Online:2019-04-18 Published:2019-04-26
  • Contact: Han-zhong LI E-mail:lihzhpumch@163.com

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摘要:

目的: 探讨肾上腺皮质癌临床及功能影像学和病理学特点,提高肾上腺皮质癌诊治水平。方法: 分析肾上腺皮质癌患者的临床资料,根据其临床表现、肾上腺内分泌功能测定、影像学特点和病理结果做出诊断,进行手术治疗和米托坦药物治疗并随访。结果: 93例患者年龄11~76岁,中位年龄48岁。男女比例1 ∶1.2。24 h尿游离皮质醇(urinary free cortisol,UFC)高者86例,促肾上腺皮质激素释放激素(adrenocorticotropic hormone,ACTH)下降88例,血浆皮质醇节律消失82例,醛固酮升高31例,性激素升高36例,术前神经烯醇化酶(neuron specific enolase,NSE)升高27例,胰岛素样生长因子-1(insulin-like growth factor-1,IGF-1)升高26例,76例大剂量和小剂量地塞米松抑制试验均不被抑制。高血压62例,典型Cushing综合征表现者81例。血糖升高54例,低血钾症21例,雄激素分泌者36例。肿瘤最大直径3~17 cm,伴肾上腺中央静脉、肾静脉及下腔静脉瘤栓者6例,手术至术后复发时间在1.2~5.0年。初发及随访中出现转移复发的56例,其中肺转移13例,肝转移17例,腹膜后淋巴结转移9例,腰椎转移7例,卵巢转移3例,腹壁及切口种植3例,其他部位转移4例,初发即远处转移者15例。行根治性切除术 77例,侵及同侧肾者做肾和肾上腺切除术11例,肾上腺肿瘤并腔静脉癌栓切除5例,腔静脉部分切除 3例。临床分期为Ⅰ期 39例,Ⅱ期 28例,Ⅲ期 16例,Ⅳ期 10例。随访8~69个月,手术5年以上的患者中有 43例仍存活。结论: 肾上腺皮质癌早期诊断非常关键,功能影像学检查结合临床特点及内分泌激素水平可确诊,根治性手术是唯一有效的治疗方法,米托坦可作为辅助治疗用于复发转移或无法手术治疗的患者,肿瘤恶性程度高,预后差。

关键词: 肾上腺皮质癌, 核素成像, 病理, 米托坦, 预后

Abstract:

Objective: To investigate the clinical and functional imaging examination and pathological features of adrenocortical carcinoma (ACC), in order to improve the diagnosis and treatment of ACC.Methods: The clinical data of 93 patients with ACC were analyzed retrospectively. Their diagnosis, surgical treatment and follow-up of mitotane medcine therapy were madeaccording to clinical manifestations, adrenal endocrine function determination, imaging examination characteristics and histopathological results.Results: Among the 93 patients, the age ranged from 11 to 76 years, with a median age of 48 years. The ratio of male to female was 1 ∶1.2. Twenty-four hours urinary free cortisol (UFC) elevated in 86 cases, adrenocorticotropic hormone (ACTH) decreased in 88 cases, blood F rhythm disappeared in 82 cases, and 31 cases of aldosterone increased. Thirty-six cases of sexual hormone increased. Neuron specific enolase (NSE) increased in 27 cases. Insulin-like growth factor-1 (IGF-1) increased in 26 cases. Seventy-six cases of high-dose dexamethasone suppression test (HDDST) and low-dose dexamethasone supression test (LDDST) were not suppressed separately. There were 62 cases of hypertension, and typical Cushing manifestations in 81 cases. Blood glucose elevated in 54 cases. Hypokalemia was in 21 cases and androgen secretion increased in 36 cases. The maximum diameter of the tumor was 3-17 cm, with 6 cases of adrenal central vein, renal vein and inferior vena cava tumor thrombus. The recurrence time was 1.2-5.0 years after operation. Metastasis and recurrence were in 56 cases during the follow-up, lung metastasis in 13 cases, liver metastasis in 17 cases, retroperitoneal lymph node metastasis in 9 cases, lumbar metastasis in 7 cases, ovarian metastasis in 3 cases, abdominal wall and incision implantation in 4 cases. Fifteen cases with distant metastasis. Seventy-seven patients were treated with radical adrenalectomy, and the other patients were treated with renal and adrenal resection on the same side of the kidney. There were 5 cases of adrenal tumor with vena cava tumor thrombus in the removal of the tumor, and the other for the partial resection of the vena cava in 3 cases. Regarding the clinical stage, stage I was in 39 cases, stage Ⅱ in 28 cases, stage Ⅲ in 16 cases and stage Ⅳ in 10 cases. The patients were followed up for 8-69 months, and 43 patients survived more than 5 years.Conclusion: Function imaging combined with clinical features and endocrine hormone levels have important roles in ACC early diagnosis. Radical excision is the only effective treatment. Adjuvant or adjuvant mitotane drug therapy can brused for the treatment of recurrence and metastasis ACC patients,. ACC is a tumor with high malignancy and poor prognosis.

Key words: Adrenocortical carcinoma, Nuclear imaging, Pathology, Mitotane, Prognosis

中图分类号: 

  • R737

图1

肾上腺皮质癌年龄分布特点"

表1

肾上腺皮质癌常见的临床症状和体征特点"

Signs and symptoms Percentage/%
Obesity 91.4
Hypertension 66.7
Diabetes 77
Central obesity 76
Tired 81.7
Muscle atrophy 71
Hypertrichiasis and hairy 72
Abnormal menstruation and hyposexuality 43/51
Purple grain 69.9
Full moon face 67
Osteoporosis 57
Bruise 48
Skin color deepened 46
Mental change 44
Edema 43
Headache 37
Poor wound healing 40

表2

肾上腺皮质癌临床和激素水平特征"

Parameters Results
Age/years 48.1±2.1
BMI /(kg/m2) 26.3±1.4
Maximum diameter of adrenal mass/cm 7.4±0.7
24 h UFC/μg 234±39
8:00 ACTH /(ng/L) 4.5±4.1
F/(μg/dL) 401.2±17.1
Control UFC (HDDST μg/24 h) 161±46
SBP/mmHg 149.3±5.2
DBP/mmHg 93.4±1.7
Blood glucose level/(mmol/L) 7.18±0.3
HbA1c/% 8.6±1.4
Total cholesterol/(mmol/L) 5.71±0.27
Triglyceride/(mmol/L) 2.0±0.3

图2

18-氟脱氧葡萄糖PET-CT显示肝、淋巴结、脊柱及胸骨多发转移"

图3

肾上腺皮质癌大体标本及病理特点"

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