Journal of Peking University(Health Sciences) ›› 2019, Vol. 51 ›› Issue (2): 298-301. doi: 10.19723/j.issn.1671-167X.2019.02.019

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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

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

CLC Number: 

  • R737

Figure 1

Characteristic of age groups distribution in adrenal cortical adenocarcinoma"

Table 1

Signs and symptoms of adrenal cortical adenocarcinoma"

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

Table 2

Clinical data and adrenocorticotropic hormone levels of adrenal cortical adenocarcinoma"

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

Figure 2

18F-FDG PET-CT Showed multiple metastases of liver, lymph nodes, spine and sternum"

Figure 3

Gross and pathological features of adrenocortical carcinoma A, enhanced CT showed of massive cortical carcinoma of the adrenal region; B, general specimen of adrenocortical carcinoma; C, high power microscopic appearance of an adrenal cortical carcinoma (×200)."

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