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Objective To investigate the current situation of patients with adrenocortical carcinoma (ACC) in China,for providing references to clinical diagnosis and treatment of ACC. Methods The information was collected of patients (from adrenocortical tumor patient group) definitely diagnosed as ACC from 2010 to 2019 by questionnaire, including gender, age, clinical manifestations, course of disease, surgical and adjuvant treatment methods and curative effects, and retrospective analysis was conducted. Results A total of 156 ACC patients were included with a male to female ratio of 1:1.33. Patients aged 40-60 years accounted for the highest proportion. Except one patient with bilateral adrenal tumors, the others were unilateral involved, and the proportion of left adrenal tumor was slightly higher than that of the right side. Eighty-one (51.9%) patients had adrenocortical hormone hypersecretion, 33 patients (21.2%) of them had manifestations of hypercortisolism such as full moon face, buffalo back,centripetal obesity, and muscle weakness, and 12 patients (7.7%) had spontaneous hypersecretion of two or more adrenal hormones, including cortisol. One hundred and forty-seven patients underwent surgery, of which open surgery accounted for the highest proportion (n=86, 55.1%), followed by laparoscopic surgery (n=51, 32.7%). The most common distant metastasis sites were liver(n=50, 32.1%), lung (n=46, 29.5%) and retroperitoneal lymph nodes (n=34, 21.8%). 79.5% of the studied population (n=124)used mitotane for adjuvant treatment, and 66.9% (n=83) of the patients started medication due to postoperative recurrence and metastasis. The median stable dosage of mitotane was 2.0 g. 64.4% (n=58) of the patients could achieve partial or complete response,26.7% (n=24) of the patients were in stable condition, and the remaining 8.9% (n=8) had disease progression. After the combined application of other adjuvant treatments was included in the analysis, no significant difference was observed in the response among the three groups of mitotane alone, mitotane combined with chemotherapy, and mitotane combined with other treatments (P>0.05). Conclusions Functional occupancy accounted for more than 50% in ACC patients, and the proportion of cortisol hypersecretion is the highest. Some patients are prone to retroperitoneal lymph node metastasis, therefore, lymph node dissection should be performed routinely in radical operations. As an essential adjuvant therapy, mitotane possesses high response rate when used alone or in conjunction with chemotherapy or other adjuvant medicines.
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| 科 Family | 属数 Number of genus | 种数 Number of species | 占总种数比例 Percentage of total species (%) | 属 Genus | 种数 Number of species | 占总种数比例 Percentage of total species (%) |
|---|---|---|---|---|---|---|
| 鹅膏菌科Amanitaceae | 2 | 11 | 5.26 | 鹅膏菌属 Amanita | 10 | 4.78 |
| 小菇科 Mycenaceae | 2 | 12 | 5.74 | 丝盖伞属 Inocybe | 5 | 2.39 |
| 多孔菌科 Polyporaceae | 8 | 14 | 6.70 | 蜡蘑属 Laccaria | 5 | 2.39 |
| 红菇科 Russulaceae | 3 | 23 | 11.00 | 小皮伞属 Marasmius | 6 | 2.87 |
| 小菇属 Mycena | 11 | 5.26 | ||||
| 光柄菇属 Pluteus | 5 | 2.39 | ||||
| 红菇属 Russula | 17 | 8.13 | ||||
| 栓菌属 Trametes | 5 | 2.39 |
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