陈琛君, 郭云飞, 马耿, 葛征, 陆如纲, 邓永继, 王利霞, 董隽. 儿童肾上腺皮质癌的诊断与预后[J]. 中国肿瘤临床, 2019, 46(13): 673-677. DOI: 10.3969/j.issn.1000-8179.2019.13.662
引用本文: 陈琛君, 郭云飞, 马耿, 葛征, 陆如纲, 邓永继, 王利霞, 董隽. 儿童肾上腺皮质癌的诊断与预后[J]. 中国肿瘤临床, 2019, 46(13): 673-677. DOI: 10.3969/j.issn.1000-8179.2019.13.662
Chen Chenjun, Guo Yunfei, Ma Geng, Ge Zheng, Lu Rugang, Deng Yongji, Wang Lixia, Dong Jun. Diagnosis and prognosis of adrenocortical carcinoma in children[J]. CHINESE JOURNAL OF CLINICAL ONCOLOGY, 2019, 46(13): 673-677. DOI: 10.3969/j.issn.1000-8179.2019.13.662
Citation: Chen Chenjun, Guo Yunfei, Ma Geng, Ge Zheng, Lu Rugang, Deng Yongji, Wang Lixia, Dong Jun. Diagnosis and prognosis of adrenocortical carcinoma in children[J]. CHINESE JOURNAL OF CLINICAL ONCOLOGY, 2019, 46(13): 673-677. DOI: 10.3969/j.issn.1000-8179.2019.13.662

儿童肾上腺皮质癌的诊断与预后

Diagnosis and prognosis of adrenocortical carcinoma in children

  • 摘要:
      目的  总结分析儿童肾上腺皮质癌的诊断与预后。
      方法  分析南京医科大学附属儿童医院2010年9月至2018年3月收治的10例肾上腺皮质癌患儿资料,分析其临床表现、实验室检查、辅助检查、病理和预后。
      结果  10例患儿年龄5个月至11岁9个月,平均年龄(53.3±48.4)个月;男性5例,女性5例。临床表现各异,主要表现为腹部包块、高血压、皮质醇症和外周性早熟。实验室检查乳酸脱氢酶升高8例,神经烯醇化酶升高5例,皮质醇升高4例,部分性激素异常2例。辅助检查全腹部CT增强表现为肾上腺区域形态不规则的肿块,密度不均匀,包块内可有坏死、钙化,部分伴有周围组织的浸润。病理提示肿瘤细胞多形性,核分裂像多见,核分裂指数高,间质血窦丰富,可见坏死,肿瘤浸润,血管侵犯,Weiss评分≥3分。年龄、LDH、皮质醇、Weiss评分、肿瘤大小、分期、手术切除率在预后因素中差异无统计学意义(P>0.05)。
      结论  对于肾上腺占位的患儿,结合其临床表现、实验室检查、辅助检查早期做出皮质癌的诊断,选择合适的治疗方案对提高患儿生存率至关重要;结合病理学检查可以较好地与其他肾上腺包块鉴别。

     

    Abstract:
      Objective  To summarize the diagnosis and prognosis of adrenocortical carcinoma in children.
      Methods  Medical records of 10 pediatric patients with adrenocortical cancer treated in Children's Hospital of Nanjing Medical University from September 2010 to March 2018 were retrospectively reviewed, and their clinical manifestation, laboratory, auxiliary, and pathological examination results, and prognosis were analyzed.
      Results  The average age of all patients (5 male and 5 female pediatric patients) was (53.3+48.4) months. Clinical manifestations included abdominal mass, hypertension, cortisol level, and peripheral precocious puberty. Laboratory tests showed that lactate dehydrogenase, neuroenolase, and cortisol levels were high in some patients, and sex hormones were abnormal. Auxiliary examination using the abdominal computed tomography enhancement showed irregular an adrenal gland, uneven density, necrosis, mass calcification, and some infiltration into the surrounding tissue. Pathological examination suggests tumor cell pleomorphism, more frequent pathological division, high mitotic index, abundant interstitial sinusoid, necrosis, tumor infiltration, vascular invasion, and Weiss score of ≥ 3 points. Lactate acid dehydrogenase and cortisol levels; Weiss score; and tumor size, staging, and resection rate were not statistically different among prognostic factors.
      Conclusions  For pediatric patients with adrenal masses, the survival rate should be improved by combining the clinical manifestation, laboratory examination, and auxiliary examination for the early diagnosis of adrenocortical carcinoma and to select the appropriate treatment. Combined with pathological results, adrenocortical carcinoma can be well differentiated from other adrenal masses.

     

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