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Clinical characteristics and prognosis of adrenocortical tumors in children

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Abstract

Purpose

The purpose of this study was to review the clinical characteristics and prognosis of children with adrenocortical tumors (ACT).

Methods

We retrospectively reviewed the medical records of 28 patients with ACT at our hospital between March 2010 and March 2017.

Results

The main clinical presentations were sexual prematurity (n = 17) and Cushing’s syndrome (n = 15). All patients without metastasis underwent complete resection by laparotomy (n = 19) or laparoscopic surgery (n = 9). Pathological diagnosis confirmed adrenocortical carcinomas (ACC, n = 12) and adrenocortical adenomas (ACA, n = 16). Dehydroepiandrosterone (939.4 ± 148.2 µg/dl vs 630.9 ± 376.3 µg/dl; p = 0.031) and testosterone (235.7 ± 89.1 ng/dl vs 164.6 ± 47.5 ng/dl; p = 0.012) were significantly increased in ACC compared with ACA. The ACC tumor volumes were larger than those in ACA (107.5 ± 69 vs 25.5 ± 23.1 cm3; average diameter 6 cm vs 4 cm p = 0.001) and the immunochemical expression of Ki-67 was higher in ACC than in ACA (30.2 ± 22.7 vs 9.9 ± 4.9 p = 0.013). The mean follow-up of patients with ACA was 40 ± 23 months without recurrence. Seven patients with ACC had postoperative distant metastases and five patients died within 2 years. Five patients with ACC survived with a median follow-up of 27 months. The 2-year overall survival was 44.6%.

Conclusions

Patients with ACC had significantly larger tumor volumes than those with ACA. The discordantly elevated serum levels of sexual corticosteroid hormones and lactate dehydrogenase may predict the malignant nature of these tumors. The prognosis of patients with ACA was good, while those with ACC had high postoperative metastasis and mortality rates.

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Funding

This study was sponsored by the Science and Technology Commission of Shanghai Municipality (15411961800, Kai Li), the Personnel Training Program for Distinguished Medical Young Scholars (2017 Kai Li), the New Hundred People Plan of Shanghai Health and Family Planning Commission (2017 BR052 Kai Li), the Shanghai Sailing Program (17YF1401400, Zuopeng Wang), and the Youth Fund of Shanghai Health and Family Planning Commission Clinical Research Special Plan (20184Y0212 Zuopeng Wang).

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Author notes

  1. Zuopeng Wang and Gongbao Liu contributed equally to this work.

Authors and Affiliations

  1. Department of Pediatric Surgery, Children’s Hospital of Fudan University, 399 Wan yuan Road, Shanghai, 201102, People’s Republic of China

    Zuopeng Wang, Gongbao Liu, Kai Li, Kuiran Dong & Shan Zheng

  2. Department of Pediatric Surgery, Shandong Dezhou People’s Hospital, Shandong, People’s Republic of China

    Hongqiang Sun

  3. Department of Pathology, Children’s Hospital of Fudan University, Shanghai, People’s Republic of China

    Yangyang Ma

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  1. Zuopeng Wang
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  2. Gongbao Liu
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  4. Kai Li
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Correspondence to Kai Li.

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The authors have no conflicts of interest to declare.

Ethical approval

All procedures performed in the studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and in accordance with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Informed consent

Written informed consent was obtained from all patients or guardians enrolled in this study.

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Wang, Z., Liu, G., Sun, H. et al. Clinical characteristics and prognosis of adrenocortical tumors in children. Pediatr Surg Int 35, 365–371 (2019). https://doi.org/10.1007/s00383-018-4409-z

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  • DOI: https://doi.org/10.1007/s00383-018-4409-z

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