Zuopeng Wang1, Gongbao Liu1, Hongqiang Sun2, Kai Li3, Kuiran Dong1, Yangyang Ma4, Shan Zheng1. 1. Department of Pediatric Surgery, Children's Hospital of Fudan University, 399 Wan yuan Road, Shanghai, 201102, People's Republic of China. 2. Department of Pediatric Surgery, Shandong Dezhou People's Hospital, Shandong, People's Republic of China. 3. Department of Pediatric Surgery, Children's Hospital of Fudan University, 399 Wan yuan Road, Shanghai, 201102, People's Republic of China. likai2727@163.com. 4. Department of Pathology, Children's Hospital of Fudan University, Shanghai, People's Republic of China.
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.
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.