Department of pathology, Kunming Children's Hospital, 288 Qianxing Road, Kunming, 650028, Yunnan, China.
Second People's Hospital of Yunnan Province, 176 Qingnian Road, Kunming, 650034, Yunnan, China.
BMC Pediatr. 2023 Aug 24;23(1):420. doi: 10.1186/s12887-023-04209-5.
Hepatocellular adenomas (HCAs) are rare benign tumors of the liver that occur predominantly in women taking oral contraceptives. In children, HCAs comprise < 5% of hepatic tumors. We report a case of HCAs in a 7-year-old girl with estrogen and glucose imbalance.
A 7-year-old girl was presented to our hospital with bilateral breast enlargement for 2 months, polydipsia, polyuria, polyphagia, hyperglycemia, and significant weight gain. Computed tomography (CT) showed a 7.2 cm×6.9 cm×5.3 cm round-shaped mass in the left inner lobe of the liver, ovarian ultrasound showed multiple follicles in the ovaries bilaterally, and cranial magnetic resonance imaging (MRI) showed an enlarged superior pituitary. Hematological and biochemical results were as follows: fasting glucose was 19.7 mmol/L, estradiol was 122.9 pmol/L, follicle-stimulating hormone 10.81 IU/L, luteinizing hormone 10.99 IU/L, insulin-like growth factor 1,513 ng/mL, glutamine aminotransferase 86 U/L, and alkaline phosphatase 362 U/L. Thyroid functions, methemoglobin, fetal protein, carcinoembryonic antigen, and chorionic gonadotropin were normal. The patient had a complete surgical resection of the liver tumor, and the postoperative histopathological diagnosis was HCAs. After the surgery, insulin was injected and the glucose levels were stable. During the 36-month follow-up period, neither tumor recurrence nor significant abnormalities were detected using color Doppler ultrasound of the liver. The child's precocious puberty is currently under control.
HCAs are particularly rare in children with liver tumors, and risk factors for the development of HCAs in children include sex hormone imbalance, obesity, Fanconi anemia (FA), glycogen storage diseases (GSDs) type I, III, and IV, galactosemia, immunodeficiency, congenital portosystemic shunts (CPSS), cardiac hepatopathy status-post Fontan procedure, Hurler syndrome, familial adenomatous polyposis, germline HNF1A mutations, and maturity-onset diabetes of the young type 3. Most HCAs are detected during a physical examination without clinical symptoms, and some patients may present with symptoms such as abdominal pain, abdominal distension, and abdominal masse. Serum liver function tests can show increased alkaline phosphatase (ALP) and γ- glutamyl transferase (GT), whereas α-Fetoprofein (AFP) levels are normal. The definitive diagnosis relies mainly on histopathological examination. Because HCAs can rupture and bleed and become malignant. Early surgical treatment is recommended after detection.
肝细胞腺瘤（HCA）是一种罕见的肝脏良性肿瘤，主要发生在服用口服避孕药的女性中。在儿童中，HCA 占肝脏肿瘤的比例<5%。我们报告了一例 7 岁女孩因雌激素和葡萄糖失衡而发生 HCA 的病例。
一名 7 岁女孩因双侧乳房增大 2 个月、多饮、多尿、多食、高血糖和明显体重增加而到我院就诊。计算机断层扫描（CT）显示左肝内叶有一个 7.2cm×6.9cm×5.3cm 的圆形肿块，卵巢超声显示双侧卵巢有多个卵泡，头颅磁共振成像（MRI）显示垂体增大。血液生化结果如下：空腹血糖 19.7mmol/L，雌二醇 122.9pmol/L，卵泡刺激素 10.81IU/L，黄体生成素 10.99IU/L，胰岛素样生长因子 1 513ng/ml，谷氨酰胺转氨酶 86U/L，碱性磷酸酶 362U/L。甲状腺功能、高铁血红蛋白、胎儿蛋白、癌胚抗原和绒毛膜促性腺激素正常。患者行肝肿瘤完全切除术，术后组织病理学诊断为 HCA。术后给予胰岛素注射，血糖水平稳定。在 36 个月的随访期间，肝彩色多普勒超声均未发现肿瘤复发或明显异常。患儿性早熟目前得到控制。
HCA 在儿童肝脏肿瘤中特别罕见，儿童 HCA 的发病危险因素包括性激素失衡、肥胖、范可尼贫血（FA）、糖原贮积病（GSD）I、III、IV 型、半乳糖血症、免疫缺陷、先天性门体分流（CPSS）、Fontan 术后心肝综合征、Hurler 综合征、家族性腺瘤性息肉病、种系 HNF1A 突变、青少年 3 型糖尿病。大多数 HCA 在体检时被发现，没有临床症状，部分患者可能出现腹痛、腹胀、腹部肿块等症状。血清肝功能检查可显示碱性磷酸酶（ALP）和γ-谷氨酰转移酶（GT）升高，而甲胎蛋白（AFP）水平正常。明确诊断主要依靠组织病理学检查。因为 HCA 可能破裂出血并恶变。建议在发现后尽早进行手术治疗。
