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.
方法:我院收治一名7岁女童,双侧乳房增大2个月,多饮,多尿,多食,高血糖症,和显著的体重增加。计算机断层扫描(CT)显示肝脏左内叶7.2cm×6.9cm×5.3cm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)显示上垂体增大。血液生化结果为:空腹血糖19.7mmol/L,雌二醇为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,绒毛膜促性腺激素正常.病人对肝脏肿瘤进行了完整的手术切除,术后组织病理学诊断为HCA。手术后,注射胰岛素,血糖水平稳定.在36个月的随访期间,使用肝脏的彩色多普勒超声既没有发现肿瘤复发也没有发现明显的异常。孩子的性早熟目前受到控制。
结论:HCA在患有肝脏肿瘤的儿童中特别罕见,儿童HCA发育的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型,III,IV,半乳糖血症,免疫缺陷,先天性门体分流术(CPSS),心脏肝病状态-Fontan手术后,Hurler综合征,家族性腺瘤性息肉病,种系HNF1A突变,和年轻的3型糖尿病。大多数HCA是在体检中检测到的,没有临床症状,有些患者可能会出现腹痛等症状,腹胀,和腹部。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α-fetoprofein(AFP)水平正常。明确的诊断主要依靠组织病理学检查。因为HCA会破裂出血并变成恶性。建议在发现后早期手术治疗。