Hepatic adenoma in a 7-year-old girl: a case report and literature review.
Hepatic adenoma in a 7-year-old girl: a case report and literature review.
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DOI：
10.1186/s12887-023-04209-5
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发表时间：
2023-08-24
期刊：
影响因子：
2.4
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中科院分区：
文献类型：
--
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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。在儿童中， <； 占肝脏肿瘤的5%。我们报告一例7岁女孩的HCAs，患有雌激素和葡萄糖失衡。1例7岁女童，双侧乳房肿大2个月，多饮、多尿、多食、高血糖，体重明显增加。CT显示左内叶有7.2 cm×6.9 cm×5.3 cm的圆形肿块，卵巢超声显示双侧卵巢内有多个卵泡，头颅磁共振成像(MRI)显示垂体上叶增大。血液学和生化结果如下：空腹血糖19.7mmoL/L，雌二醇122.9 pmoL/L，促卵泡激素10.81IU/L，促黄体生成素10.99IU/L，胰岛素样生长因子1513 ng/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。患者接受了完整的肝肿瘤手术切除，术后组织病理学诊断为肝细胞癌。手术后注射了胰岛素，血糖水平稳定。在36个月的随访期内，肝脏彩色多普勒超声未发现肿瘤复发或明显异常。孩子的青春期早熟目前得到了控制。HCA在患有肝脏肿瘤的儿童中尤其罕见，儿童HCAs的危险因素包括性激素失衡、肥胖、Fanconi贫血(FA)、糖原储存病(GSD)I、III和IV型、半乳糖血症、免疫缺陷、先天性门体分流(CPSS)、Fontan手术后的心脏病、Hurler综合征、家族性腺瘤性息肉病、胚系HNF1a突变和幼年3型糖尿病。大多数HCAs是在没有临床症状的体检中发现的，一些患者可能会出现腹痛、腹胀和腹胀等症状。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(α)水平正常。确诊主要依靠组织病理学检查。因为HCAs可能破裂、出血并变得恶性。建议发现后早期手术治疗。
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.
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