Muthuvel Esakki, Chander Vimal, Srinivasan Chitra
Associate Professor, Department of Pathology, Saveetha Medical College, Chennai, Tamil Nadu, India.
Assistant Professor, Department of Pathology, Saveetha Medical College, Chennai, Tamil Nadu, India.
J Clin Diagn Res. 2017 Mar;11(3):EC50-EC53. doi: 10.7860/JCDR/2017/24223.9592. Epub 2017 Mar 1.
Paediatric primary liver tumours are the third largest group of solid abdominal neoplasms in children next to neuroblastoma and Wilms tumour, accounting for about 0.5% to 2% of all paediatric neoplasms, in which hepatoblastoma is the most common.
The present study was done to estimate the incidence of paediatric liver tumours over a period of five years and also, to study the clinical behaviour, alpha-fetoprotein correlation and histopathological features of paediatric liver tumours.
The details of patients treated for paediatric liver tumours for a period of five years were retrospectively retrieved from the tumour board and medical records. The gross features and all the slides were reviewed and the pathologic diagnosis was confirmed and clinicopathological correlation was then done.
A total of 39 paediatric liver tumours were detected during five years, of which 32 (82%) were malignant and seven (18%) were benign with a male to female ratio of 1:1.1. Hepatoblastoma was the most common liver tumour accounting for 28 cases (71.8%), of which 25 cases (89.4%) were of pure epithelial type. The second most common primary tumour was epithelioid haemangioendothelioma with six cases (15.4%) with female preponderance, followed by two cases each of hepatocellular carcinoma and undifferentiated embryonal sarcoma and one case of hepatocellular adenoma. Serum AFP level was increased in hepatoblastoma and in hepatocellular carcinoma, normal in hepatocellular adenoma and embryonal sarcoma. Serum AFP level was increased in few cases of epithelioid haemangioendothelioma.
The spectrum of liver tumours in children is different from that in the adults. Hepatoblastoma is the most common paediatric liver tumour, followed by epithelioid haemangioendothelioma. Through better understanding of pathological diagnosis, refined surgical staging, newer and more effective radiological techniques and standardized multimodal therapies, a substantial number of children diagnosed with this highly malignant tumour can expect to survive the disease.
小儿原发性肝肿瘤是儿童腹部实体肿瘤中的第三大类型,仅次于神经母细胞瘤和肾母细胞瘤,约占所有小儿肿瘤的0.5%至2%,其中肝母细胞瘤最为常见。
本研究旨在评估五年期间小儿肝肿瘤的发病率,并研究小儿肝肿瘤的临床行为、甲胎蛋白相关性及组织病理学特征。
回顾性检索肿瘤委员会和病历中五年期间接受小儿肝肿瘤治疗的患者详细信息。对大体特征和所有切片进行复查,确认病理诊断并进行临床病理相关性分析。
五年期间共检测到39例小儿肝肿瘤,其中32例(82%)为恶性,7例(18%)为良性,男女比例为1:1.1。肝母细胞瘤是最常见的肝肿瘤,占28例(71.8%),其中25例(89.4%)为单纯上皮型。第二常见的原发性肿瘤是上皮样血管内皮瘤,共6例(15.4%),女性居多,其次是肝细胞癌和未分化胚胎性肉瘤各2例,肝细胞腺瘤1例。肝母细胞瘤和肝细胞癌患者血清甲胎蛋白水平升高,肝细胞腺瘤和胚胎性肉瘤患者血清甲胎蛋白水平正常。少数上皮样血管内皮瘤患者血清甲胎蛋白水平升高。
儿童肝肿瘤谱与成人不同。肝母细胞瘤是最常见的小儿肝肿瘤,其次是上皮样血管内皮瘤。通过更好地理解病理诊断、完善手术分期、更新更有效的放射学技术以及标准化多模式治疗,大量被诊断患有这种高度恶性肿瘤的儿童有望存活。