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肝脏良性间叶性错构瘤(HMH)——病例报告

Benign Hepatic Mesenchymal Hamartoma (HMH) - A Case Report.

作者信息

Patel Shaileshkumar Radheshyam, Misra Vatsala, Verma Kachnar, Gupta Pooja, Dhingra Vishal

机构信息

Junior Resident, Department of Pathology, Motilal Nehru Medical College , Allahabad, Uttar Pradesh, India .

Head and Professor, Department of Pathology, Motilal Nehru Medical College , Allahabad, Uttar Pradesh, India .

出版信息

J Clin Diagn Res. 2014 Mar;8(3):119-20. doi: 10.7860/JCDR/2014/6522.4129. Epub 2014 Mar 15.

Abstract

A one-year-old male child presented with progressive enlargement of abdomen and loss of appetite which he had suffered from, since 4 months. Physical examination showed a palpable mass in right hypochondrium. Ultrasonography and MRI showed a solid mass arising from right lobe of liver. The resected mass was well encapsulated, yellowish white in colour and it measured 20 x 18 x 17cm. Cut surface showed a Nutmeg like appearance. Histology showed a well-defined, fibrous capsule with numerous ducts which were lined by flat to low cuboidal epithelium and which were surrounded by concentric layers of fibrous tissue which showed myxoid changes at places. Intervening area showed normal appearing hepatocytes with clear cell changes, dilated congested sinusoids, along with extramedullary haematopoiesis. Based on distinctive morphological features, a histological diagnosis of Benign Hepatic Mesenchymal Hamartoma of liver was made. IHC was not done, as no specific marker has been described for making a differential diagnosis. Benign Hepatic Mesenchymal Hamrtoma (HMH) of liver is an extremely rare tumour of childhood and therefore, the case was considered worth documentation.

摘要

一名一岁男童自4个月大起出现腹部进行性增大和食欲不振的症状。体格检查发现右季肋部可触及肿块。超声检查和磁共振成像显示肝脏右叶有一个实性肿块。切除的肿块包膜完整,呈黄白色,大小为20×18×17厘米。切面呈肉豆蔻样外观。组织学检查显示有一个界限清楚的纤维性包膜,内有许多导管,导管内衬扁平至低柱状上皮,周围有同心层状纤维组织,部分区域呈黏液样改变。其间区域可见外观正常的肝细胞伴有透明细胞改变、扩张充血的血窦以及髓外造血。基于独特的形态学特征,作出了肝脏良性间叶性错构瘤的组织学诊断。由于尚未描述用于鉴别诊断的特异性标志物,故未进行免疫组化检查。肝脏良性间叶性错构瘤(HMH)是一种极其罕见的儿童肿瘤,因此,该病例被认为值得记录。

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Hepatic mesenchymal hamartoma.
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