Azar Georgia M, Kutin N, Kahn Ellen
Department of Pathology, New York University Medical Center, New York, NY, USA.
Pediatr Dev Pathol. 2003 May-Jun;6(3):265-9. doi: 10.1007/s10024-003-7073-8. Epub 2003 Apr 9.
We report a hepatic tumor in an adolescent that does not fit into any of the described categories of liver tumors. The patient presented with hepatomegaly, abdominal pain, and normal liver function test; the tumor was cystic in imaging studies. The resected specimen, result of a partial hepatectomy, measured 21 cm and was multicystic with solid areas. Microscopically, the cysts were lined by a mucous-producing or intestinal-type epithelium, associated with smooth muscle and small mucous-producing glands. The solid component contained fibrous and adipose tissue, smooth muscle and thick-walled vessels. Aneuploidy was demonstrated by flow cytometry. We interpreted the tumor as having features of a mesenchymal hamartoma and congenital solitary nonparasitic cyst. It is conceivable that the lesions originated with small peribiliary glands with dilatation and intestinal metaplasia.
我们报告了一例青少年肝脏肿瘤,其不符合已描述的任何肝脏肿瘤类别。患者表现为肝肿大、腹痛,肝功能检查正常;影像学检查显示肿瘤为囊性。经部分肝切除获得的切除标本长21厘米,为多囊性且伴有实性区域。显微镜下,囊肿内衬有产生黏液的或肠型上皮,伴有平滑肌和小的黏液分泌腺。实性成分包含纤维组织、脂肪组织、平滑肌和厚壁血管。流式细胞术显示有非整倍体现象。我们将该肿瘤解释为具有间叶性错构瘤和先天性孤立性非寄生虫性囊肿的特征。可以想象,这些病变起源于小的胆管周围腺体,伴有扩张和肠化生。