Halefoglu A M, Oz A
Department of Radiology, Sisli Etfal Training and Research Hospital, Istanbul, Turkey.
JBR-BTR. 2014 Jul-Aug;97(4):248-50. doi: 10.5334/jbr-btr.91.
Primary undifferentiated embryonal sarcoma (UES) of the liver is a highly malignant mesenchymal origin tumor and has a peak incidence between the ages of 6 and 10 years. We hereby report a case of primary UES of the liver in a 7-year-old male patient who initially was misdiagnosed and treated as hydatid cyst of the liver. The tumor was occupying almost the entire right lobe of the liver and had a mostly cystic appearance with some solid components in it. Because hydatid disease is endemic in this region, it can often lead to misdiagnosis. The correct diagnosis was established after a biopsy and following neo-adjuvant chemotherapy the patient underwent a successful right hepatic lobectomy with complete resection of the tumor. The patient also received adjuvant chemotherapy and is currently disease-free in the present six month period. Primary UES of the liver has a predominantly solid appearance on US in contrast to its mostly cystic appearance on CT and MRI. These paradoxical imaging findings should be kept in mind in order to be able to distinguish this rare tumor from other entities, especially hydatid cyst. Thus, early diagnosis and prompt surgical resection of these tumors together with adjuvant and/or neo-adjuvant chemotherapy can provide complete remission.
肝脏原发性未分化胚胎性肉瘤(UES)是一种高度恶性的间叶源性肿瘤,发病高峰年龄在6至10岁之间。我们在此报告一例7岁男性肝脏原发性UES病例,该患者最初被误诊为肝包虫囊肿并接受了相应治疗。肿瘤几乎占据了肝脏的整个右叶,大部分呈囊性外观,内部有一些实性成分。由于该地区包虫病流行,常导致误诊。经活检后确诊,新辅助化疗后患者成功接受了右肝叶切除术,肿瘤被完全切除。患者还接受了辅助化疗,目前在六个月内无疾病复发。肝脏原发性UES在超声检查中主要表现为实性,而在CT和MRI上大多呈囊性。应牢记这些矛盾的影像学表现,以便能够将这种罕见肿瘤与其他疾病,尤其是肝包虫囊肿区分开来。因此,早期诊断并及时对这些肿瘤进行手术切除,同时辅以辅助和/或新辅助化疗,可实现完全缓解。