Undifferentiated embryonal sarcoma with unusual features arising within mesenchymal hamartoma of the liver: Report of a case and review of the literature
Undifferentiated embryonal sarcoma with unusual features arising within mesenchymal hamartoma of the liver: Report of a case and review of the literature
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DOI：
10.1007/s10024001-0047-9
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发表时间：
2001-09-01
影响因子：
1.9
通讯作者：
Dehner, LP
中科院分区：
文献类型：
--
作者：
O'Sullivan, MJ;Swanson, PE;Dehner, LP
未分化胚胎肉瘤（UES）是一种罕见且高度恶性的肝肿瘤，几乎只影响儿科人群。它已经取代了恶性间叶瘤，前三个病例是在该诊断术语下描述的。由于胚胎肉瘤和肝脏间质错构瘤 (MHL) 的临床病理学重叠，长期以来人们就提出了这些实体之间的联系。然而，直到最近，这种联系仍然很脆弱。此前曾报道过两名十几岁女孩因肝脏间质错构瘤而出现 UES 病例。在 MHL 和 UES 中发现类似的遗传异常已经证实了它们之间的假设联系。还有两份报告称 UES 具有显着的囊肿化，其中一份与外周嗜酸性粒细胞增多相关，从而伪装成肝脏包虫囊肿。我们报告一例发生于一名患有 MHL 的小男孩的 UES 病例，具有不寻常的组织学特征，包括大的间皮囊肿和 Glisson 囊下的异位肾上腺皮质组织。
Undifferentiated embryonal sarcoma (UES) is a rare and highly malignant hepatic neoplasm, affecting almost exclusively the pediatric population. It has replaced malignant mesenchymoma, under which diagnostic term the first three cases were described. A link between embryonal sarcoma and mesenchymal hamartoma of the liver (MHL) has long been proposed, because of clinicopathologic overlaps of these entities; however, until recently, this association remained tenuous. Cases of UES arising in a background of mesenchymal hamartoma of the liver have previously been reported in two teenage girls. Discovery of a similar genetic abnormality in MHL and UES has clinched the supposed link between them. There have also been two reports of UES with prominent cystification, one associated with peripheral eosinophilia, and thereby masquerading as hydatid cyst of the liver. We report a case of UES arising in a young boy with MHL, with unusual histologic features, including large mesothelial-lined cysts and ectopic adrenal cortical tissue under Glisson's capsule.