Lauwers G Y, Grant L D, Donnelly W H, Meloni A M, Foss R M, Sanberg A A, Langham M R
Department of Pathology and Laboratory Medicine, University of Florida College of Medicine, Gainesville 32610, USA.
Am J Surg Pathol. 1997 Oct;21(10):1248-54. doi: 10.1097/00000478-199710000-00018.
We report the case of a hepatic undifferentiated (embryonal) sarcoma (UES) arising within a mesenchymal hamartoma (MH) in a 15-year-old girl. Mapping of the tumor demonstrated a typical MH transforming gradually into a UES composed of anaplastic stromal cells. When evaluated by flow cytometry, the MH was diploid and the UES showed a prominent aneuploid peak. Karyotypic analysis of the UES showed structural alterations of chromosome 19, which have been implicated as a potential genetic marker of MH. The histogenesis of MH and UES is still debated, and reports of a relationship between them, although suggested on the basis of histomorphologic similarities, have never been convincing. The histologic, flow cytometric, and cytogenetic evidence reported herein suggests a link between these two hepatic tumors of the pediatric population.
我们报告了一名15岁女孩的病例，其肝脏间叶性错构瘤（MH）内发生了肝未分化（胚胎性）肉瘤（UES）。肿瘤定位显示典型的MH逐渐转变为由间变的基质细胞组成的UES。通过流式细胞术评估时，MH为二倍体，而UES显示出明显的非整倍体峰。UES的核型分析显示19号染色体存在结构改变，这被认为是MH的潜在遗传标志物。MH和UES的组织发生仍存在争议，尽管基于组织形态学相似性曾提示它们之间存在关系，但从未令人信服。本文报道的组织学、流式细胞术和细胞遗传学证据表明这两种儿童肝脏肿瘤之间存在联系。
