Kuhn Susanne A, Hanisch Uwe-Karsten, Ebmeier Kristian, Beetz Christian, Brodhun Michael, Reichart Rupert, Ewald Christian, Deufel Thomas, Kalff Rolf
Department of Neurosurgery, Medical Centre, Friedrich-Schiller-University, Erlanger Allee 101, 07747 Jena, Germany.
Neurosurg Rev. 2007 Apr;30(2):143-9; discussion 149. doi: 10.1007/s10143-007-0069-5. Epub 2007 Feb 27.
The case of a 7-year-old boy suffering from a supratentorial primitive neuroectodermal tumour (sPNET) at the age of 5 is presented. The tumour has been characterized by astrocytic areas within the sPNET revealing malignant transformation up to a multiform glioblastoma during the course of the disease. The clonal origin of both tumour components was established by loss of heterozygosity (LOH) analysis. Clinically, the tumour showed an aggressive biological behaviour with two recurrences. We discuss this very rare case and the first description of the clonal origin of distinct and distinguishable tumour components taking into consideration published literature.
本文报告了一例5岁时患幕上原始神经外胚层肿瘤（sPNET）的7岁男孩病例。该肿瘤的特征是在sPNET内存在星形细胞区域，在疾病过程中显示出向多形性胶质母细胞瘤的恶性转化。通过杂合性缺失（LOH）分析确定了两种肿瘤成分的克隆起源。临床上，该肿瘤表现出侵袭性生物学行为，出现了两次复发。我们结合已发表的文献讨论了这一非常罕见的病例以及对不同且可区分的肿瘤成分克隆起源的首次描述。
