Brown Angela E, Leibundgut Kurt, Niggli Felix K, Betts David R
Department of Oncology, University Children's Hospital, Zurich, Switzerland.
Cancer Genet Cytogenet. 2006 Oct 15;170(2):175-9. doi: 10.1016/j.cancergencyto.2006.06.009.
Pineoblastoma represents a class of primitive neuroectodermal tumors (PNET) with poorly differentiated neuroepithelial cells that are histologically indistinguishable from medulloblastomas. It is a rare tumor, typically arising in childhood, and to date only a few cytogenetic cases have been published. We report four new cases in which conventional cytogenetics demonstrated the presence of an abnormal clone. The tumors showed a variety of ploidy levels, from hypodiploid to hypertetraploid. Both structural and numerical aberrations were frequent, and in three out of the four cases a large degree of cell-to-cell variation was observed. The most frequently involved chromosome in structural rearrangements was chromosome 1, observed in three of the four cases. The short arm was involved in two of the three cases; in the third case, the anomaly was in the long arm. Two cases showed unbalanced gain of chromosome 17q, one of them showing i(17)(q10). Together, the four cases illustrate the complex karyotypic nature of this tumor type and represent a step toward determining whether a nonrandom cytogenetic picture exists and how this may be related to other associated tumor types.
松果体母细胞瘤是一类原始神经外胚层肿瘤(PNET),其神经上皮细胞分化不良,在组织学上与髓母细胞瘤无法区分。它是一种罕见的肿瘤,通常发生于儿童期,迄今为止仅有少数细胞遗传学病例被报道。我们报告了4例新病例,其中常规细胞遗传学显示存在异常克隆。这些肿瘤呈现出多种倍体水平,从亚二倍体到超四倍体。结构和数目畸变都很常见,在4例中的3例观察到了很大程度的细胞间变异。结构重排中最常涉及的染色体是1号染色体,在4例中的3例中观察到。3例中的2例涉及短臂;在第3例中,异常发生在长臂。2例显示17q染色体不平衡增加,其中1例显示i(17)(q10)。这4例共同说明了这种肿瘤类型复杂的核型性质,并代表了朝着确定是否存在非随机细胞遗传学图谱以及这可能如何与其他相关肿瘤类型相关迈出的一步。