Massara F M, Tonello M, Forni M, Fruttero A, Lantermo R, Pugno F, Cordero di Montezemolo L, Ruà S
Istituto di Discipline Pediatriche, Università di Torino, Italia.
Pediatr Med Chir. 1996 Mar-Apr;18(2):141-8.
Nuclear DNA content was determined on paraffin blocks of 28 neuroblastic tumors retrieved from Surgical Pathology files. In 26 cases cytofluorimetric analysis for DNA content was satisfactory. 8 tumor were diploid, 12 were aneuploid and 6 tetraploid. All but one of the children with diploid neuroblastoma (NB) died after a survival ranging from 14 to 32 months. The only surviving child had a Stage I thoracic ganglioneuroblastoma (GNB) and was alive after 27 months. Conversely 11 of 12 patients with aneuploid tumors were alive with prolonged follow-up. Two children are alive after a period exceeding 146 months. The only exception in this group of aneuploid tumors was a child with Stage IV neuroblastoma of the adrenal gland with pleuro pulmonary metastasis who died after 6 months. In the group with tetraploid tumors 3 patients died (2 children with Stage III and IV died early while the third, with thoracic Stage II GNB, survived for 72 months). Two patients with tetraploid GNB Stage II and III are alive 65 and 72 months after diagnosis respectively. Another child with Stage IV adrenal NB is alive after 14 months but with metastatic spread to bones, bone marrow and lymph nodes. Cox analysis of the cases demonstrated ploidy as an independent prognostic factor. These results are in agreement with other molecular analysis linking near-ploidy of Neuroblastic tumors to poor prognosis. Ploidy, as detected by flow cytometry for nuclear DNA content, may represent an important prognostic factor in Neuroblastic tumors. The advantage of flow cytometry over other techniques of molecular analysis is represented by the simple methodology suitable for fixed and paraffin embedded tissue, even on retrieval material.
对从外科病理档案中获取的28例神经母细胞瘤石蜡块进行了核DNA含量测定。在26例中，DNA含量的细胞荧光分析结果令人满意。8例肿瘤为二倍体，12例为非整倍体，6例为四倍体。除1例二倍体神经母细胞瘤（NB）患儿外，其余患儿均在14至32个月的生存期后死亡。唯一存活的患儿患有I期胸段神经节神经母细胞瘤（GNB），27个月后仍存活。相反，12例非整倍体肿瘤患者中有11例在长期随访后仍存活。2例患儿在超过146个月后仍存活。在这组非整倍体肿瘤中，唯一的例外是1例患有肾上腺神经母细胞瘤伴胸膜肺转移的IV期患儿，6个月后死亡。在四倍体肿瘤组中，3例患者死亡（2例III期和IV期患儿早期死亡，第3例胸段II期GNB患儿存活72个月）。2例II期和III期四倍体GNB患者分别在诊断后65个月和72个月仍存活。另1例IV期肾上腺NB患儿在14个月后仍存活，但已发生骨、骨髓和淋巴结转移。对这些病例的Cox分析表明，倍性是一个独立的预后因素。这些结果与其他将神经母细胞瘤近倍性与不良预后联系起来的分子分析结果一致。通过流式细胞术检测核DNA含量所确定的倍性，可能是神经母细胞瘤的一个重要预后因素。流式细胞术相对于其他分子分析技术的优势在于其简单的方法，适用于固定和石蜡包埋组织，甚至是存档材料。
