Chapman A D, Pritchard S C, Yap W W, Rooney P H, Cockburn J S, Hutcheon A W, Nicolson M C, Kerr K M, McLeod H L
Department of Pathology, University of Aberdeen, Aberdeen, Scotland, United Kingdom.
Cancer. 2001 Feb 15;91(4):779-84.
Primary pulmonary osteosarcoma is an extremely rare malignancy. To date, only 12 cases have been reported, with a high mortality rate. The authors report on a newly diagnosed patient and describe investigations that were performed using immunohistochemistry and comparative genomic hybridization (CGH).
The clinical course of a woman age 37 years is presented. Along with routine histologic examination, immunohistochemistry was used to demonstrate differentiation-associated proteins, oncoproteins, and other markers; CGH analysis for genomic alterations; and histochemistry to demonstrate alkaline phosphatase activity.
Immunohistochemical analysis showed varying expression patterns using antibodies against a panel of tumor markers. Most notable was high overexpression of BCL-2 and cyclin D. CGH analysis showed that this neoplasm contained a much higher level of genetic aberrations compared with skeletal osteosarcoma.
This tumor exhibited features common to skeletal osteosarcomas but also had some unique features. Genome analysis suggests that this tumor has several genetic aberrations in common with extraskeletal osteosarcoma. The novel regions of instability identified within the tumor genome may contribute toward the unique tumor phenotype and relative chemoresistance.
原发性肺骨肉瘤是一种极其罕见的恶性肿瘤。迄今为止，仅报道了12例，死亡率很高。作者报告了1例新诊断的患者，并描述了使用免疫组织化学和比较基因组杂交（CGH）进行的研究。
介绍了一名37岁女性的临床病程。除常规组织学检查外，免疫组织化学用于显示分化相关蛋白、癌蛋白和其他标志物；CGH分析用于检测基因组改变；组织化学用于显示碱性磷酸酶活性。
免疫组织化学分析显示，使用一组肿瘤标志物抗体时表达模式各异。最显著的是BCL-2和细胞周期蛋白D的高表达。CGH分析表明，与骨骨肉瘤相比，该肿瘤的遗传畸变水平要高得多。
该肿瘤表现出骨骨肉瘤的常见特征，但也有一些独特特征。基因组分析表明，该肿瘤与骨外骨肉瘤有一些共同的遗传畸变。在肿瘤基因组中确定的新的不稳定区域可能导致独特的肿瘤表型和相对化疗耐药性。
