Moritake Hiroshi, Sugimoto Tohru, Asada Yujiro, Yoshida Mitsuaki A, Maehara Yoshihiko, Epstein Alan L, Kuroda Hiroshi
Department of Pediatrics, Miyazaki Medical College, 5200 Kihara, Kiyotake, Miyazaki, Japan.
Cancer Genet Cytogenet. 2002 May;135(1):48-56. doi: 10.1016/s0165-4608(01)00641-0.
Clear cell sarcoma (CCS), malignant melanoma of soft parts, is a rare malignant tumor with a poor prognosis. In this study, a CCS cell line, designated MP-CCS-SY, was established from a metastatic tumor of a 17-year-old Japanese girl that originated in the left Achilles tendon. A small number of melanosomes were detected in the cytoplasm by electron microscopy. The melanosomes immunoreacted with two melanoma-associated antibodies, HMB45 and Melan-A. A Western blot demonstrated the existence of a Melan-A antigen in this cell line. Although a t(12;22)(q13;q12), which is characteristic of CCS, was not identified by a chromosomal analysis with conventional banding techniques, fluorescence in situ hybridization analysis with painting probes of chromosomes 12 and 22 revealed the insertion of a chromosome 12 fragment into one of the long arms of chromosome 22. The chimeric EWS/ATF1 transcript was detected by the reverse transcriptase polymerase chain reaction. Extra copies and structural abnormalities of chromosome 8 were observed. Overexpression of c-myc mRNA was detected by Northern blot analysis and may have a role in malignant progression of CCS. The availability of this MP-CCS-SY cell line will help to understand the molecular biology of this malignancy and should be useful as a tool for developing an immunotherapy.
透明细胞肉瘤(CCS),即软组织恶性黑色素瘤,是一种预后较差的罕见恶性肿瘤。在本研究中,从一名17岁日本女孩起源于左跟腱的转移性肿瘤中建立了一种CCS细胞系,命名为MP-CCS-SY。通过电子显微镜在细胞质中检测到少量黑素小体。这些黑素小体与两种黑色素瘤相关抗体HMB45和Melan-A发生免疫反应。蛋白质免疫印迹法证明该细胞系中存在Melan-A抗原。尽管用传统显带技术进行染色体分析未鉴定出CCS特有的t(12;22)(q13;q12),但用染色体12和22的涂染探针进行荧光原位杂交分析显示染色体12的一个片段插入到了染色体22的一条长臂中。通过逆转录聚合酶链反应检测到了嵌合的EWS/ATF1转录本。观察到染色体8有额外拷贝和结构异常。通过Northern印迹分析检测到c-myc mRNA过表达,其可能在CCS的恶性进展中起作用。MP-CCS-SY细胞系的可用性将有助于了解这种恶性肿瘤的分子生物学,并且作为开发免疫疗法工具应该是有用的。