Abstract
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.
Publication types
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Case Reports
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Research Support, Non-U.S. Gov't
MeSH terms
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Achilles Tendon / pathology
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Adolescent
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Antigens, Neoplasm
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Chromosomes, Human, Pair 12 / genetics
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Chromosomes, Human, Pair 12 / ultrastructure
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Chromosomes, Human, Pair 22 / genetics
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Chromosomes, Human, Pair 22 / ultrastructure
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Chromosomes, Human, Pair 8 / genetics
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Chromosomes, Human, Pair 8 / ultrastructure
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Disease Progression
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Fatal Outcome
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Female
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Gene Expression Regulation, Neoplastic
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Genes, myc*
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Humans
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In Situ Hybridization, Fluorescence
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MART-1 Antigen
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Melanosomes / chemistry
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Melanosomes / ultrastructure
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Mutagenesis, Insertional
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Neoplasm Proteins / biosynthesis*
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Neoplasm Proteins / genetics
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Oncogene Proteins, Fusion / genetics
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Proto-Oncogene Proteins c-myc / biosynthesis*
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RNA, Messenger / biosynthesis
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RNA, Messenger / genetics
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RNA, Neoplasm / biosynthesis
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RNA, Neoplasm / genetics
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Reverse Transcriptase Polymerase Chain Reaction
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Sarcoma, Clear Cell / genetics
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Sarcoma, Clear Cell / metabolism
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Sarcoma, Clear Cell / pathology*
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Soft Tissue Neoplasms / genetics
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Soft Tissue Neoplasms / metabolism
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Soft Tissue Neoplasms / pathology*
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Transcription Factors
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Tumor Cells, Cultured* / metabolism
Substances
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Antigens, Neoplasm
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EWS-ATF1 fusion protein, human
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MART-1 Antigen
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MLANA protein, human
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Neoplasm Proteins
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Oncogene Proteins, Fusion
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Proto-Oncogene Proteins c-myc
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RNA, Messenger
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RNA, Neoplasm
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Transcription Factors