BACKGROUND: Alveolar soft-part sarcoma is a rare tumor of uncertain histogenesis. METHODS: The authors report a patient who was studied using immunohistochemistry, cytogenetic analysis, and molecular probes for MyoD1 and MYCN (N-myc proto-oncogene). RESULTS: By immunoperoxidase, the tumor was focally positive for vimentin, neuron-specific enolase, and S-100 protein but negative for muscle-specific actin, desmin, and low-molecular-weight keratin. Direct chromosome analysis of primary tumor cells using G-banded preparations yielded two clonally abnormal lines: one demonstrated trisomy 47,XX+5; the other demonstrated 46,XX,1p-,17q+. Expression of the MYCN RNA was detectable at a low level, and MYCN was single copy at the DNA level. Expression of the myogenic molecular marker MyoD1 was not detected by Northern blotting analysis. CONCLUSIONS: This is the first detailed study to address the molecular biology and tumor cytogenetics of alveolar soft-part sarcoma. The results of this study indicate a neurogenic origin for this unusual tumor and fail to provide support for the notion of a myogenic origin.
BACKGROUND:Alveolar soft-part sarcoma is a rare tumor of uncertain histogenesis. METHODS: The authors report a patient who was studied using immunohistochemistry, cytogenetic analysis, and molecular probes for MyoD1 and MYCN (N-myc proto-oncogene). RESULTS: By immunoperoxidase, the tumor was focally positive for vimentin, neuron-specific enolase, and S-100 protein but negative for muscle-specific actin, desmin, and low-molecular-weight keratin. Direct chromosome analysis of primary tumor cells using G-banded preparations yielded two clonally abnormal lines: one demonstrated trisomy 47,XX+5; the other demonstrated 46,XX,1p-,17q+. Expression of the MYCN RNA was detectable at a low level, and MYCN was single copy at the DNA level. Expression of the myogenic molecular marker MyoD1 was not detected by Northern blotting analysis. CONCLUSIONS: This is the first detailed study to address the molecular biology and tumor cytogenetics of alveolar soft-part sarcoma. The results of this study indicate a neurogenic origin for this unusual tumor and fail to provide support for the notion of a myogenic origin.
Authors: Brendan C Dickson; Catherine T-S Chung; David J Hurlbut; Paula Marrano; Mary Shago; Yun-Shao Sung; David Swanson; Lei Zhang; Cristina R Antonescu Journal: Genes Chromosomes Cancer Date: 2019-08-21 Impact factor: 5.006
Authors: Luke H Stockwin; David T Vistica; Susan Kenney; David S Schrump; Donna O Butcher; Mark Raffeld; Robert H Shoemaker Journal: BMC Cancer Date: 2009-01-15 Impact factor: 4.430