C. Kuhnen et al., APC and beta-catenin in alveolar soft part sarcoma (ASPS) - Immunohistochemical and molecular genetic analysis, PATH RES PR, 196(5), 2000, pp. 299-304
Apart from its role in cell-adhesion, beta-catenin is regarded as an oncopr
otein, the cytoplasmic level of which is regulated by APC as a turner suppr
essor protein. Changes of chromosome 5q, the region that includes the APC-g
ene, are known to be important in the pathogenesis of fibromatosis; however
, little is known about the significance of APC and beta-catenin ill other
mesenchymal tumors. Therefore, we used immunohistochemistry and DNA-analysi
s to investigate four cases of alveolar soft-part sarcoma (ASPS) as a mesen
chymal tumor with a distinct histologic appearance.
In three cases of ASPS the APC-gene product was found to have strong nuclea
r expression and only faint cytoplasmic staining, beta-catenin showed a par
tly membranous, partly strong intracytoplasmic expression. No gene mutation
s for APC and beta-catenin were detected in any of the four cases.
These investigations suggest that, apart from their function in carcinogene
sis and fibromatoses, APC and beta-catenin play a role in the pathogenesis
of soft tissue tumors such as ASPS, The significance of a striking nuclear
accumulation of non-mutated, virtually functionally active APC-tumor suppre
ssor protein has not yet been investigated. A nuclear function of APC in AS
PS in down-regulating nuclear transcription processes linked to overexpress
ion of beta-catenin, as is known in colorectal carcinogenesis, may be hypot
hesized.