The presence of activating ras mutations in a cardiac leiomyosarcoma w
hich occurred in the right atrium of the heart of a female patient was
examined. The tumor had the appearance of leiomyosarcoma in rutine hi
stopathological examination and the definite diagnosis was confirmed b
y a positive immunohistochemical reaction to smooth muscle actin. Mole
cular analysis by polymerase chain reaction (PCR) restriction fragment
length polymorphism (RFLP) technique showed a point mutation of H-ras
gene at codon 12. To the best of our knowledge, this is the first rep
ort describing ras gene mutation in a cardiac leiomyosarcoma implying
a role for the ras oncogenes in the development of this tumor.