A mullerian carcinosarcoma or malignant mesodermal mixed tumor (MMMT) origi
nated from the retroperitoneum of a 51-year-old woman; only two previous si
milar cases have been reported. The 1,040 gm tumor was found in the left re
troperitoneal space; the center of the tumor was extensively liquified. The
uterus, fallopian tubes, and ovaries were unremarkable. The histopathologi
cal features of the tumor were typical of MMMT, homologous type. The adenoc
arcinomatous component of the tumor was immunohistochemically positive for
cytokeratin 7 and negative for cytokeratin 20. MMMTs arising in extragenita
l sites are rare, and most of them arise from the peritoneum. The histogene
sis of extragenital MMMTs remains speculative, but the origin from the "sec
ondary mullerian system" is most likely.