We present the second reported case of a smooth muscle neoplasm involving t
he placental parenchyma. On gross examination, the tumor easily separated f
rom the uterus and had a whorled cut surface with finger-like extensions in
to the villous parenchyma, very similar to the previously described case. T
he differential diagnosis included a primary smooth muscle tumor of the pla
centa (placental leiomyoma), a primary uterine neoplasm incorporated into t
he placenta, and a metastatic sarcoma. In this case, the infant was male, a
nd the polymerase chain reaction technique demonstrated the presence of Y c
hromosome gene in the placental parenchyma and its absence in the placental
neoplasm. Thus, this neoplasm, despite its gross appearance of a primary p
lacental tumor, actually represented an incorporated benign uterine leiomyo
ma.