A 31-year-old woman presented with multiple pulmonary leiomyomatous hamarto
ma (MPLH) with secondary ossification. She had a past history of parosteal
osteosarcoma. The pulmonary lesions were composed of spindle-shaped cells a
rranged in interlacing fascicles, among which glands or duct-like spaces we
re scattered. As some lesions contained bony tissues, it was unclear whethe
r or not the pulmonary lesions were metastases of parosteal osteosarcoma. H
owever, the majority of spindle-shaped cells were positive for a-smooth mus
cle actin, including cells proliferating around the bony tissues. Clonality
analysis using a target of human androgen receptor (HUMARA) gene disclosed
that the pulmonary nodules were polyclonal, These findings do not indicate
that the lesions were metastatic. We would like to emphasize that MPLH can
show osseous metaplasia.