A long-term survivor with late-onset-repeated pulmonary metastasis of a PEComa.

A 59-year-old male underwent a surgical resection of a retroperitoneum tumor in 1990 that was diagnosed as leiomyoblastoma. Thereafter he demonstrated pulmonary metastases followed by a total of 3 pulmonary resections from 1995 to 1997. In 2008, he was incidentally found to have a tumor in the left lower lobe, which was diagnosed as a metastatic perivascular epithelioid cell (PEComa) neoplasm. Retrospectively, a primary tumor and pulmonary metastases were also diagnosed as PEComa. We experienced late-onset repeated pulmonary metastasis of a PEComa.