Extremely late-onset pulmonary metastasis from uterine PEComa

Area of Science:

• Oncology
• Pathology

Background:

• Perivascular Epithelioid Cell Tumors (PEComas) are rare mesenchymal neoplasms.
• PEComas are characterized by a propensity for late metastasis.

Observation:

• A 79-year-old woman presented with a solitary pulmonary lesion.
• The lesion was diagnosed as a malignant PEComa.
• The patient had a history of uterine leiomyosarcoma excised 20 years prior.

Findings:

• Re-evaluation confirmed the pulmonary lesion as a lung metastasis from the primary uterine PEComa.
• The 20-year interval between the primary tumor and metastasis is the longest documented for PEComas.

Implications:

• This case highlights the potential for exceptionally long latency in PEComa metastasis.
• It underscores the importance of long-term surveillance for patients with a history of PEComa.
• Further research into PEComa biology may elucidate mechanisms driving such prolonged metastatic potential.