PEComa (TSC2, TFE3): Molecular Oncology Tumor Boards

This free case-based activity includes a crowd-sourced discussion of a 35 year old female who presented with presented with 10 cm mass in her uterus which was thought to be a leiomyoma. Two years from her initial diagnosis, she recurred in the pelvis and subsequently developed lung nodules one year later. After a pathology review, her original diagnosis was revised to metastatic malignant PEComa.

Originally posted on a forum in the ASCO Connection, users now have the opportunity to claim CME, completion, or participation certificates for completing this activity. 

Learning Objectives:

  • Describe the characteristic histologic and immunophenotypic features of PEComas (perivascular epithelioid cell tumors).
  • Analyze the molecular genetic alterations underlying the pathogenesis of PEComas.
  • Evaluate the rationale for targeted therapy directed against mTOR for metastatic malignant PEComas.

Competencies Addressed

Medical Knowledge (ACGME, AAPA)

Health Promotion, Health Protection, Disease Prevention, and Treatment (ONS)