Rapid Response of a BRCA2/TP53/PTEN-Deleted Metastatic Uterine Leiomyosarcoma to Olaparib: A Case Report

Perm J. 2021 May;25:20.251. doi: 10.7812/TPP/20.251.

Abstract

Patients with metastatic uterine leiomyosarcoma (uLMS) have poor prognosis due to limited treatment options, especially when disease progresses on doxorubicin and gemcitabine-docetaxel regimens. Here we report a patient whose metastatic uLMS contains a BRCA2 deep deletion as well as TP53 and PTEN deep deletion. The patient responded rapidly to olaparib, a poly (ADP-ribose) polymerase inhibitor, after progressing on gemcitabine-docetaxel, doxorubicin, and temozolomide regimens. This case report shall be helpful to the treatment of other patients with metastatic uLMS that harbors a BRCA2 mutation or deletion.

Publication types

  • Case Reports

MeSH terms

  • BRCA2 Protein*
  • Female
  • Humans
  • Leiomyosarcoma* / drug therapy
  • Leiomyosarcoma* / genetics
  • PTEN Phosphohydrolase* / genetics
  • Phthalazines / therapeutic use
  • Piperazines / therapeutic use
  • Tumor Suppressor Protein p53*
  • Uterine Neoplasms* / drug therapy
  • Uterine Neoplasms* / genetics

Substances

  • BRCA2 Protein
  • BRCA2 protein, human
  • Phthalazines
  • Piperazines
  • TP53 protein, human
  • Tumor Suppressor Protein p53
  • PTEN Phosphohydrolase
  • PTEN protein, human
  • olaparib