PARP inhibition utilized in combination therapy with Olaparib-Temozolomide to achieve disease stabilization in a rare case of BRCA1-mutant, metastatic myxopapillary ependymoma

Preethika Mahalingam; Sam Smith; Juanita Lopez; Rajaei K Sharma; Thomas Millard; Khin Thway; Cyril Fisher; David A Reardon; Robin Jones; Andrew G Nicholson; David Cunningham; Liam Welsh; Bhupinder Sharma

doi:10.1177/20363613231152333

PARP inhibition utilized in combination therapy with Olaparib-Temozolomide to achieve disease stabilization in a rare case of BRCA1-mutant, metastatic myxopapillary ependymoma

Rare Tumors. 2023 Jan 19:15:20363613231152333. doi: 10.1177/20363613231152333. eCollection 2023.

Authors

Preethika Mahalingam¹, Sam Smith¹, Juanita Lopez^{1

2}, Rajaei K Sharma³, Thomas Millard¹, Khin Thway^{1

2}, Cyril Fisher^{1

2}, David A Reardon⁴, Robin Jones^{1

2}, Andrew G Nicholson^{2

5

6}, David Cunningham^{1

2}, Liam Welsh¹, Bhupinder Sharma^{1

2}

Affiliations

¹ The Royal Marsden Hospital NHS Foundation Trust, London, UK.
² Institute of Cancer Research, London and Sutton, UK.
³ College of Medicine and Health, University of Exeter, Exeter, UK.
⁴ Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
⁵ Department of Histopathology, Royal Brompton & Harefield NHS Foundation Trust, London, UK.
⁶ National Heart and Lung Division, Imperial College, London, UK.

Abstract

Myxopapillary ependymoma (MPE) is a primary tumor of the central nervous system (CNS), characteristically an indolent malignancy involving the spinal conus medullaris, Filum terminale or cauda equina. We present a rare case of MPE, recurrent in the pelvic soft tissue with eventual pleural and intra-pulmonary metastasis. Refractory to repeated gross resection, adjuvant radiotherapy, platinum-based chemotherapy and temozolomide exploitation of mutant somatic BRCA1 status with the addition of a poly (ADP-ribose); polymerase inhibitor (PARPi) in a novel combination regimen with olaparib-temozolomide (OT) has achieved stable radiological disease after 10 cycles.

Keywords: Oncology; PARP inhibitor; ependymoma.

Publication types

Case Reports