Rare Tumors (Jan 2023)

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
https://doi.org/10.1177/20363613231152333
Journal volume & issue
Vol. 15

Abstract

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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.