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

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-...

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Published inRare tumors Vol. 15; p. 20363613231152333
Main Authors Mahalingam, Preethika, Smith, Sam, Lopez, Juanita, Sharma, Rajaei K, Millard, Thomas, Thway, Khin, Fisher, Cyril, Reardon, David A, Jones, Robin, Nicholson, Andrew G, Cunningham, David, Welsh, Liam, Sharma, Bhupinder
Format Journal Article
LanguageEnglish
Published London, England SAGE Publications 01.01.2023
Sage Publications Ltd
SAGE Publishing
Subjects
BRCA1 protein
Case Report
Central nervous system
Chemotherapy
Combination therapy
Malignancy
Metastases
Mutants
Radiation therapy
Spinal cord
Temozolomide
ependymoma
Oncology
PARP inhibitor
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Summary: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.
ISSN:2036-3613
2036-3605
2036-3613
DOI:10.1177/20363613231152333