Targeted Therapy with Sirolimus and Nivolumab in a Child with Refractory Multifocal Anaplastic Ependymoma

Pediatric ependymoma (EPN) is the third most common central nervous system (CNS) tumor, with 90% arising intracranially. Management typically involves maximal surgical resection and radiotherapy, but patients’ outcome is poor. Moreover, there are only a few therapeutical options available for recurrent or refractory disease. In this report, we present the case of a 7-year-old girl with relapsed refractory multifocal grade III EPN who failed conventional treatments and experienced a stable and durable response to the immune checkpoint inhibitor (ICPI) nivolumab in association with the mammalian target of rapamycin (m-TOR) inhibitor sirolimus. This experimental therapy was targeted on immune phenotypical analyses of the patient’s last relapse tumor sample, and this procedure should be routinely done to find new possible therapeutical approaches in recurrent solid tumors.

Systemic Management of Relapse Wilms Tumor After Radical Nephrectomy in An Adult Female, A Case Report

ABSTRACTWilms tumor (nephroblastoma) is rare in adults. We present a case of 28-year old female with Wilms tumor diagnosed with pre-operative abdominal magnetic resonance imaging, histopathological analysis and immunohistochemistry. She had relapse tumor two years after primary open radical nephrectomy. She was managed with chemotherapy with the ICE regimen (ifosfamide, carboplatin, and etoposide) for six cycles with partial response and excellent functional status. Keywords: ABSTRAKTumor Wilms (nefroblastoma) adalah kasus yang jarang ditemukan pada orang dewasa. Kami melaporkan satu kasus, pasien perempuan, 28 tahun dengan tumor Wilms yang didiagnosis dengan pemeriksaan magnetic resonance imaging pre-operasi, histopatologi, dan imunohistokimia. Pasien tersebut mengalami relaps dua tahun pasca-radikal nefrektomi. Pasien kemudian menjalani kemoterapi dengan regimen ICE (ifosfamid, karboplatin, dan etoposid) selama enam siklus dengan repons parsial dan status fungsional baik.