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Clinical outcome of pediatric choroid plexus tumors: retrospective analysis from a single institute

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Abstracts

Background

Choroid plexus tumor is a rare brain tumor with variable clinical features according to the histological grade. We reviewed the treatment outcome of 23 children, focusing on the biological behavior of the atypical choroid plexus papilloma (ACPP) and the current therapeutic strategy in choroid plexus carcinoma (CPC).

Methods

The demographics, clinical features, surgical treatments, adjuvant therapies, and survival were reviewed.

Results

The median age at diagnosis was 18 months—55 months for choroid plexus papilloma (CPP), 8 months for ACPP, and 15 months for CPC. Gross total resections were achieved in seven of eight patients with CPP, seven of seven with ACPP, and three of eight with CPC. Seven patients with CPC received chemotherapy. Four patients received high-dose chemotherapy (HDCT) and autologous peripheral blood stem cell transplantation (aPBSCT), and three among them have survived. Four patients with CPC received radiotherapy. One CPP patient and one CPC patient underwent radiosurgery. All CPP and ACPP patients have survived. The overall survival rate of the CPC patients was 62.5 % in the first year and 42.9 % in the second year. The progression-free survival rate of the CPC patients was 50 % in the first year and 0 % in the second year. Seven patients underwent permanent cerebrospinal fluid diversion surgery because of hydrocephalus or subdural effusion.

Conclusion

CPP and ACPP were surgically curable. Multi-modal treatments are necessary in the management of CPC with poor prognosis. HDCT and aPBSCT may be important to treat infants for whom radiotherapy is limited. Hydrocephalus and subdural effusion should be resolved with appropriate management.

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Acknowledgment

This study was supported by a grant from the Korea Health Technology R&D Project, Ministry of Health & Welfare, Republic of Korea (A120446).

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Authors and Affiliations

  1. Division of Pediatric Neurosurgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul, 110-744, Republic of Korea

    Eun Jung Koh, Kyu-Chang Wang, Ji Hoon Phi, Ji Yeoun Lee, Jung Won Choi & Seung-Ki Kim

  2. Adolescent Cancer Center, Seoul National University Cancer Hospital, Seoul, Republic of Korea

    Ji Hoon Phi, Ji Yeoun Lee, Jung Won Choi & Seung-Ki Kim

  3. Department of Pathology, Seoul National University Hospital, Seoul, Republic of Korea

    Sung-Hye Park

  4. Department of Pediatrics, Seoul National University Children’s Hospital, Seoul, Republic of Korea

    Kyung Duk Park

  5. Department of Radiation Oncology, Seoul National University Hospital, Seoul, Republic of Korea

    Il Han Kim

  6. The Armed Forces Capital Hospital, Seongnam, Gyeonggi-do, Republic of Korea

    Byung-Kyu Cho

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  1. Eun Jung Koh
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Correspondence to Seung-Ki Kim.

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Koh, E.J., Wang, KC., Phi, J.H. et al. Clinical outcome of pediatric choroid plexus tumors: retrospective analysis from a single institute. Childs Nerv Syst 30, 217–225 (2014). https://doi.org/10.1007/s00381-013-2223-4

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  • DOI: https://doi.org/10.1007/s00381-013-2223-4

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