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Outcome of Chinese children with craniopharyngioma: a 20-year population-based study by the Hong Kong Pediatric Hematology/Oncology Study Group

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Abstract

Purpose

Craniopharyngioma is a rare low-grade neoplasm in children. Tumor progression occurs frequently, and survivors are at risk of long-term disease and treatment-related morbidities. We reviewed the population-based experience of managing pediatric craniopharyngioma in Hong Kong.

Methods

The Hong Kong Pediatric Hematology/Oncology Study Group database was interrogated for patients with craniopharyngioma younger than 18 years between 1999 and 2018. Patient demographics, clinical characteristics, outcomes, and long-term morbidities were summarized.

Results

Twenty-eight patients with craniopharyngioma were included (approximate incidence of 1.1 per 1,000,000 individuals). The treatment approaches were heterogeneous and included surgery only, surgery with adjuvant radiation, and surgery with intracystic interferon. With a median follow-up of 6.1 years, 12 (43%) patients experienced disease progression, and 3 patients died of progression, postoperative complication, and gastrointestinal bleeding. The 5-year progression-free survival (PFS) and overall survival (OS) rates were 56.8% (± 10.0%) and 92.0% (± 5.4%), respectively. The 10-year PFS and OS rates were 37.3% (± 11.4) and 92.0% (± 5.4%), respectively. Patients receiving treatment in a high-volume center had significantly better outcomes than did those treated at other centers (PFS, p = 0.007; OS, p = 0.029). Period of diagnosis, sex, age at diagnosis, greatest tumor dimension, extent of resection, and radiotherapy use did not significantly affect patient survival. Long-term visual impairment (60%) and endocrinopathies (92%) were common.

Conclusion

Prognosis of pediatric craniopharyngioma in Hong Kong compares unfavorably with published reports. Centralization and standardization of treatment may prove valuable in mitigating patient outcomes.

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Acknowledgments

The authors thank the medical and nursing teams from Prince of Wales Hospital, Princess Margaret Hospital, Queen Elizabeth Hospital, Queen Mary Hospital, and Tuen Mun Hospital for providing exceptional patient care. We also express our gratitude to Joyce Le and Heidi Wong, data officers of the Hong Kong Pediatric Hematology/Oncology Study Group funded by the Children’s Cancer Foundation, for managing the study group database and Otto Tam for assistance with the submission. We acknowledge Nisha Badders, PhD, ELS, of St. Jude Children’s Research Hospital, for editing the manuscript.

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Authors and Affiliations

  1. Department of Paediatrics & Adolescent Medicine, Queen Mary Hospital, University of Hong Kong, Room 117, 1/F, New Clinical Building, 102 Pokfulam Road, Pok Fu Lam, Hong Kong

    Anthony Pak-Yin Liu, Joanna Yuet-Ling Tung & Godfrey Chi-Fung Chan

  2. Department of Paediatrics & Adolescent Medicine, Hong Kong Children’s Hospital, Ngau Tau Kok, Hong Kong

    Joanna Yuet-Ling Tung, Dennis Tak-Loi Ku, Chung-Wing Luk, Matthew Ming-Kong Shing & Godfrey Chi-Fung Chan

  3. Department of Paediatrics & Adolescent Medicine, Princess Margaret Hospital, Toronto, ON, Canada

    Alvin Siu-Cheung Ling

  4. Department of Clinical Oncology, Queen Mary Hospital, University of Hong Kong, Pok Fu Lam, Hong Kong

    Dora Lai-Wan Kwong

  5. Division of Neurosurgery, Department of Surgery, Queen Mary Hospital, Pok Fu Lam, Hong Kong

    Kevin King-Fai Cheng & Wilson Wai-Shing Ho

  6. Division of Neurosurgery, Department of Surgery, Hong Kong Children’s Hospital, Ngau Tau Kok, Hong Kong

    Kevin King-Fai Cheng & Wilson Wai-Shing Ho

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  1. Anthony Pak-Yin Liu
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  10. Godfrey Chi-Fung Chan
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Correspondence to Godfrey Chi-Fung Chan.

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This study was approved by the University of Hong Kong/Hong Kong West Cluster Institutional Review Board, with the requirement for informed consent waived.

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The authors declare no conflicts of interest.

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Liu, A.PY., Tung, J.YL., Ku, D.TL. et al. Outcome of Chinese children with craniopharyngioma: a 20-year population-based study by the Hong Kong Pediatric Hematology/Oncology Study Group. Childs Nerv Syst 36, 497–505 (2020). https://doi.org/10.1007/s00381-019-04480-x

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