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Long-term outcomes following maximal safe resection in a contemporary series of childhood craniopharyngiomas

  • Original Article - Pediatric Neurosurgery
  • Published:
Acta Neurochirurgica Aims and scope Submit manuscript

Abstract

Background

The optimal management of pediatric craniopharyngiomas remains controversial. This study aimed to characterize long-term outcomes in a contemporary cohort of children undergoing surgery for craniopharyngiomas.

Methods

This was a retrospective review of 37 consecutive children who underwent surgery for craniopharyngioma with a median follow-up duration of 79 months (range 5–127 months). Patients were stratified by extent of resection (EOR) and need for adjuvant radiation therapy (RT). Imaging studies were reviewed to grade extent of hypothalamic involvement. Data on functional outcomes, pituitary function, and obesity were analyzed.

Results

Gross total resection was achieved in 16 patients (43.2%), near total resection in six patients (16.2%), and subtotal resection (STR) in 15 patients (40.5%). The recurrence-free survival rate was 81.1% and 70.3% at 5- and 10-year follow-up, respectively. Survival analysis showed superior disease control in patients undergoing STR + RT (p = 0.008). Functional outcomes were independent of EOR, postoperative RT or recurrence. Diabetes insipidus was present in 75% and 44.4% of patients required >2 hormone replacements at last follow-up. Obesity was present in 36.1% patients after treatment, and was associated with preoperative obesity (p = 0.019), preoperative hypothalamic involvement (p = 0.047) and STR + RT (p = 0.011).

Conclusions

Gross or near total resection may be achieved safely in almost 60% of cases; however, radical surgery does not eliminate the risk of recurrence. Over long-term follow-up, STR + RT offers the best disease control rates. Patients with preoperative hypothalamic involvement, obesity, and those with tumors not amenable to radical resection are at risk for developing obesity on long-term follow-up.

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Abbreviations

CI:

Confidence interval

CT:

Computed tomography

EOR:

Extent of resection

GTR:

Gross total resection

MRI:

Magnetic resonance imaging

NTR:

Near total resection

OR:

Odds ratio

RT:

Radiation therapy

STR:

Subtotal resection

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Authors and Affiliations

  1. Department of Neurological Sciences, Christian Medical College, Vellore, 632004, India

    Sauradeep Sarkar & Ari George Chacko

  2. Department of Internal Medicine, Einstein Medical Center, Philadelphia, PA, USA

    Shireen R. Chacko

  3. Department of Pediatric Endocrinology, Christian Medical College, Vellore, India

    Sophy Korula, Anna Simon & Sarah Mathai

  4. Department of Pathology, Christian Medical College, Vellore, India

    Geeta Chacko

Authors
  1. Sauradeep Sarkar
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  2. Shireen R. Chacko
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  3. Sophy Korula
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  4. Anna Simon
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  5. Sarah Mathai
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  6. Geeta Chacko
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  7. Ari George Chacko
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Contributions

All authors contributed to the study conception and design. Material preparation, data collection, and analysis were performed by Sauradeep Sarkar and Shireen Chacko. The first draft of the manuscript was written by Sauradeep Sarkar, and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Ari George Chacko.

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The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee (name of institute/committee) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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For this type of study formal consent is not required.

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Comments

The authors present a single-surgeon cohort of paediatric craniopharyngioma managed along a single, clear and uniform pathway. They describe 37 patients, with a mean age of ten, followed up for a median of 79 months, including 27 patients for five years and over; none of the patients were lost to follow up, although two died within two years. 16 underwent gross total resection, 6 near total and 15 subtotal resection; almost all surgery was transcranial. Complication rates are within the expected range; 75% had diabetes insipidus on long-term follow up. Recurrence free survival rates were 81% at five years and 70.3% at ten years. The authors make a number of conclusions; most importantly they demonstrate that radical surgery does not eliminate the risk of long-term recurrence, that the combination of subtotal resection and radiotherapy leads to the best disease control rates, and that surgical complications were independent of the extent of resection.This is not new territory, and the problems and controversies related to the holistic management of paediatric craniopharyngioma have been extensively presented in the literature over the past ten years. The value of this study lies in the robust and consistent management paradigm, describing a paediatric population that has been followed up closely.

Kristen Aquilina

London, UK

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Sarkar, S., Chacko, S.R., Korula, S. et al. Long-term outcomes following maximal safe resection in a contemporary series of childhood craniopharyngiomas. Acta Neurochir 163, 499–509 (2021). https://doi.org/10.1007/s00701-020-04591-4

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