Management strategies for recurrent pediatric craniopharyngioma: new recommendations

View More View Less
  • 1 Department of Neurosurgery, Boston Children’s Hospital, Boston, Massachusetts;
  • 2 Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;
  • 3 Division of Biostatistics, Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children’s Hospital;
  • 4 Division of Radiation Oncology, Boston Children’s Hospital; and
  • 5 Dana Farber/Boston Children’s Cancer and Blood Disorders Center, Boston, Massachusetts
Restricted access

Purchase Now

USD  $45.00

JNS + Pediatrics - 1 year subscription bundle (Individuals Only)

USD  $505.00

JNS + Pediatrics + Spine - 1 year subscription bundle (Individuals Only)

USD  $600.00
Print or Print + Online

OBJECTIVE

The goal of this study was to identify the independent risk factors for recurrence or progression of pediatric craniopharyngioma and to establish predictors of the appropriate timing of intervention and best management strategy in the setting of recurrence/progression, with the aim of optimizing tumor control.

METHODS

This is a retrospective cohort study of all pediatric patients with craniopharyngioma who were diagnosed and treated at Boston Children’s Hospital between 1990 and 2017. This study was approved by the institutional review board at Boston Children’s Hospital. All statistical analyses were performed using Stata software.

RESULTS

Eighty patients (43 males and 37 females) fulfilled the inclusion criteria. The mean age at the time of diagnosis was 8.6 ± 4.4 years (range 1.2–19.7 years). The mean follow-up was 10.9 ± 6.5 years (range 1.3–24.6 years). Overall, 30/80 (37.5%) patients developed recurrence/progression. The median latency to recurrence/progression was 12.75 months (range 3–108 months). Subtotal resection with no adjuvant radiotherapy (p < 0.001) and fine calcifications (p = 0.008) are independent risk factors for recurrence/progression. An increase (%) in the maximum dimension of the tumor at the time of recurrence/progression was considered a statistically significant predictor of the appropriate timing of intervention.

CONCLUSIONS

Based on the identified independent risk factors for tumor recurrence/progression and the predictors of appropriate timing of intervention in the setting of recurrence/progression, the authors propose an algorithm for optimal management of recurrent pediatric craniopharyngioma to increase the likelihood of tumor control.

ABBREVIATIONS AUC = area under the curve; BCH = Boston Children’s Hospital; GTR = gross-total resection; NPV = negative predictive value; PPV = positive predictive value; ROC = receiver operating characteristic; STR = subtotal resection; XRT = radiotherapy.

JNS + Pediatrics - 1 year subscription bundle (Individuals Only)

USD  $505.00

JNS + Pediatrics + Spine - 1 year subscription bundle (Individuals Only)

USD  $600.00

Contributor Notes

Correspondence Mohammed A. Fouda: Johns Hopkins University School of Medicine, Baltimore, MD. mfouda1@jhmi.edu.

INCLUDE WHEN CITING Published online March 5, 2021; DOI: 10.3171/2020.9.PEDS20606.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

  • 1

    Ali ZS , Lang SS , Kamat AR , . Suprasellar pediatric craniopharyngioma resection via endonasal endoscopic approach . Childs Nerv Syst . 2013 ;29 (11 ):2065 2070 .

    • Search Google Scholar
    • Export Citation
  • 2

    Clark AJ , Cage TA , Aranda D , . A systematic review of the results of surgery and radiotherapy on tumor control for pediatric craniopharyngioma . Childs Nerv Syst . 2013 ;29 (2 ):231 238 .

    • Search Google Scholar
    • Export Citation
  • 3

    Cohen M , Guger S , Hamilton J . Long term sequelae of pediatric craniopharyngioma—literature review and 20 years of experience . Front Endocrinol (Lausanne) . 2011 ;2 :81 .

    • Search Google Scholar
    • Export Citation
  • 4

    Elliott RE , Hsieh K , Hochm T , . Efficacy and safety of radical resection of primary and recurrent craniopharyngiomas in 86 children . J Neurosurg Pediatr . 2010 ;5 (1 ):30 48 .

    • Search Google Scholar
    • Export Citation
  • 5

    Fouda MA , Scott RM , Marcus KJ , . Sixty years single institutional experience with pediatric craniopharyngioma: between the past and the future . Childs Nerv Syst . 2020 ;36 (2 ):291 296 .

    • Search Google Scholar
    • Export Citation
  • 6

    Prabhu VC , Brown HG . The pathogenesis of craniopharyngiomas . Childs Nerv Syst . 2005 ;21 (8-9 ):622 627 .

  • 7

    Fouda MA , Riordan CP , Zurakowski D , Goumnerova LC . Analysis of 2141 pediatric craniopharyngioma admissions in the USA utilizing the Kids’ Inpatient Database (KID): predictors of discharge disposition . Childs Nerv Syst . 2020 ;36 (12 ):3007 3012 .

    • Search Google Scholar
    • Export Citation
  • 8

    Gonc EN , Yordam N , Ozon A , . Endocrinological outcome of different treatment options in children with craniopharyngioma: a retrospective analysis of 66 cases . Pediatr Neurosurg . 2004 ;40 (3 ):112 119 .

    • Search Google Scholar
    • Export Citation
  • 9

    Miller DC . Pathology of craniopharyngiomas: clinical import of pathological findings . Pediatr Neurosurg . 1994 ;21 (suppl 1 ):11 -17 .

    • Search Google Scholar
    • Export Citation
  • 10

    Caldarelli M , Massimi L , Tamburrini G , . Long-term results of the surgical treatment of craniopharyngioma: the experience at the Policlinico Gemelli, Catholic University, Rome . Childs Nerv Syst . 2005 ;21 (8-9 ):747 757 .

    • Search Google Scholar
    • Export Citation
  • 11

    Hoffman HJ , De Silva M , Humphreys RP , . Aggressive surgical management of craniopharyngiomas in children . J Neurosurg . 1992 ;76 (1 ):47 52 .

    • Search Google Scholar
    • Export Citation
  • 12

    Merchant TE , Kiehna EN , Sanford RA , . Craniopharyngioma: the St. Jude Children’s Research Hospital experience 1984-2001 . Int J Radiat Oncol Biol Phys . 2002 ;53 (3 ):533 542 .

    • Search Google Scholar
    • Export Citation
  • 13

    Muller HL . Childhood craniopharyngioma. Recent advances in diagnosis, treatment and follow-up . Horm Res . 2008 ;69 (4 ):193 202 .

    • Search Google Scholar
    • Export Citation
  • 14

    Ahmet A , Blaser S , Stephens D , . Weight gain in craniopharyngioma—a model for hypothalamic obesity . J Pediatr Endocrinol Metab . 2006 ;19 (2 ):121 127 .

    • Search Google Scholar
    • Export Citation
  • 15

    Elliott RE , Jane JA Jr , Wisoff JH . Surgical management of craniopharyngiomas in children: meta-analysis and comparison of transcranial and transsphenoidal approaches . Neurosurgery . 2011 ;69 (3 ):630 643 .

    • Search Google Scholar
    • Export Citation
  • 16

    Poretti A , Grotzer MA , Ribi K , . Outcome of craniopharyngioma in children: long-term complications and quality of life . Dev Med Child Neurol . 2004 ;46 (4 ):220 229 .

    • Search Google Scholar
    • Export Citation
  • 17

    Steňo J , Bízik I , Steňo A , Matejčík V . Craniopharyngiomas in children: how radical should the surgeon be? . Childs Nerv Syst . 2011 ;27 (1 ):41 54 .

    • Search Google Scholar
    • Export Citation
  • 18

    Zacharia BE , Amine M , Anand V , Schwartz TH . Endoscopic endonasal management of craniopharyngioma . Otolaryngol Clin North Am . 2016 ;49 (1 ):201 212 .

    • Search Google Scholar
    • Export Citation
  • 19

    De Vile CJ , Grant DB , Kendall BE , . Management of childhood craniopharyngioma: can the morbidity of radical surgery be predicted? . J Neurosurg . 1996 ;85 (1 ):73 81 .

    • Search Google Scholar
    • Export Citation
  • 20

    van Iersel L , Brokke KE , Adan RAH , . Pathophysiology and individualized treatment of hypothalamic obesity following craniopharyngioma and other suprasellar tumors: a systematic review . Endocr Rev . 2019 ;40 (1 ):193 235 .

    • Search Google Scholar
    • Export Citation
  • 21

    Visser J , Hukin J , Sargent M , . Late mortality in pediatric patients with craniopharyngioma . J Neurooncol . 2010 ;100 (1 ):105 111 .

    • Search Google Scholar
    • Export Citation
  • 22

    Cohen M , Bartels U , Branson H , . Trends in treatment and outcomes of pediatric craniopharyngioma, 1975-2011 . Neuro Oncol . 2013 ;15 (6 ):767 774 .

    • Search Google Scholar
    • Export Citation
  • 23

    Kiehna EN , Merchant TE . Radiation therapy for pediatric craniopharyngioma . Neurosurg Focus . 2010 ;28 (4 ):E10 .

  • 24

    Puget S , Garnett M , Wray A , . Pediatric craniopharyngiomas: classification and treatment according to the degree of hypothalamic involvement . J Neurosurg . 2007 ;106 (1)(suppl) :3 12 .

    • Search Google Scholar
    • Export Citation
  • 25

    Zhou X-H , McClish DK , Obuchowski NA . Statistical Methods in Diagnostic Medicine . Vol 569 . John Wiley & Sons ; 2009 .

  • 26

    Hosmer DW Jr , Lemeshow S , May S . Applied Survival Analysis: Regression Modeling of Time-to-Event Data . Vol 618 . John Wiley & Sons ; 2011 .

    • Search Google Scholar
    • Export Citation
  • 27

    Vittinghoff E , Glidden DV , Shiboski SC , McCulloch CE . Regression Methods in Biostatistics: Linear, Logistic, Survival, and Repeated Measures Models . Springer Science & Business Media ; 2011 .

    • Search Google Scholar
    • Export Citation
  • 28

    Carmel PW , Antunes JL , Chang CH . Craniopharyngiomas in children . Neurosurgery . 1982 ;11 (3 ):382 389 .

  • 29

    Lafferty AR , Chrousos GP . Pituitary tumors in children and adolescents . J Clin Endocrinol Metab . 1999 ;84 (12 ):4317 4323 .

    • Search Google Scholar
    • Export Citation
  • 30

    Müller HL . Childhood craniopharyngioma—current concepts in diagnosis, therapy and follow-up . Nat Rev Endocrinol . 2010 ;6 (11 ):609 618 .

    • Search Google Scholar
    • Export Citation
  • 31

    Paja M , Lucas T , García-Uría J , . Hypothalamic-pituitary dysfunction in patients with craniopharyngioma . Clin Endocrinol (Oxf) . 1995 ;42 (5 ):467 473 .

    • Search Google Scholar
    • Export Citation
  • 32

    Sughrue ME , Yang I , Kane AJ , . Endocrinologic, neurologic, and visual morbidity after treatment for craniopharyngioma . J Neurooncol . 2011 ;101 (3 ):463 476 .

    • Search Google Scholar
    • Export Citation
  • 33

    Thomsett MJ , Conte FA , Kaplan SL , Grumbach MM . Endocrine and neurologic outcome in childhood craniopharyngioma: review of effect of treatment in 42 patients . J Pediatr . 1980 ;97 (5 ):728 735 .

    • Search Google Scholar
    • Export Citation

Metrics

All Time Past Year Past 30 Days
Abstract Views 17 17 17
Full Text Views 8 8 8
PDF Downloads 2 2 2
EPUB Downloads 0 0 0