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Cancer treatment in disabled children

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Abstract

The incidence of cancer in children with intellectual disability has been poorly documented. We report our experience of treating children and adolescents with cancer and intellectual disability (40 patients), from 2004 to 2018. A treatment-sparing approach was adopted for 6 patients with severe intellectual impairment to minimize toxicity: a child with postpartum asphyxia and medulloblastoma did not receive radiotherapy; 1 patient with mitochondrial encephalopathy and a testicular germ cell tumor did not receive bleomycin and lung metastasectomy; 2 patients (1 with Down + West syndrome + Wilms tumor (WT) and 1 with Denys-Drash syndrome + WT) did not receive vincristine; 1 child with corpus callosum agenesis and anaplastic ependymoma did not receive chemotherapy; 1 child with structural chromosomal aberrations and a primitive neuro-ectodermal tumor received personalized chemotherapy. Heminephrectomy was performed in 4 patients with WT to preserve their kidney function. We found no statistically significant correlation between relapse or mortality rates and the use of a treatment-sparing approach. The 5-year overall survival (OS) and event-free survival (EFS) rates were 84.5% and 66.1% as opposed to 82.5% and 46.9%, respectively, for patients in our usual-treatment and treatment-sparing groups.

Conclusion: We only opted for a treatment-sparing approach for patients with severe disabilities, and their OS was in line with that of children without intellectual disability.

What is Known:

• There are few reports on children/adolescents with cancer and intellectual disability (ID).

• It is not clear how to manage them and whether a treatment sparing should be considered, especially in the case of severe disability.

What is New:

• Most patients received the standard cancer treatment and only in the case of severe disability, a therapeutic saving approach was applied.

• No statistically significant correlations between relapse/mortality rates and the use of a treatment-sparing approach were found.

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Abbreviations

ASRE:

European age-standardized rate

EFS:

Event-free survival

ID:

Intellectual disability

OS:

Overall survival

PFS:

Progression-free survival

WAGR:

Wilms tumor, aniridia, genitourinary anomalies, and mental retardation

WT:

Wilms tumor

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Authors and Affiliations

  1. Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Via G. Venezian, 1, 20133, Milan, MI, Italy

    Cristina Meazza, Elisabetta Schiavello, Veronica Biassoni, Marta Podda, Giovanna Gattuso, Monica Terenziani, Andrea Ferrari, Filippo Spreafico, Roberto Luksch, Michela Casanova, Stefano Chiaravalli, Nadia Puma, Luca Bergamaschi & Maura Massimino

  2. General Medicine I, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy

    Chiara Barteselli

  3. Clinical Epidemiology and Trial Organization, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy

    Francesco Barretta

Authors
  1. Cristina Meazza
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  2. Elisabetta Schiavello
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  3. Veronica Biassoni
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  4. Marta Podda
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  5. Chiara Barteselli
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  6. Francesco Barretta
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  7. Giovanna Gattuso
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  8. Monica Terenziani
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  9. Andrea Ferrari
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  11. Roberto Luksch
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  12. Michela Casanova
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  13. Stefano Chiaravalli
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  14. Nadia Puma
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  15. Luca Bergamaschi
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  16. Maura Massimino
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Contributions

All authors contributed to the study conception and design. Data collection was performed by Chiara Barteselli and Cristina Meazza; methodology by Cristina Meazza; formal analysis by Francesco Barretta; writing - original draft preparation by Cristina Meazza, Elisabetta Schiavello, Veronica Biassoni, Marta Podda, Giovanna Gattuso; writing - review and editing by Giovanna gattuso, Monica Terenziani, Andrea Ferrari, Filippo Spreafico, Roberto Luksch, Michela Casanova, Stefano Chiaravalli, Nadia Puma, Luca Bergamaschi; supervision by Maura Massimino. All authors read and approved the final manuscript.

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Correspondence to Cristina Meazza.

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The authors declare that they have no conflict of interest.

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This article does not contain any studies with human participants or animals performed by any of the authors.

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Communicated by Peter de Winter

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Meazza, C., Schiavello, E., Biassoni, V. et al. Cancer treatment in disabled children. Eur J Pediatr 179, 1353–1360 (2020). https://doi.org/10.1007/s00431-020-03607-6

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  • DOI: https://doi.org/10.1007/s00431-020-03607-6

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