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Nimotuzumab-vinorelbine combination therapy versus other regimens in the treatment of pediatric diffuse intrinsic pontine glioma

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Abstract

Purpose

Pediatric diffuse intrinsic pontine glioma (DIPG) is a fatal disease associated with a median survival of < 1 year despite aggressive treatments. This retrospective study analyzed the treatment outcomes of patients aged < 18 years who were diagnosed with DIPG between 2012 and 2022 and who received different chemotherapy regimens.

Methods

After radiotherapy, patients with DIPG received nimotuzumab-vinorelbine combination or temozolomide-containing therapy. When nimotuzumab was unavailable, it was replaced by vincristine, etoposide, and carboplatin/cyclophosphamide (VECC). Temozolomide was administered as a single agent or a part of the combination chemotherapy comprising temozolomide, irinotecan, and bevacizumab. Furthermore, 1- and 3-year overall survival (OS), progression-free survival (PFS), and median OS and PFS were analyzed.

Results

The median age of 40 patients with DIPG was 97 ± 46.93 (23–213) months; the median follow-up time was 12 months. One and 3-year OS were 35.0% and 7.5%, respectively. Median OS was 12 months in all patients (n = 40), and it was 16, 10, and 11 months in those who received first-line nimotuzumab-vinorelbine combination (n = 13), temozolomide-based (n = 14), and VECC (n = 6) chemotherapy regimens, respectively (p = 0.360). One patient who received gefitinib survived for 16 months. Conversely, patients who never received radiotherapy and any antineoplastic medicamentous therapy (n = 6) had a median OS of 4 months.

Conclusion

Nimotuzumab-vinorelbine combination therapy prolonged OS by 6 months compared with temozolomide-containing chemotherapy, although the difference was not statistically significant.

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Data Availability

Authors can provide the data set upon reasonable request.

Abbreviations

COG:

Children’s Oncology Group

CR:

Complete response

CTC-AE:

Common Terminology Criteria for Adverse Events

CUFM:

Çukurova University Faculty of Medicine

DIPG:

Diffuse intrinsic pontine glioma

EGFRs:

Epidermal growth factor receptors

EFS:

Event-free survival

LTS:

Long-term survival

MRI:

Magnetic resonance imaging

MGMT:

O6-Methylguanine–DNA methyltransferase

OS:

Overall survival

PR:

Partial response

PFS:

Progression-free survival

PD:

Progressive disease

SD:

Stable disease

TMZIRIBEV:

Temozolomide, irinotecan, bevacizumab

UHS AFM:

University of Health Sciences Adana Faculty of Medicine

VECC:

Vincristine, etoposide, and carboplatin/cyclophosphamide

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Authors and Affiliations

  1. Department of Pediatric Oncology and Pediatric Bone Marrow Transplantation Unit, Faculty of Medicine, Balcali Hospital, Çukurova University, Adana, Turkey

    Ayşe Özkan, Serhan Küpeli, Gülay Sezgin & İbrahim Bayram

  2. Department of Pediatric Hematology and Oncology, Adana City Training and Research Hospital, University of Health Sciences, Adana, Turkey

    Begül Yağcı Küpeli

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  1. Ayşe Özkan
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  2. Begül Yağcı Küpeli
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Contributions

A.Ö. and B.Y.K. contributed to the study conception and design. Material preparation, data collection, and analysis were performed by A.Ö., S.K., G.S., and İ.B. The first draft of the manuscript was written by A.Ö., and all the authors commented on previous versions of the manuscript. All the authors reviewed and approved the final manuscript.

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Correspondence to Ayşe Özkan.

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Özkan, A., Yağcı Küpeli, B., Küpeli, S. et al. Nimotuzumab-vinorelbine combination therapy versus other regimens in the treatment of pediatric diffuse intrinsic pontine glioma. Childs Nerv Syst (2024). https://doi.org/10.1007/s00381-024-06329-4

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