The efficacy and safety of imatinib therapy in a case of non-familial cherubism

CASE REPORTS

The efficacy and safety of imatinib therapy in a case of non-familial cherubism

Sarah M. Alfaqaih¹, Mohamed Bashaga^2,3, Yaser Omar Howayw⁴.

¹Pediatric Oncology Department, National Cancer Institute, Misurata, Libya,
²Faculty of Medicine, University of Misurata, Libya,
³Diagnostic Radiology department, National cancer institute, Misurata, Libya,
⁴Oral and Maxillofacial Department, Misurata Medical Center, Misurata, Libya.

Show affiliations

Abstract
Background: Cherubism is a rare autosomal dominant inherited disorder, caused by a mutation in the SH3BP2 gene. Imatinib has been effectively reported as an option of treatment. Imatinib, a tyrosine kinase inhibitor (TKI), has been established to promote osteoblast differentiation. According to our knowledge, this is the first reported case of cherubism in Libya that suggest effectivity of Imatinib.
Case presentation: We present the case of a 4-year-old boy was diagnosed with cherubism. The child was consistently administered a daily oral dosage of imatinib (300 mg/m²) for three months. Throughout the treatment, the patient was closely monitored every two weeks for potential side effects and signs of clinical improvement.
Conclusion: The observed therapeutic potential of imatinib for cherubism in this case study suggests a promising avenue for future exploration. However, further research is needed to establish a safe and effective protocol for imatinib treatment in pediatric cherubism.

Why this article important?

The treatment options for cherubism raise several dilemmas for the treating physician, as expected of the self-limiting and usually involuntary consequences of treatment effects. On the other hand, the early aggressive course of the disease and the major disfigurement and functional complications indicate a search for a minimally invasive modality of treatment. The surgical intervention is the typically reported first-line module of treatment for symptomatic and plastic surgery. For severe cases, the timing of intervention and surgical approach remain the most challenging parts for surgeons. The key finding of our case report highlights the significant reduction in swelling size with minimal adverse events observed during imatinib therapy for Cherubism. This study suggests a promising avenue for further exploration. We thoroughly discuss our findings alongside similar reports in the literature involving patients treated with different pharmacological therapies.

Summary of article

We are pleased to submit a manuscript titled "Efficacy and Safety of Imatinib Therapy in a Case of Non-Familial Cherubism" for your consideration in the Pediatric On-Call Journal. Imatinib, an inhibitor of c-abl, c-kit, and platelet-derived growth factor tyrosine kinases, has been shown to promote osteoblast differentiation. While the Food and Drug Administration approves it for treating chronic myeloid leukemia and acute lymphoblastic leukemia in children, its effectiveness in treating Cherubism remains uncertain due to the rare cases. To address this gap, we present a case study where Imatinib was used to treat Cherubism, reporting its efficacy and adverse events during treatment and follow-up.

READ FULL TEXT

Home

Articles & Issues

About

Contribute

Pediatric Oncall Journal View Article

Home

Partner Sites