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A Murine Ommaya Xenograft Model to Study Direct-Targeted Therapy of Leptomeningeal Disease
Published on: January 29, 2021
Leptomeningeal melanoma in childhood
G W Makin1, O B Eden, L S Lashford
1Academic Unit of Pediatric Oncology, Christie Hospital NHS Trust, Withington, Manchester, United Kingdom.
Background:
Malignant melanoma (MM) is one of the least common types of childhood cancer, accounting for less than 1% of all pediatric malignancies. Neurocutaneous melanosis (NCM) is a rare phakomatosis consisting of congenital abnormal pigmentation of the skin and meninges. The meningeal lesions are particularly prone to malignant change.
Methods:
The authors describe 5 patients with NCM and 1 with primary leptomeningeal melanoma (LMM) seen at 2 treatment centers in the north of England over a 13-year period (1984-1997).
Results:
The clinical features, progress, radiological findings, and treatment of these patients are discussed. All six died within eight months of their diagnosis, illustrating the difficulties faced in treating patients with these conditions. The authors reviewed the published literature on NCM, concentrating on the various therapeutic strategies that have been tried. Very little consistency in approach was found. Malignant skin lesions in NCM may be less responsive than primary malignant melanoma, but the small number of patients with primary LMM or brain metastases of MM make comparisons with NCM difficult. The authors' own series illustrates well the piecemeal nature of therapy for patients with these rare conditions.
Conclusions:
The rate of incidence of MM melanoma in the U.K. is increasing, and it will represent an increasing proportion of the pediatric oncologist's workload. A consistent approach to the therapy of patients with metastatic MM and NCM is needed if we are to have any hope of offering more than palliative therapy to these children in the future.
Insights
Pediatric malignant melanoma (MM) and neurocutaneous melanosis (NCM) are rare, aggressive childhood cancers. Current treatments offer limited success, highlighting the urgent need for consistent therapeutic strategies for better outcomes.
Area of Science:
- Pediatric Oncology
- Dermatology
- Neurology
Background:
- Malignant melanoma (MM) is a rare pediatric cancer, comprising less than 1% of childhood malignancies.
- Neurocutaneous melanosis (NCM) is a rare congenital disorder characterized by abnormal skin and meningeal pigmentation, with meningeal lesions posing a high risk for malignant transformation.
Observation:
- A study reviewed 5 patients with NCM and 1 with primary leptomeningeal melanoma (LMM) over 13 years.
- All patients diagnosed with these conditions died within eight months, underscoring treatment challenges.
Findings:
- Therapeutic strategies for NCM show significant inconsistency in the literature.
- Malignant skin lesions in NCM may exhibit lower responsiveness compared to primary malignant melanoma.
- Limited data on primary LMM and MM brain metastases hinder direct comparisons with NCM.
Implications:
- The increasing incidence of MM in the UK will impact pediatric oncologists' caseloads.
- A standardized therapeutic approach for metastatic MM and NCM is crucial for improving survival rates in affected children.
- Developing consistent treatment protocols is essential for moving beyond palliative care for these rare pediatric cancers.
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