Novel Pediatric Aggressive Adipose Tumor With Pathogenic TERT Promoter Variant and an Initial Durable Response to Vincristine and Dactinomycin

Key Points

• This research aims to explore a new aggressive adipose tumor in pediatric patients with a specific TERT promoter variant and treatment response.
• Identification of a novel pediatric adipose tumor
• Analysis of TERT promoter variant
• Evaluation of treatment response to vincristine and dactinomycin
• The tumor displayed a pathogenic TERT promoter variant
• There was an initial durable response to vincristine and dactinomycin
• This suggests potential effectiveness of these therapies in similar cases.