When Rare Meets Young: Pediatric Presentation of Desmoplastic Fibroblastoma

Abstract

Desmoplastic fibroblastoma (DF), also known as collagenous fibroma, is a rare benign soft tissue tumor that predominantly occurs in adults. It consists of a well-circumscribed proliferation of stellate and/or spindle-shaped fibroblasts embedded within an abundant, hypovascular collagenous stroma. We report a very rare case of DF arising in the left thigh of a 12-year-old boy, located in the subcutaneous tissue and measuring 4.2 cm at its greatest dimension. Two years after the complete surgical excision, the patient remains free of recurrence. To date, only 5 additional cases of DF have been reported in pediatric patients, predominantly affecting males and demonstrating a uniformly benign clinical course. Our report further supports the potential occurrence of DF in childhood and adolescence, underscoring the importance of including this entity in the differential diagnosis of benign fibroblastic or fibrohistiocytic lesions even in pediatric soft-tissue pathology.