Aggressive Fibromatosis in Children

Synonyms

Aggressive fibromatosis; Desmoid tumor

Definition

Aggressive fibromatosis (AF) is a rare soft tissue tumor and rare in childhood with high potential for local invasiveness and recurrence. Primary surgery with negative margins is the most successful primary treatment modality for children with AF. Positive resection margins after surgery indicate a high risk for relapse. Multicenter prospective (randomized) trials are necessary to clarify the role of and best strategy for treatment in pediatric AF after incomplete surgery. For this purpose, chemotherapy or alternatively radiotherapy can be considered, each with its own potential side effects in consequence.

Characteristics

Aggressive fibromatosis (AF) (Supportive care) is a soft tissue tumor, which arises principally from the connective tissue of muscle and the overlying fascia (aponeurosis). The previously most used synonym is desmoid tumor. The histological pattern is characterized by elongated fibroblast-like cells....