Myofibroma in Infancy and Childhood

Area of Science:

• Pediatric Oncology
• Pediatric Pathology
• Surgical Oncology

Background:

• Myofibromas are rare fibroblastic-myofibroblastic tumors in children.
• Their clinical behavior is unpredictable, with documented spontaneous regressions.
• Understanding their characteristics is crucial for appropriate management.

Purpose of the Study:

• To review the clinical characteristics, treatment, and outcomes of pediatric myofibromas.
• To evaluate the impact of surgical margins on tumor progression.
• To identify potential benefits of chemotherapy for unresectable cases.

Main Methods:

• Retrospective review of 42 children diagnosed with myofibroma between 1999 and 2013.
• Analysis of patient demographics, lesion location, treatment modalities, and follow-up data.
• Correlation of surgical margin status with tumor progression and patient survival.

Main Results:

• The median age at diagnosis was 37 months; 90% had solitary lesions, predominantly in the head and neck.
• Complete surgical resection was performed in 12 patients.
• Positive surgical margins did not adversely affect outcomes, with only 1 of 30 patients experiencing progression.
• Two patients had multicentric or generalized disease; one with progressive disease benefited from chemotherapy.

Conclusions:

• Pediatric myofibromas exhibit significant clinical variability and can undergo spontaneous regression.
• Positive surgical margins are not necessarily indicative of adverse outcomes.
• Chemotherapy may be beneficial for rare cases of progressive, unresectable myofibromas.