Infantile Myofibroma of Scalp

Summary:

Infantile myofibroma (IM) is the most common congenital fibrous tumor of infancy, originating from fibroblasts and myofibroblasts and present at birth in approximately 50%–60% of cases. Solitary IMs most often develop in the head and neck, less frequently on the trunk or extremities, and usually have an excellent prognosis in the absence of visceral involvement. Clinically, IM appears as a painless, firm, bluish-purple, freely mobile nodule and may occasionally show ulceration or skin atrophy, clinically mimicking hemangioma. Imaging typically shows well-defined masses with low-flow or peripheral vascularity, distinguishing them from highly vascular hemangiomas. Although solitary lesions may spontaneously regress within 18–24 months, surgical excision is advised when diagnosis is uncertain, functional risk exists, or for cosmetic or psychosocial reasons. Multicentric IM without visceral involvement also has a favorable outcome, whereas lesions with visceral involvement carry significantly higher morbidity and mortality. Mutations in PDGFRB and, less commonly, NOTCH3 are reported in IM and may guide future targeted therapies. In the absence of standardized guidelines, management must be individualized, with options including observation, surgical excision, and chemotherapy for extensive or visceral disease. Regular clinical and imaging follow-up is essential until lesion stabilization or regression is confirmed. Here, we present a female neonate with a scalp IM. Persistent ulceration, recurrent bleeding, inability to definitively exclude malignancy on imaging, and significant maternal distress warranted the decision for surgical excision. This case emphasizes the importance of individualized care in IM, guided by current literature on presentation, pathology, and treatment.