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  • Purely intraparenchymal solitary Infantile Myofibroma of the Central Nervous System without dural attachment

    Final Number:

    Alexander Gregory Weil MD FRCSC; Sanjiv Bhatia MD; John Ragheb MD, FACS, FAAP; Toba Niazi MD

    Study Design:

    Subject Category:

    Meeting: Congress of Neurological Surgeons 2014 Annual Meeting

    Introduction: Infantile myofibroma, the most common fibrous disorder of infancy and early childhood, is characterized by the formation of mesenchymal tumors of skin, soft tissue, muscle, bone and viscera. Intracranial central nervous system (CNS) involvement has been reported with invariable skull or dural involvement with variable intracranial extension. We report the rare occurrence of a purely intra-axial CNS infantile myofibroma with no bony or dural involvement.

    Methods: Case report and literature review

    Results: A 14-month-old boy presented with a 1-week history of left hemiparesis. Brain Magnetic Resonance Imaging revealed a large right hemispheric peri-Sylvian cystic mass with a mildly enhancing nodule with significant vasogenic oedema, mass effect and midline shift. The patient underwent gross total resection of the purely intraparenchymal lesion. At 4-month follow-up, the child has no deficits. Pathology revealed a spindle cell neoplasm of low cellularity/proliferation and smooth muscle actin expression (mesenchymal differentiation), consistent with a myofibroma.

    Conclusions: Myofibromatosis of infancy may occur in either a multicentric or solitary form. The solitary infantile myofibroma form is usually characterized by isolated myofibroma occurring most commonly in the head, neck, extremity or trunk. Involvement of the brain is rare, and when present they usually originate form the dura and extend to invade the skull and compress the brain. The multicentric form, which represents 25% infantile myofibroma, is characterized my multiple soft tissue, bony, and viscera. Involvement of the CNS is also rare, and usually has dural involvement with extension through bone and compression of parenchyma, but usually do not transgress the arachnoid or galea. We report a rare case of a purely intraparenchymal solitary infantile myofibroma with no dural attachment in a child. Clinicians should be alerted to the possible existence of this rare presentation of infantile myofibroma.

    Patient Care: This report will alert clinicians of infantile myofibromatosis and this rare presentation of this common condition

    Learning Objectives: 1. Understand infantile myofibromatosis as a disease entity in children 2. Recognize that CNS involvement of myofibromatosis usally involves the skull or dura matter 3. Recognize the existence of the rare variant of a purely intraparenchymal myofibroma


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