Intracranial infantile myofibromatosis

Abstract

Infantile myofibromatosis is a proliferative disorder of infancy and early childhood characterized by nodular or diffuse growth of lesions that are comprised of a mixture of mesenchymal elements within the skin, subcutaneous tissues, skeletal muscle, bone, and/or visceral organs. Although these pseudotumors are considered to be the most common fibrous "neoplasm" of infancy, central nervous system involvement is reportedly rare. During the last 7 years, the authors have treated three children with intracranial myofibromas who presented at 6 weeks, 7 months, and 3 3/4 years of age, respectively. Each child had a large calvarial mass that produced significant brain compression despite a paucity of neurological signs. On computerized tomography, these tumors were isodense to brain tissue, enhanced strongly with intravenous contrast material, and showed smoothly marginated bone erosion without surrounding sclerosis. On magnetic resonance imaging, the tumors were hypointense on T1-weighted images, with dense enhancement following the administration of intravenous contrast medium, and hyperintense on T2-weighted images. At operation, the tumors were highly vascular and appeared to arise from within the leaves of the dura, eroding through the overlying bone, but not violating the galeal or arachnoidal layers. Two of the lesions were adherent to major dural venous sinuses. Both of these lesions were completely resected in continuity with the involved dura, and have not recurred 6 years and 1 year, respectively, postoperatively. However, in one patient in whom the involved dura was not resected at the initial procedure, the tumor recurred rapidly. A complete excision of the tumor and involved dura was then performed and the patient is now recurrence-free, 5 1/2 years after the second surgical resection. All patients tolerated resection well, but two have required cranioplasty for persistent calvarial defects. The surgical experience with these lesions is reviewed and the distinctive features of their clinical presentation, radiographic appearance, operative management, and outcome are discussed.