Solitary intracranial myofibroma in a child

Poor outcome has generally been associated with extensive visceral involvement and relates either to mass effect with compression of vital organs and structures, or to pulmonary involvement, when subintimal or submucosal cellular proliferation results in vascular or bronchial obliteration. 2 Central nervous system involvement is exceptionally rare and has been reported as a finding in the multicentric type of myofibromatosis. 6-9 We describe a solitary interhemispheric myofibroma which presented as an intracranial mass in a 20 month old child. Solitary lesions of infantile myofibromatosis are more common than multiple lesions, with twice as many males as females being afflicted, and generally involve the skin and soft tissues, especially of the head and neck. 2 Solitary lesions are less commonly found in viscera or bones. 11 Involvement of the CNS is exceedingly rare and only one other case of a solitary mass is reported 10 along with few cases of CNS involvement in the generalised form of infantile myofibromatosis. 6-9 11-13 The prognosis is best for cases with solitary masses and less favourable for multicentric cases, particularly where visceral lesions are present, in which morbidity and mortality derive predominantly from pulmonary involvement or mass effect.