Wada H, Akiyama H, Seki H, Ichihara T, Ueno K, Miyawaki T, Koizumi S
Department of Pediatrics, School of Medicine, Kanazawa University, Ishikawa, Japan.
J Pediatr Hematol Oncol. 1998 Jul-Aug;20(4):353-6. doi: 10.1097/00043426-199807000-00015.
An unusual case of infantile myofibromatosis with spinal canal involvement is reported and the literature is reviewed.
A female neonate had bladder and bowel dysfunction and paresis of the lower extremities.
Intrapelvic and paravertebral masses with extension into the spinal canal were detected by imaging studies. In addition, radiologic examination showed multiple metaphyseal radiolucent lesions of the long bones and pathologic fracture of the left femur. The histopathologic diagnosis of the paravertebral tumor and bone lesions was infantile myofibromatosis. Surgical resection of the paravertebral and intrapelvic masses was performed to improve her neurologic impairments. Paresis of the lower limbs gradually improved, whereas bladder and bowel dysfunction remained unchanged.
Only six cases of infantile myofibromatosis associated with spinal canal involvement have been reported. Three patients with flaccid paresis of extremities and respiratory distress died in the newborn period. The other three patients showed improvement of the paresis. The prognosis of infantile myofibromatosis without visceral complication is generally good, but neurologic impairment may occur at birth if the spinal cord is compressed.
