Solitary congenital fibromatosis (infantile myofibromatosis) of bone.
Congenital fibromatosis is a rare and benign myofibroblastic tumor that may occur in either a solitary or multicentric form. The soft-tissue form of this entity is well recognized. This report analyzes the clinical and pathologic features of 14 cases of congenital fibromatosis in which the tumor existed as a solitary lesion in bone. Most of the tumors (71%) were in patients who were 2 years old or younger. All but one tumor involved the craniofacial bones. Radiographically, they were purely lucent lesions in which a sclerotic rim was visible in each skull lesion. The myofibroblastic appearance closely resembled the microscopic features seen in the soft-tissue counterpart. For appropriate surgical management, it is imperative to recognize that this tumor occurs as a solitary lesion in bone and that it is distinct from other, more aggressive fibrous lesions.
Section of Surgical Pathology, Mayo Clinic, Rochester, MN 55905.
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D000293:Adolescent; D001859:Bone Neoplasms / Q000151:congenital* / Q000473:pathology / Q000601:surgery; D001842:Bone and Bones / Q000000981:diagnostic imaging / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D005350:Fibroma / Q000151:congenital* / Q000473:pathology / Q000601:surgery; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D014057:Tomography, X-Ray Computed