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Infantile myofibromatosis. Report of two cases
Insights
Infantile myofibromatosis presents as nodular lesions in infants. These lesions are characterized by a proliferation of myofibroblasts, though their origin remains unclear.
Area of Science:
- Pediatric Pathology
- Dermatopathology
- Oncology
Background:
- Infantile myofibromatosis is a rare soft tissue tumor affecting infants.
- Understanding its histological and ultrastructural features is crucial for diagnosis.
Observation:
- Two cases of infantile myofibromatosis are presented, one with multiple lesions and another with a solitary, enlarging lesion.
- Histological examination revealed spindle-shaped cells resembling smooth muscle.
- Electron microscopy showed cells with characteristics of both fibroblasts and smooth muscle cells.
Findings:
- Immunoperoxidase staining confirmed the presence of smooth muscle antibodies in the cytoplasm of the tumor cells.
- The lesions are concluded to be a pure proliferation of myofibroblasts.
- The peculiar growth behavior was noted, but the histogenesis remains undetermined.
Implications:
- This study contributes to the understanding of infantile myofibromatosis, aiding in its diagnosis and management.
- Further research into the histogenesis of myofibroblastic tumors is warranted.
- Accurate identification of myofibroblasts is key for differentiating from other pediatric soft tissue neoplasms.
Abstract:
Two cases of infantile myofibromatosis were presented. Case 1 was a 6-month-old girl with multiple nodular lesions in the left parietal bone, dermis of abdominal wall, chest wall, right upper arm, and bilateral femur. Case 2 was a 12-month-old girl with solitary nodular lesions in the left parietal bone having a tendency to enlarge. Histologically, these tumorous lesions consisted of broad bundles of well oriented spindle-shaped cells superficially resembling smooth muscle tissue. In electron microscopy, the constituent cells combined ultrastructural characteristics of both fibroblasts and smooth muscle cells., Immunoperoxidase method showed that anti-smooth muscle antibodies binded to the cytoplasm of the bundle forming cells. From these results, it may be concluded that the lesion consisted of pure proliferation of myofibroblasts, and its peculiar growth behavior was discussed, though the histogenesis still remains obscure.