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Infantile myofibromatosis with a solitary lesion in the skull--case report
Insights
Infantile myofibromatosis, a rare infant soft-tissue tumor, can present as a solitary skull lesion. This case highlights the computed tomography (CT) findings of such a rare cranial presentation.
Area of Science:
- Pediatric Oncology
- Pathology
- Radiology
Background:
- Infantile myofibromatosis is a rare benign soft-tissue tumor typically presenting as solitary lesions in infants.
- Cranial involvement, particularly solitary lesions in the skull, is extremely rare in infantile myofibromatosis.
Observation:
- A 6-month-old girl presented with a 2x2 cm mass in the left parietal region.
- Skull X-rays revealed an osteolytic lesion with a sclerotic margin.
- CT scans showed a low-density, homogeneously enhancing mass adhering to the dura and destroying the parietal bone.
Findings:
- Histological examination confirmed spindle-shaped cells and abundant vasculature, consistent with myofibroblasts.
- Phosphotungstic acid hematoxylin staining supported the myofibroblast identification.
- This report details the CT findings in a rare case of solitary infantile myofibromatosis involving the skull.
Implications:
- This case expands the understanding of infantile myofibromatosis presentation.
- CT imaging characteristics are crucial for diagnosing rare cranial lesions.
- Early diagnosis and characterization aid in appropriate management of infantile myofibromatosis.
Abstract:
The term "infantile myofibromatosis" was coined in 1981 to describe a rare type of soft-tissue tumor in infants. Solitary lesions are usual, but extremely rare in the skull. An infantile case involving a lesion in the left parietal bone is described. The patient was a 6-month-old girl admitted with a mass measuring 2 x 2 cm in the left parietal region. Skull x-rays showed an osteolytic lesion with a sclerotic margin in the parietal bone. Computed tomographic (CT) scans revealed a low-density mass with homogeneous enhancement. The tumor adhered to the dura and had destroyed the left parietal bone. Histological examination disclosed spindle-shaped cells arranged in short bundles and abundant vasculature. Phosphotungstic acid hematoxylin staining revealed longitudinal fibrils resembling myofibroblasts. This is the first report in which CT findings are described in a case of infantile myofibromatosis with a solitary lesion occurring in the skull.