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May 2007
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Lipofibromatosis is a rare pediatric lesion composed of abundant adipose tissue together with bundles of fibroblasts. According to Fetsch et al - [
this tumour comprises part of the spectrum of what has
been referred to in the literature as infantile/juvenile fibromatosis, its
clinicopathologic features and, in particular, its distinctive tendency to
contain fat as an integral component, warrant separate classification as a
"lipofibromatosis."] Age: 11 days to 12 years Site: The patients usually presented with a soft tissue mass involving upper and lower extremities, trunk , head or neck. Microscopic features: Two components:1.Bundles of spindle shaped fibroblast-like cells with focal fascicular pattern 2. Disorganized mature adipose tissue.The lesion mainly involved the septa of fat and skeletal muscle. Mitotic activity is limited - Less than I/10HPF. Cytological atypia is not prominent. The tumours entrapped vessels, nerves, skin adnexa and skeletal muscle. Immunohistochemistry: CD99 ; smooth muscle actin ; bcl2 - Focal Positivity , S100 protein ; muscle actin ; EMA - Less frequently positive , Desmin ; Keratins ; CD57 - Negative Differential diagnosis: -Fibrous hamartoma of infancy: In lipofibromatosis primitive nodular fibromyxoid component is absent. -Calcifying aponeurotic fibroma ; -Lipoblastoma
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