Aggressive infantile fibromatosis
D P Yesudian1, S G Krishnan, M Jayaraman
1Department of Dermatology, Madras Medical College and Government General Hosptial, Madras, India.
Insights
An aggressive infantile fibromatosis, a rare childhood fibrous tumor, was diagnosed in an infant with rapidly growing skin lesions. Histopathology confirmed fusiform fibroblast proliferation, suggesting this juvenile fibromatosis type.
Area of Science:
- Pediatric Dermatology
- Surgical Pathology
- Oncology
Background:
- Juvenile fibromatoses are a group of fibrous tissue tumors that manifest at birth or in early childhood.
- Infantile fibromatosis represents a subset of these conditions, characterized by localized fibrous proliferation.
Purpose of the Study:
- To present a case of aggressive infantile fibromatosis in an infant.
- To highlight the clinical and histopathological features aiding in diagnosis.
Main Methods:
- Clinical examination of an 8-month-old infant with skin lesions on the lower limb.
- Histopathological analysis of biopsied tissue, focusing on cellular morphology and arrangement.
Main Results:
- The infant presented with two ulcerated, indurated plaques on the right lower limb.
- Biopsy revealed a proliferation of fusiform fibroblasts, with some arranged in whorls.
- Lesion growth initially rapid, but stabilized over the preceding two months.
Conclusions:
- The clinical presentation and histopathological findings strongly supported a diagnosis of aggressive infantile fibromatosis.
- This case underscores the importance of considering juvenile fibromatoses in the differential diagnosis of pediatric skin tumors.
Abstract:
An 8-month-old infant presented with 2 ulcerated indurated plaques in the right lower limb. The lesions grew rapidly initially but for the last 2 months, they have been stationary. Biopsy showed proliferation of fusiform fibroblasts some of which were arranged in whorls. The clinical picture and the histopathology suggested the diagnosis of an aggressive infantile fibromatosis. This is one of the types of juvenile fibromatoses, a group of tumours characterised by fibrous tissue proliferation present at birth or acquired early in childhood.
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