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Infantile (desmoid-type) fibromatosis
M Keltz1, D DiCostanzo, P Desai
1Department of Dermatology, Beth Israel Medical Center, New York, NY 10003, USA.
Pediatric Dermatology
|June 1, 1995
Summary
Infantile (desmoid-type) fibromatosis is a rare, aggressive tumor in children. Early diagnosis and treatment, including surgical amputation, are crucial for managing this potentially fatal condition.
Area of Science:
- Dermatology
- Pediatric Oncology
- Surgical Pathology
Background:
- Infantile (desmoid-type) fibromatosis is a rare soft tissue tumor affecting infants and children.
- It is characterized by local aggressiveness and a tendency for recurrence.
- Dermatologic literature infrequently reports on this condition.
Observation:
- A case of infantile (desmoid-type) fibromatosis in a 2-year-old girl is presented.
- The tumor affected a digit, necessitating surgical intervention.
- Clinical presentation and diagnostic challenges are highlighted.
Findings:
- Histopathological confirmation of desmoid-type fibromatosis.
- The tumor's aggressive nature required aggressive management.
- Successful surgical removal achieved through amputation.
Implications:
- Highlights the importance of recognizing infantile (desmoid-type) fibromatosis in pediatric dermatology.
- Emphasizes the need for prompt diagnosis and multidisciplinary treatment approaches.
- Underscores the potential severity and fatality of untreated infantile fibromatosis.