Gardner-associated Fibroma
Definition
- Hypocellular collagenous lesion with surrounded fat similar to nuchal-type fibroma but occurring in children or in extra-nuchal sites
Diagnostic Criteria
- Histologic features similar to nuchal-type fibroma
- Not encapsulated, radiates into fat
- Frequent entrapped fat
- Subcutaneous component in all cases
- Paucicellular formless sheets of collagen
- Few, small fibrocytes
- Rare entrapped nerves
- Lacks traumatic neuroma like pattern
- Infrequent infiltration of skeletal muscle
- Not encapsulated, radiates into fat
- Most located on trunk
- Also head and neck, extremites
- May be associated with Gardner syndrome
- Such lesions in extra-nuchal sites should prompt clinical investigation for Gardner syndrome
- Found in 70%
- This may be the presenting finding
- Such lesions in extra-nuchal sites should prompt clinical investigation for Gardner syndrome
- Beta-catenin positive in 64%
- Recurrence appears to be more common than with nuchal-type fibroma
- Recurrences may be as desmoid type fibromatosis (20%)
- Gardner syndrome
- Familial adenomatous polyposis
- Osteomas, especially of mandible
- Mesenteric, and rarely abdominal, fibromatosis
- Cutaneous epidermoid cysts
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
Original posting : November 20, 2007
Last Update: December 22, 2008