Lipofibromatosis
Differential Diagnosis
- Hemosiderotic fibrolipomatous lesion
- Neural Fibrolipoma
- Juvenile fibromatosis
- Fibrous hamartoma of infancy
- Lipoblastoma / lipoblastomatosis
- Calcifying aponeurotic fibroma
| Hemosiderotic Fibrohistiocytic Lipomatous Lesion | Lipofibromatosis |
|---|---|
| Circumscribed | Poorly circumscribed |
| Prominent hemosiderin | Lacks hemosiderin |
| Includes histiocytes and other inflammatory cells | Histiocytes and inflammation not integral to lesion |
| Occasional floret cells | Lacks floret cells |
| Neural Fibrolipoma (Fibrolipomatous Hamartoma of Nerve) | Lipofibromatosis |
|---|---|
| Fat with fibrous component | Fat with cellular fibroblastic septa |
| Centered on and surrounds a major nerve with epineurial and perineurial thickening | Soft tissue mass, may surround nerve focally |
| Frequently associated with macrodactyly | Not associated with macrodactyly |
| Infantile Fibromatosis | Lipofibromatosis |
|---|---|
| Solid, sheet-like pattern | Septal fibroblastic pattern |
| Overrun, effaced fat | Fat is integral part of lesion |
| Usually head, neck, shoulder, thigh | Usually distal extremities |
| Fibrous Hamartoma of Infancy | Lipofibromatosis |
|---|---|
| Rarely if ever in hands or feet | Frequently hand or feet |
| Hyalinized, "wire-like" collagen | Collagen not prominent |
| Whorled, myxoid nodules of immature cells | Myxoid areas lack nodular pattern |
| Lipoblastoma / Lipoblastomatosis | Lipofibromatosis |
|---|---|
| Septa infrequently cellular | Prominently cellular septa |
| Usually immature fat mixed with mature | Mature fat |
| Fat cells may have multiple vacuoles | Single fat vacuoles |
| Calcifying Aponeurotic Fibroma | Lipofibromatosis |
|---|---|
| Lacks fatty component | Fat is integral to lesion |
| Cellular nodules of epithelioid cells with palisading | Septal fibroblastic pattern |
| Chondroid differentiation and calcification common | Lacks chondroid differentiation and calcification |
