Surgical Pathology Criteria–

Lipofibromatosis

Differential Diagnosis

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

Fibrolipomatous Hamartoma of Nerve Lipofibromatosis
Fat with fibrous component Fat with cellular fibroblastic septa
Centered on and surrounds a major nerve 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
Lipofibromatosis may represent part of the spectrum of infantile fibromatosis

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
Both frequently involve hands or feet

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