Lipofibromatosis
Elizabeth A. Montgomery, MD
Key Facts
Clinical Issues
Painless mass
Hands and feet of children
Median age: 1 year
Male predominance (M:F = 2:1)
Local recurrences common
No metastases reported
Microscopic Pathology
Alternating fascicles of fibrous tissue and adipose tissue
Infiltrative growth pattern
Overall lobulated arrangement of adipose tissue
Vacuolated (lipoblast-like) cells
Seen at areas of interface between adipose and fibrous tissue
 Hematoxylin & eosin shows lipofibromatosis at low magnification. The tumor is fibroblastic, but adipose tissue is an integral part of the lesion. Such tumors usually arise in the hands of children. |
 Hematoxylin & eosin shows higher magnification of lipofibromatosis. Cells resembling lipoblasts  are encountered in the zones where adipose tissue and fibrous components merge. |
TERMINOLOGY
Synonyms
Infantile fibromatosis, nondesmoid type
Definitions
Fibroadipose tissue tumor of childhood with predilection for distal extremities
ETIOLOGY/PATHOGENESIS
Genetics
3-way t(4;9;6) translocation reported in 1 case
CLINICAL ISSUES
Presentation
Painless mass
Hands and feet of children
Median age: 1 year
Male predominance (M:F = 2:1)
Prognosis
Local recurrences common
Risk factors for recurrence
Congenital onset, male gender, mitotic activity in fibrous component, incomplete excision
No metastases reported
MACROSCOPIC FEATURES
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