Lipofibromatosis



Lipofibromatosis


Elizabeth A. Montgomery, MD









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 image 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

Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Lipofibromatosis

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