Inclusion Body Fibromatosis



Inclusion Body Fibromatosis


Thomas Mentzel, MD









This photograph of a hand shows an exophytic, dome-shaped mass. Patients with inclusion body fibromatosis usually present with clinically evident masses.






The presence of eosinophilic cytoplasmic inclusions image represents the morphologic hallmark of inclusion body fibromatosis.


TERMINOLOGY


Synonyms



  • Infantile digital fibromatosis


  • Digital fibrous tumor of childhood


  • Reye tumor


Definitions



  • Benign proliferation of fibroblasts and myofibroblasts containing scattered eosinophilic spherical inclusions that occur on the digits of young children


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Rare fibroblastic/myofibroblastic neoplasm


  • Age



    • Occurs usually in 1st year of life


    • Very rare in adult patients


  • Gender



    • M = F


Site



  • Dorsal aspects of hands or feet


  • Rarely synchronous or asynchronous involvement of more than 1 digit


  • Thumb or big toe is only very rarely affected


  • Extradigital soft tissues (i.e., arm, breast) are extremely rarely affected


Presentation



  • Digital enlargement


  • Dome-shaped swelling overlying phalanges or interphalangeal joints


  • Nontender nodules


  • Rarely erosion of bone


Natural History



  • May recur locally


  • May regress spontaneously


  • No progression


  • No metastases


Treatment



  • Surgical approaches



    • Local excision with preservation of function


Prognosis



  • Excellent overall prognosis


  • May recur locally


  • May show spontaneous regression


  • Main prognostic indicator represents adequacy of primary excision


MACROSCOPIC FEATURES


General Features



  • Ill-defined neoplasms


  • Dermal neoplasms with gray-white, indurated cut surfaces covered by intact skin


  • No areas of hemorrhage


  • No areas of necrosis


Size



  • Nodules of variable size



    • Nodules usually measure < 2 cm


MICROSCOPIC PATHOLOGY


Histologic Features

Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Inclusion Body Fibromatosis
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