Digital Fibromatosis (Infantile Digital Fibromatosis)



Digital Fibromatosis (Infantile Digital Fibromatosis)


Elizabeth A. Montgomery, MD





Key Facts


Terminology



  • Benign proliferation of fibroblasts and myofibroblasts containing scattered eosinophilic inclusion bodies that occur on digits of young children


Clinical Issues



  • Rare fibroblastic/myofibroblastic neoplasm


  • Occurs usually in 1st year of life


  • Dorsal aspects of hands or feet


  • Presents with digital enlargement


  • Dome-shaped swelling overlying phalanges or interphalangeal joints


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


  • May recur locally, but excellent prognosis


  • May show spontaneous regression


  • Local excision with preservation of function


Macroscopic Features



  • Ill-defined neoplasms


Microscopic Pathology



  • Infiltrating fascicles


  • Uniform spindle-shaped tumor cells


  • No significant cytologic atypia


  • Pale eosinophilic, fibrillary cytoplasm


  • Intracytoplasmic eosinophilic spherical inclusions


  • Inclusions are trichrome positive


Ancillary Tests



  • Spindled cells show features of myofibroblasts


  • Expression of actins, desmin, calponin, and CD99


  • Inclusions show granular &/or filamentous features by EM


  • Cytoplasmic filaments extend onto inclusions







Clinical examination of infantile digital fibromatosis shows an exophytic, dome-shaped superficial neoplasm, which presents in infants and small children.






Eosinophilic-staining cytoplasmic inclusions image are the morphologic hallmark of infantile digital fibromatosis. The proliferating myofibroblasts each contain a small to slightly enlarged nucleolus image.


TERMINOLOGY


Synonyms



  • Infantile digital fibromatosis


  • Digital fibrous tumor of childhood


  • Inclusion body fibromatosis


Definitions



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


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Rare fibroblastic/myofibroblastic neoplasm


  • Age



    • Most cases occur in 1st year of life


    • Very rare in adult patients


  • Gender



    • M = F


Site



  • Dorsal aspects of hands or feet most common


  • Rarely synchronous or asynchronous involvement of more than 1 digit


  • Thumb or great toe is only very rarely affected


  • Extradigital soft tissues (i.e., arm, breast) are only 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 is adequacy of primary excision


MACROSCOPIC FEATURES


General Features



  • Ill-defined neoplasm


  • Dermal-based neoplasm with gray-white, indurated cut surface covered by intact skin


  • No areas of hemorrhage


  • No areas of necrosis


Size



  • Nodules of variable size



    • Usually measure < 2 cm


MICROSCOPIC PATHOLOGY


Histologic Features

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Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Digital Fibromatosis (Infantile Digital Fibromatosis)

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