Infantile Fibrosarcoma



Infantile Fibrosarcoma


Thomas Mentzel, MD









Gross photograph shows a large, hemorrhagic, congenital soft tissue neoplasm arising on the arm of a 13-day-old female baby.






Hematoxylin & eosin shows a cellular neoplasm containing numerous thin-walled blood vessels. The neoplasm is composed of plump spindled, immature-appearing fibroblastic tumor cells.


TERMINOLOGY


Synonyms



  • Congenital fibrosarcoma


  • Juvenile fibrosarcoma


  • Aggressive infantile fibromatosis


Definitions



  • Occurs in infants and young children and represents a low-grade fibrosarcoma that carries favorable prognosis


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Accounts for ˜ 12% of mesenchymal malignancies in infants


  • Age



    • Congenital or in 1st 2 years of life


    • Rare in children older than 2 years


  • Gender



    • Slight male predominance


Site



  • Superficial and deep soft tissues of extremities are most common sites


  • More rarely seen on trunk and in head and neck region


  • Very rare in retroperitoneum and in mesentery


Presentation



  • Limb enlargement


  • Painless mass



    • Solitary neoplasms


    • Usually very large neoplasms


    • Overlying skin may be ulcerated


  • Suddenly enlarging mass


Natural History



  • Often short preoperative duration


Treatment



  • Surgical approaches



    • Complete excision with tumor-free margins


  • Adjuvant therapy



    • Chemotherapy has been proven effective


Prognosis



  • Recurrence rate varies (5-50%)


  • Metastases are very rare


  • Mortality ranges from 5-25%


  • Spontaneous regression has been reported


  • Favorable prognosis


IMAGE FINDINGS


General Features



  • Usually large soft tissue mass


  • Shows heterogeneous enhancement pattern


  • May show osseous erosion


MACROSCOPIC FEATURES


General Features



  • Poorly circumscribed, lobulated soft tissue tumor


  • Infiltration of adjacent tissues


  • Firm gray-white cut surfaces


  • Variable myxoid changes


  • Areas of hemorrhage and necrosis are sometimes present


MICROSCOPIC PATHOLOGY


Histologic Features



  • Cellular neoplasms


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