Juvenile Fibrosarcoma (Infantile Fibrosarcoma) of the Eyelid or Orbit

Features:

(1) onset below 10 years of age

(2) may present as a painless, rapidly enlarging mass or proptosis

(3) histology shows malignant spindled fibroblasts or myofibroblasts which are mitotically active

(4) tumor cells may show herringbone pattern or interlacing fascicles

(5) may show areas of hemorrhage and necrosis

Immunohistochemistry:

(1) negative for S100, desmin and CD34

(2) actin variable

A translocation t(12;15) (p13;q26) is present in most cases.

The diagnosis requires exclusion of other types of sarcomas.

The tumor has a good prognosis if managed properly with excision with or without chemotherapy.