Plexiform fibrohistiocytic tumor of bone

Plexiform fibrohistiocytic tumor is an extremely rare soft tissue tumor with a low malignancy potential. The patient is usually a child or a young adolescent and the tumor is usually localized in the upper extremities. We report on a case of a 21-year old male with a plexiform fibrohistiocytic tumor in the left fibula admitted to our hospital due to a swelling and pain in the left lower extremity. Radiologically a lytic lesion in the distal end of left fibula consistent with a non-aggressive lesion with low biological activity was found. Treated with curettage, the specimen revealed plexiform proliferation of mononuclear histiocyte-like cells, multinucleated osteoclast-like cells, and spindle fibroblast-like cells in variable proportions histopathologically. Immunohistochemical stains were positive for CD68 in scattered fashion in histiocytes and giant cells, and spindle like cells showed positivity for smooth muscle actin. Under electron microscopy, rough endoplasmic reticulum and collagen bundles in the spindle cells suggested fibroblastic differentiation. Also multiple large electron-dense lysosomal granules in histiocytoid cells were found. Multinucleated giant cells exhibited osteoclast-like appearance. All these findings suggested plexiform fibrohistiocytic tumor. Interestingly, the tumor was localized in bone. During the follow up for 27 months after the resection, there was no recurrence or metastasis.