Fibroblastic Reticulum Cell Sarcoma

Case Study

This is a 44 year old man who presents with a tumor in the right hip.

Diagnosis:

Fibroblastic Reticulum Cell Sarcoma

This is an extremely rare and controversial soft tissue sarcoma. Additional immunoperoxidase studies were performed showing positivity of the large atypical cells for desmin and caldesmon. Additional stains for cytokeratins, S-100 protein, LCA (leukocyte common antigen), CD34, CD68, CD15, CD39, and smooth muscle actin were negative.

This case was reviewed by Christopher D.M. Fletcher, M.D., director of surgical pathology at the the Brigham and Women's Hospital in Boston, Massachusetts, and an expert in soft tissue tumors. He felt the unusual morphology of the the large atypical cells combined with the immunohistochemical profile is most consistent with the above diagnosis.

The differential diagnosis for this unusual tumor is extensive and includes Hodgkin lymphoma and an anaplastic lymphoma, both excluded by the negative stains for LCA, CD15, and CD30. A metastatic carcinoma and melanoma are excluded by the negative stains for cytokeratins and S-100.

Most cases have pursued a low grade clinical course. A recommendation was made for wide excision and careful clinical follow up.

References:

The Doctor's Doctor-Reticulum Cell Sarcoma

Modern Pathology 2002;15:50-58. Extranodal Follicular Dendritic Cell Sarcoma of the Head and Neck Region: Three New Cases, with a Review of the Literature

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