Subcutaneous Panniculitis-Like T-Cell Lymphoma in Children: A Review of the Literature

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a type of skin lymphoma. Pleomorphic T-cells infiltrate the subcutaneous tissue and mimic lobular panniculitis. We report a case occurring in a 12-year-old Chinese boy who presented with multiple indolent erythematous subcutaneous nodules on both extremities without systemic symptoms. He had a protracted course of multiple erythematous subcutaneous nodules for 1year and underwent biopsy of lesional skin for histology and T-cell receptor (TCR) gene analysis. Histopathology showed infiltration of medium to large atypical pleomorphic cells involving the subcutis with characteristic rimming of fat spaces. TCR gene rearrangement shows monoclonal rearrangements of the TCR and chains. Immunophenotypic studies showed that CD3, CD4, and CD8 were strongly and diffusely positive in lesional cells and that CD56 was focally positive. In contrast, these cells were negative for CD20, CD30, and CD68. The combined morphology, characteristic histologic features, and positive T-CR gene rearrangement supported a diagnosis of SPTCL. He is being treated with combination chemotherapy of cyclophosphamide, doxorubicin, vincristine, and prednisolone.