Reappraisal of the provisional entity primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoma: A series of 10 adult and pediatric patients and review of the literature

Background: Primary cutaneous CD4⁺ small/medium-sized pleomorphic T-cell lymphoma (PCSM-TCL) was defined as a provisional entity in the 2005 World Health Organization - European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas. A limited number of reports describe a generally indolent but heterogeneous condition. Objective: We reviewed the concept of PCSM-TCL when applied to our experience and to the published literature. Methods: We conducted a retrospective chart review and a PubMed search to identify all reported cases of PCSM-TCL from 2005 to 2010. Results: Ten patients, including 4 of the youngest described, were identified in our institution, and their clinical and pathologic features were analyzed. All had a benign clinical course. Ten reports of patients with PCSM-TCL were reviewed and commonalities were found within an otherwise variable spectrum of clinical presentation, pathology, and biologic behavior. Limitations: This study was retrospective, follow-up was short term in some cases, and data were limited in a number of published reports. Conclusions: Our experience of 10 cases that met the diagnostic criteria of the provisional entity PCSM-TCL highlights its occurrence in children and further supports characterization of this condition as a clonal T-cell lymphoproliferative disorder with indolent behavior.