Another Look at PCSM-TCL
A small case series shows occurrence in children and supports its classification as a low-grade lymphoma.
Baum CL et al. 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. J Am Acad Dermatol 2011 Oct; 65:739.
Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma (PCSM-TCL) was provisionally included in the World Health Organization–European Organization for Research and Treatment of Cancer (WHO-EORTC) lymphoma classification of 2005. The umbrella term described disease characterized by dermal infiltrates of small- to medium-sized T cells without clinical or pathological evidence of mycosis fungoides.
This review of 10 cases identified in one tertiary academic center includes the youngest patient (age, 6 years) reported in the literature. Most of these patients (4 pediatric [age range, 6 to 16 years]; median age, 38 years; male:female ratio, 3:2) presented with a single papule, nodule, or plaque on the proximal trunk, head, or neck. No evidence of recurrence was seen in 70% of patients at a median of 2 years after treatment by excision, radiation, or some combination of excision, radiation, prednisone, and topical corticosteroids. No evidence of systemic spread was identified. Histopathology showed dermal infiltrates of small- to medium-sized lymphocytes without epidermotropism. CD4:CD8 T-cell ratios varied from 3:1 to 20:1. The numbers of eosinophils, histocytes, and mast cells varied, but a monoclonal population of T-cell lymphocytes was detected in all patients.
Comment: PCSM-TCL lymphoma should be considered in the differential diagnosis of plaques or nodules appearing on the head or neck of adults and children. These lesions result from clonal expansion of CD4+ T-cell lymphocytes that infiltrate the dermis. This provisional entity appears in most reports to be an indolent disease with a 5-year survival rate of 60% to 80%. Cases of CD8+ lymphoid infiltrates of the face and ear with the same indolent behavior have been reported, and additional cases are needed to show if these lymphomas are aspects of the same disease. The clinical data show PCSM-TCL to be a low-grade lymphoma; therefore, excision, radiation, and prednisone are the treatments of choice in the adult population. For the pediatric patient, excision or prednisone is favored over radiation, and long-term follow-up is very important.
— Angelica Selim, MD. Published in Journal Watch Dermatology October 7, 2011
Medline abstract (Free)
