Total Skin Electron Irradiation (TSEI) May Induce Systemic Progression of Mycosis Fungoides (MF) - Cutaneous T-Cell Lymphoma (CTCL)

Abstract

The management of MF-CTCL is based on a "stage-based" approach and an evaluation by a multidisciplinary team is preferred. Initial treatment in patients with local (patch/ plaque) disease includes skin-directed therapies (localized or generalized), with the addition of systemic biologic therapy for refractory, or progressive disease. In patients with unfavorable prognostic features (e.g., folliculotropic or large-cell transformed MF, or B1 involvement) systemic biologic therapies may be introduced earlier in the treatment algorithm. Patients who do not respond to biologic therapy or those with very aggressive or extracutaneous disease may be treated with chemotherapy [1-3].TSEI represents an integral part in the treatment of advanced MF. TSEI is characterized by a favorable local toxicity profile with excellent response rates. The impact of TSEI on systemic progression remains unclear. A 75-YOAAM with stage IIB Cutaneous T-Cell Lymphoma had clinically indolent disease for many years. He then presented with multiple cutaneous lesions with increasing evolution of his disease. He received TSEI to 18 Gy in 12 fractions followed by additional boost to many sites. One month later, he presented with diffuse neck swelling, difficulty breathing, and hoarseness. PET/CT scan revealed diffuse systemic progression. Despite salvage radiation (RT) and chemotherapy, his disease quickly progressed further and was ultimately fatal. TSEI's impact on systemic progression of MF merits further investigation, especially in the context of clinicopathological evaluation of MF transformation and the potential value of combining novel immunotherapeutic agents like checkpoint inhibitors with RT to potentially further improve outcomes for such patients with aggressive transformation MFCTCL.