Primary Cutaneous Anaplastic Large Cell Lymphoma With 6p25.3 Rearrangement and Epidermotropism

Primary cutaneous anaplastic large cell lymphoma may harbor a 6p25.3 rearrangement, which has been associated with an epidermotropic small cell component. We report the case of a patient with said lymphoma harboring that rearrangement. It presented as a forehead nodule, histologically composed of an intermediate-to-large cell dermal component alongside a small-to-intermediate cell epidermotropic component. After multiple cutaneous and regional lymph node relapses, disease progression has been documented to a distant lymph node, despite local radiotherapy of the cutaneous lesions, chemotherapy, and anti-CD30 therapy, albeit with an indolent course over 6 years. Cases of pcALCL with nonregional lymph node involvement are unusual. Nevertheless, in this case, progression to a distant lymph node was not associated with an aggressive transformation of the disease.