Peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) accounts for the largest proportion of mature T-cell and natural killer (NK)-cell neoplasms in the 2017 World Health Organization (WHO) classification. 1 We have found evidence that nodal cytotoxic molecule (CM)-positive PTCL-NOS [(also called cytotoxic T-cell lymphoma (CTL)] constitutes a unique category. This group encompasses a wide spectrum of presentations, ranging from indolent disease in a minority to an aggressive neoplasm in the majority; in 45-51% of CTL cases, the pathogenesis is closely associated with Epstein-Barr virus (EBV). 2,3 These biological properties are in clear contrast to those of nodal CM-negative T-cell neoplasms, which predominantly comprise PTCL-NOS, angioimmunoblastic T-cell lymphoma, and adult T-cell leukemia (ATLL, positive for human T-cell leukemia virus type 1)-although a small subset of patients (2%) may exhibit EBV-positive bystander lymphocytes. 2 This suggests that EBV is detected as the result of immunological deterioration in the tumor microenvironment, but that it plays little role in the pathogenesis of the latter neoplasm.Among mature T/NK-cell tumors, the detection of EBV on tumor cells is essential for the diagnosis of nasal type NK/ T-cell lymphoma (NKTL), aggressive NK-cell leukemia, or EBV + T/NK-cell lymphoproliferative diseases of childhood-all of which exhibit CM expression and frequent extranodal involvement. 4 In most NKTL cases, the prototypic disease initially presents in the upper aerodigestive tract. This disease can also involve a wide variety of extranodal sites, including the skin and gastrointestinal tract, and may exhibit secondary lymph node lesions. Indeed, NKTL affecting middle-aged or elderly patients may feature de novo symptoms with clonal and rapid expansion of neoplastic EBV + T/NK cells. 5 Patients with nodal EBV + CTL present with lymphadenopathy without nasopharyngeal involvement. However, this nodal lymphoma and NKTL share some biological properties, including CM expression and EBV positivity. We first elucidated the clinicopathological characteristics of nodal EBV + CTL, which is characterized by diffuse monomorphic infiltration of large cells that often have a centroblastoid