Both cases illustrate that EBV-positive T/NK LPD involving extranodal sites, such as ANKL and SEBVTCL, can occasionally show prominent nodal disease at presentation or following transformation from systemic or localized forms of CAEBVD and should not be misdiagnosed as primary nodal-EBV-TNKL, a disease of adults and elderly patients [27–29]. This evidence concerns the gene TNKS2 and disseminated peritoneal leiomyomatosis.