In addition, EBV+ T/NK LPD involving extranodal sites in children and adults, such as SEBVTCL and ANKL, can occasionally show prominent LN involvement either at disease presentation or following transformation from CAEBV disease and should not be diagnosed as primary nodal-EBV-TNKL, which affects elderly patients and often associated with immunosuppression. The gene discussed is TNKS2; the disease is disseminated peritoneal leiomyomatosis.