Recently, familial syndromes with Hodgkin lymphomas have been reported including germinal homozygote CD27 deficiency [134], DICER1 syndrome [36,135,136], association with the human leucocyte antigen (HLA) abnormalities [137], KLHDC8B translocation [138], NPAT germinal mutation [139], homologous germinal variant of ACAN [140], and familial KDR mutations [141]. The gene discussed is ACAN; the disease is DICER1-related tumor predisposition.