Tumors with features of LCNEC or focal positive IHC staining for TTF-1/NapsinA or diffuse positive IHC staining for p40/p63/CK5/6 are excluded from LCC and are now classified as solid adenocarcinoma or non-keratinizing squamous compared to the WHO 2004 classification [2]. The gene discussed is KRT5; the disease is leukoencephalopathy with calcifications and cysts.