Table 1.

Overview of the key similarities and differences between NPC, PPLELC, and EBVaGC.

	NPC	PPLELC	EBVaGC
Etiology	EBV infection	Postulated to be associated with EBV infection	EBV infection
Epidemiology	Rare in Western Countries, and more commonly found in Guangdong province of China, Hongkong, Southeast Asia, East Asia, and the Mediterranean area Males > Females	Rare in Western countries, commonly found in Asian countries like South China Females > Males	More common in Western countries like Germany and the United States Males > Females
Risk Factors	Smoking and preserved food containing volatile nitrosamine and salted fish	Smoking is not a risk factor	Smoking, salty food, exposure to wood dust and iron filings.
EBV Latency	Type II latency	Postulated to be type II latency	Type I latency
Key Molecular and Immune Characteristics	NF-кB pathway activation, mutations in CDKN2A, CCND1, TP53, JAK/STAT, PI3K-Akt pathway, and Chromosomal instability phenotype EBV-CIMP (CpG island methylator phenotype). Upregulation of SSTR2.	NF-кB pathway activation, mutations in CDKN2A, CCND1, TP53, JAK/STAT. Upregulation of SSTR2.	Mutations in PIK3CA and mutations in CDKN2A. EBV-CIMP
Somatic Mutations	Loss of chromosomes in 3p, 9p (9p21), 11q (11q13), and 14q Hypermethylation at 6p21.3	9p21.3 deletion and 11q13.3 amplification	Hypermethylation at 6p21.3 Methylation of TSG (APC, PTEN and RASSF1A), cell adhesion molecules (THBS1 and E-cadherin), CDKN2A, MLH1, CXXC4, TIMP2 and PLXND1. Mutations in ARID1A and BCOR