EBV-associated diseases of the head and neck

EBV-related disorders of the head and neck are fairly common and can be either benign or malignant. Infectious mononucleosis is the classic EBV infected disorder involving lymph nodes and extranodal lymphoid tissues (i.e., Waldeyer ring including palatine tonsil, lingual tonsil and nasopharyngeal adenoids) of the head and neck.

Extranodal NK/T-cell lymphoma, nasal-type (ENKTL) and EBV positive diffuse large B cell lymphoma of the elder (EBV-associated DLBCL) are not infrequently diagnosed in the head and neck.

Classical Hodgkin lymphoma can involve Waldeyer ring and is EBV positive in approximately 50% of cases.

Less frequently, aggressive NK-cell leukemia, systemic EBV+ T cell lymphoproliferative disorder of childhood, angioimmunoblastic T cell lymphoma, post-transplant lymphoproliferative disorder (PTLD), HV-like lymphoma, and peripheral T cell lymphoma, not otherwise specified can also involve the head and neck and show EBV expression.

EBV can even rarely be found in hepatosplenic T cell lymphoma.

EBV is so common in the differential diagnosis of many T and NK cell neoplasms that it should be a routine part of the work up of these disorders.

Other B cell lymphomas that can have EBV positivity include diffuse large B cell lymphoma with chronic inflammation, plasmablastic lymphoma, and lymphomatoid granulomatosis. Some cases of Burkitt lymphoma are also EBV positive.

The presence of EBV is best determined by in situ hybridization testing for Epstein-Barr virus encoded RNA (EBER).

Immunohistochemistry for EBV related proteins, such as LMP1, is much less sensitive than EBER in situ hybridization but can help determine latency patterns of EBV infection.

Polymerase chain reaction studies can be used to detect EBV; however, they are non-specific and may be positive from bystander EBV positive B cells.

EBV-related lymphoproliferative processes occur in the head and neck ranging from reactive processes to high grade malignant lymphomas.

Both T and B cell lymphomas can be associated with EBV and evidence shows that an individual’s response to the acute EBV infection may be critical in the development of subsequent lymphoma.

Currently, in situ hybridization for EBER is the most sensitive available test to detect EBV and should be routinely performed in lymphoproliferative lesions of the head and neck. Although relatively rare, primary EBV-related lymphomas must be considered in the differential of atypical lymphoid proliferations in the head and neck.

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