Extranodal NK-/T-Cell Lymphoma, Nasal Type

NK/T cell lymphoma, nasal type is an aggressive lymphoma commonly presenting as a destructive process of the facial midline, often demonstrating clinical and histologic features of necrosis and angioinvasion.  It was initially categorized as an angiocentric lymphoma in the Revised European-American Lymphoma (REAL) classification.  It has been recategorized as extranodal NK/T cell lymphoma in the WHO classification of lymphoid neoplasms. 

Historically these tumors were considered part of “midline lethal granuloma.” This entity frequently arises in extranodal regions: nasal or paranasal sinus. In addition, other extranodel sites include palate, trachea, skin and, the gastrointestinal tract. In most instances, EBV genomes are detectable in the tumor cells and immunohistochemistry detects CD56 positivity.

The pathologic diagnosis of nasal-type NK/T cell lymphoma is based on the following criteria: expression of cytoplasmic CD3, CD56 and positivity for EBV in situ hybridization. If EBV in situ hybridization is negative, the immunophenotype studies should demonstrate cytoplasmic CD3 expression and positive cytotoxic molecules such as TIA-1.

Nasal-type NK/T cell lymphoma is known to be one of the most aggressive lymphomas, so it is imperative to offer an appropriately aggressive treatment at an early stage of disease.

We present the clinical and therapeutic treatment course and outcome of a 56 year-old caucasian male diagnosed with NK/T cell lymphoma.  This includes a clinicopathologic review, approaches to diagnosis.  and surgical and prosthetic treatment strategies for patient optimization throughout oncologic therapies.

References: 

R. Liang, “Advances in the management and monitoring of extranodal NK/T-cell lymphoma, nasal type,” British Journal of Haematology, vol. 147, no. 1, pp. 13–21, 2009.

Yamaguchi, M., Suzuki, R., Kwong, Y.-L., Kim, W. S., Hasegawa, Y., Izutsu, K., Suzumiya, J., Okamura, T., Nakamura, S., Kawa, K. and Oshimi, K. (2008), Phase I study of dexamethasone, methotrexate, ifosfamide, l-asparaginase, and etoposide (SMILE) chemotherapy for advanced-stage, relapsed or refractory extranodal natural killer (NK)/T-cell lymphoma and leukemia. Cancer Science, 99: 1016–1020. doi: 10.1111/j.1349-7006.2008.00768.x