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Presented by Matthew F. Georgy, M.D. and prepared by Jeffrey T. Schowinsky, M.D.
Case 3: A 28-year old man presented with a destructive nasal lesion.
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1. Question
Week 289: Case 3
A 28-year old man presented with a destructive nasal lesion.images/110606MG3a.jpg
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images/110606MG3d.jpgCorrect
Answer: Extranodal NK/T cell lymphoma, nasal type
Histology: There is a dense submucosal infiltrate that comprises a population of highly pleomorphic, medium to large lymphoid cells. Mitotic figures are relatively abundant. Necrosis is not identified in these sections. The neoplastic cells are positive for CD56 and Epstein-Barr virus (EBER).
Discussion: NK/T cell lymphoma, nasal type refers to a distinct group of aggressive lymphoid neoplasms. The majority of these tumors originate in the upper aerodigestive tract where they show extensive infiltration and destruction of adjacent tissues. The classic histologic findings are angiocentric growth and prominent necrosis. Before the neoplastic nature of this process was known, the term “lethal midline granuloma” was used. It is now clear that the majority of these tumors have a true NK-cell phenotype, although a subset of cases are of T cell origin. Nearly all cases are reactive for EBV by in situ hybridization. The majority of cases are also positive for CD56 and cytoplasmic CD3 by immunohistochemistry, although the latter represents cross-reactivity with cytoplasmic CD3-epsilon and does not indicate a T cell phenotype.
Incorrect
Answer: Extranodal NK/T cell lymphoma, nasal type
Histology: There is a dense submucosal infiltrate that comprises a population of highly pleomorphic, medium to large lymphoid cells. Mitotic figures are relatively abundant. Necrosis is not identified in these sections. The neoplastic cells are positive for CD56 and Epstein-Barr virus (EBER).
Discussion: NK/T cell lymphoma, nasal type refers to a distinct group of aggressive lymphoid neoplasms. The majority of these tumors originate in the upper aerodigestive tract where they show extensive infiltration and destruction of adjacent tissues. The classic histologic findings are angiocentric growth and prominent necrosis. Before the neoplastic nature of this process was known, the term “lethal midline granuloma” was used. It is now clear that the majority of these tumors have a true NK-cell phenotype, although a subset of cases are of T cell origin. Nearly all cases are reactive for EBV by in situ hybridization. The majority of cases are also positive for CD56 and cytoplasmic CD3 by immunohistochemistry, although the latter represents cross-reactivity with cytoplasmic CD3-epsilon and does not indicate a T cell phenotype.
