Polymorphic extranodal lymphoma, expressing NK or rarely cytotoxic T cell phenotype
Alternate/Historical Names
Polymorphic reticulosis
Lethal midline granuloma
Angiocentric T cell lymphoma
Diagnostic Criteria
Primarily extranodal
Most common site is nose
Nodes may be involved secondarily
Necrosis nearly always present
Vascular destruction may be seen
Ulceration common
Pseudoepitheliomatous hyperplasia may be seen
Wide range of appearance from small to large cell
Frequently mixed
Inflammatory cells may be prominent
Cytoplasm may be clear with azurophilic granules on Giemsa stained cytologic preparations
CD56 positive
Rare cases may lack CD56, but must then be EBV+, CD3+ and cytotoxic protein+
EBV positive
May be lacking in some extranasal, North American or Western European cases
Note that the term "nasal type" originally applied only to lymphomas of this type that occurred in sites other than the nasal cavity; it now includes nasal and extra-nasal locations
Dita Gratzinger MD PhD
Yasodha Natkunam MD PhD
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
Initial posting : September 1, 2007
Supplemental studies
Immunohistology and Flow
NK type
CD56
100%
EBV LMP
Positive, less sensitive than in situ hybridization
Warnke RA, Weiss LM, Chan JKC, Cleary ML, Dorfman RF . Tumors of the Lymph Nodes and Spleen, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 14, 1995
Jaffe ES, Harris NL Stein H, Vardiman JW eds. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues, World Health Organization Classification of Tumours 2001
Chan JK, Sin VC, Wong KF, Ng CS, Tsang WY, Chan CH, Cheung MM, Lau WH. Nonnasal lymphoma expressing the natural killer cell marker CD56: a clinicopathologic study of 49 cases of an uncommon aggressive neoplasm. Blood. 1997 Jun 15;89(12):4501-13.
Blakolmer K, Vesely M, Kummer JA, Jurecka W, Mannhalter C, Chott A. Immunoreactivity of B-cell markers (CD79a, L26) in rare cases of extranodal cytotoxic peripheral T- (NK/T-) cell lymphomas. Mod Pathol 2000 Jul;13(7):766-72
Cheung MM, Chan JK, Lau WH, Foo W, Chan PT, Ng CS, Ngan RK. Primary non-Hodgkin's lymphoma of the nose and nasopharynx: clinical features, tumor immunophenotype, and treatment outcome in 113 patients. J Clin Oncol 1998 Jan;16(1):70-7
Chan JK, Sin VC, Wong KF, Ng CS, Tsang WY, Chan CH, Cheung MM, Lau WH. Nonnasal lymphoma expressing the natural killer cell marker CD56: a clinicopathologic study of 49 cases of an uncommon aggressive neoplasm. Blood 1997 Jun 15;89(12):4501-13
Kwong YL, Chan AC, Liang R, Chiang AK, Chim CS, Chan TK, Todd D, Ho FC. CD56+ NK lymphomas: clinicopathological features and prognosis. Br J Haematol 1997 Jun;97(4):821-9
Jaffe ES, Chan JK, Su IJ, Frizzera G, Mori S, Feller AC, Ho FC. Report of the Workshop on Nasal and Related Extranodal Angiocentric T/Natural Killer Cell Lymphomas. Definitions, differential diagnosis, and epidemiology. Am J Surg Pathol 1996 Jan;20(1):103-11
Nakamura S, Suchi T, Koshikawa T, Kitoh K, Koike K, Komatsu H, Iida S, Kagami Y, Ogura M, Katoh E, et al. Clinicopathologic study of CD56 (NCAM)-positive angiocentric lymphoma occurring in sites other than the upper and lower respiratory tract. Am J Surg Pathol 1995 Mar;19(3):284-96
Wong KF, Chan JK, Ng CS, Lee KC, Tsang WY, Cheung MM. CD56 (NKH1)-positive hematolymphoid malignancies: an aggressive neoplasm featuring frequent cutaneous/mucosal involvement, cytoplasmic azurophilic granules, and angiocentricity. Hum Pathol 1992 Jul;23(7):798-804
Teruya-Feldstein J, Jaffe ES, Burd PR, Kanegane H, Kingma DW, Wilson WH, Longo DL, Tosato G. The role of Mig, the monokine induced by interferon-gamma, and IP-10, the interferon-gamma-inducible protein-10, in tissue necrosis and vascular damage associated with Epstein-Barr virus-positive lymphoproliferative disease. Blood 1997 Nov 15;90(10):4099-105
Liang R, Todd D, Chan TK, Chiu E, Lie A, Kwong YL, Choy D, Ho FC. Treatment outcome and prognostic factors for primary nasal lymphoma. J Clin Oncol 1995 Mar;13(3):666-70
Quintanilla-Martinez L, Franklin JL, Guerrero I, Krenacs L, Naresh KN, Rama-Rao C, Bhatia K, Raffeld M, Magrath IT. Histological and immunophenotypic profile of nasal NK/T cell lymphomas from Peru : high prevalence of p53 overexpression. Hum Pathol 1999 Jul;30(7):849-55
van de Rijn M, Bhargava V, Molina-Kirsch H, Carlos-Bregni R, Warnke RA, Cleary ML, Kamel OW. Extranodal head and neck lymphomas in Guatemala : high frequency of Epstein-Barr virus-associated sinonasal lymphomas. Hum Pathol 1997 Jul;28(7):834-9
Elenitoba-Johnson KS, Zarate-Osorno A, Meneses A, Krenacs L, Kingma DW, Raffeld M, Jaffe ES. Cytotoxic granular protein expression, Epstein-Barr virus strain type, and latent membrane protein-1 oncogene deletions in nasal T-lymphocyte/natural killer cell lymphomas from Mexico . Mod Pathol 1998 Aug;11(8):754-61
Ho FC, Choy D, Loke SL, Kung IT, Fu KH, Liang R, Todd D, Khoo RK. Polymorphic reticulosis and conventional lymphomas of the nose and upper aerodigestive tract: a clinicopathologic study of 70 cases, and immunophenotypic studies of 16 cases. Hum Pathol 1990 Oct;21(10):1041-50
Arber DA, Weiss LM, Albujar PF, Chen YY, Jaffe ES. Nasal lymphomas in Peru . High incidence of T-cell immunophenotype and Epstein-Barr virus infection. Am J Surg Pathol 1993 Apr;17(4):392-9
Jaffe ES, Krenacs L, Kumar S, Kingma DW, Raffeld M. Extranodal peripheral T-cell and NK-cell neoplasms. Am J Clin Pathol 1999 Jan;111(1 Suppl 1):S46-55
Cuadra-Garcia I, Proulx GM, Wu CL, Wang CC, Pilch BZ, Harris NL, Ferry JA. Sinonasal lymphoma: a clinicopathologic analysis of 58 cases from the Massachusetts General Hospital . Am J Surg Pathol 1999 Nov;23(11):1356-69
Shipley WR, Hammer RD , Lennington WJ, Macon WR. Paraffin immunohistochemical detection of CD56, a useful marker for neural cell adhesion molecule (NCAM), in normal and neoplastic fixed tissues. Appl Immunohistochem 1997;5:87-93
Hasserjian RP, Harris NL. NK-cell lymphomas and leukemias: a spectrum of tumors with variable
manifestations and immunophenotype. Am J Clin Pathol. 2007 Jun;127(6):860-8
