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NK/T- or T-cell lymphomas

B-cell lymphomas (mostly DLBCL)

NK/T- or T-cell lymphomas

B-cell lymphomas (mostly DLBCL)

Asian series {420,1837}

71%

29%

18%

82%

Western series {5}

54%

46%

25%

75%

DLBCL = diffuse large B-cell lymphoma

Fig. 1.79 Nasal NK/T cell lymphoma. A In situ hybridization for EBER shows nuclear labeling in practically all lymphoma cells. B The lymphoma cells show immunoreactivity for CD3E. Surface CD3 as detected on frozen section is negative (not shown). C The lymphoma cells show membrane staining for CD56.

Fig. 1.80 Nasal large B cell lymphoma. A Centroblastic morphology, characterized by cells with round nuclei and membrane-bound nucleoli. B This example is dominated by very large pleomorphic cells with prominent nucleoli. C There is diffuse and uniform immunostaining for CD20.

have been reported in extranodal NK/T cell lymphoma of nasal-type, most commonly isochromosome 1q, isochromosome 6p, partial deletion of 6q, and aberration at 11 q {2606,2811}. Comparative genomic hybridization and loss of het-erozygosity studies have suggested frequent DNA loss at 1p, 6q, 11q, 12q, 13q, 17p, whole X, and frequent gain at 1p, 2q, 6p, 10q, 11q, 12q, 13q, 17q, 19p, 20q, Xp {1346,2382,2383}. Overall, the most frequent changes are del(6)(q21-25), del(17)(p12-p13), del(13)(q14-q34) and gain of 1p32-pter {422}. P53 protein overexpression occurs in 45-86% of cases {1496,2104,2105}, but P53 mutation is less common (24-48%) {1496,2105}. TP53mutation has been associated with large cell morphology and advanced stage {2105}. FAS gene mutation is frequently observed {2331,2534}. Aberrant methylation of promoter CpG region of P73 gene occurs in 94% of cases {2381}, and its detection by methylation-specific polymerase chain reaction may be helpful for monitoring residual disease or early relapse {2380}. There are no molecular data on DLBCL specifically occurring in the sinonasal tract.

Genetic susceptibility

Extranodal NK/T cell lymphoma of nasaltype has been reported in both the father and son of a family with known pesticide exposure {1354}.

Prognosis and predictive factors

Radiotherapy and/or systemic chemotherapy is the treatment of choice for localized disease. {420-422,1505, 1550}. Treatment of DLBCL follow protols for similar tumours elsewhere in the body, as some series showed that chemotherapy might be beneficial {1550,2091}. The overall survival for extranodal NK/T cell lymphoma of nasal-type is only 3050% {420-422,1312,1838}. In patients achieving complete remission, local relapse occurs in one-third to one-half of cases {421,1312}, and systemic failure is also common {421}. Factors associated with a worse outcome include: advanced stage, poor performance status, B symptoms, and bulky disease {422}. There is no conclusive evidence to suggest that the histologic grading of NK/T cell lymphoma can predict the clinical outcome. Expression of cutaneous lymphocyte antigen (CLA) may be associated with a worse prognosis, but this finding has yet to be confirmed {2863}. The prognosis is slightly more favourable for DLBCL compared with extranodal NK/T cell lymphoma of nasal-type {420}. The overall survival for DLBCL is 35-60% {420,511,1550}. Prognostic factors have not been studied in detail in sinonasal DLBCL. A Western series reporting treatment results of lymphomas of the nasal cavity and the paranasal sinuses showed that the International Prognostic Index is the only significant predictor for freedom from progression rate {1550}.

Extramedullary plasmacytoma

Definition

A mass-forming lesion of monoclonal plasma cells that occurs outside the bone and bone marrow. By definition, patients with primary extramedullary plasmacytoma (EMP) do not have evidence of underlying multiple myeloma.

ICD-O code 9734/3

Epidemiology

The mean age of patients with EMP of the head and neck is 60 years (range 34-78 years), with a male predominance 4:1 {1267}.

Localization

Most frequent sites of envolvement are nasal cavity, paranasal sinuses and nasopharynx {433,827,1267,1613,1972, 2347,2656,2746}.

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