Salivary Type Low Grade Adenocarcinomas

Fig. 2.17. Sinonasal NK/T lymphoma. a Angiocentric infiltrate of atypical lymphocytes with extensive necrotic areas. b Strongly positive CD56 immunoreaction v . r.

Fig. 2.17. Sinonasal NK/T lymphoma. a Angiocentric infiltrate of atypical lymphocytes with extensive necrotic areas. b Strongly positive CD56 immunoreaction inflammatory cells. Pseudoepitheliomatous hyperplasia of the covering epithelium may occur [49]. NK/T cell lymphoma is almost always associated with EBV posi-tivity. The most typical immunophenotypes are CD2+, CD56+ (Fig. 2.17b), surface CD3-, and cytoplasmic CD3epsilon+. Most cases are also positive for cytotoxic granule-associated proteins (granzyme B, TIA-1 and perforin). They are usually negative for other T and NK-cell associated markers. Sinonasal lymphomas demonstrating CD3epsilon+, CD56-, cytotoxic molecule+, and EBV+ are also included within the NK/T category. The commonest cytogenetic abnormality found in NK/T-cell lymphoma is the 6q-22-23 deletion [233]. The prognosis of nasal NK/T-cell lymphoma is variable. Some patients respond well to therapy and others die of disease despite aggressive therapy [52].

Sinonasal malignant lymphomas of either B-cell or T-cell derivation need a careful differential diagnosis with other small round cell tumours (Table 2.2) and with ex-tramedullary plasmacytoma [6, 46], as well as with ex-tramedullary tumours composed of myeloid or lymphoid blasts [133].

ICD-O:9731/3

Plasmacytoma of the sinonasal tract appears as a diffuse infiltration of mature plasma cells of the mucosa; occasionally, tumour cells are less differentiated, and diagnosis may be difficult on a histologic basis [2, 6, 46, 185]. Immunohistochemical staining for CD138 and k and X chains may be helpful. Full examination of the patient is required to exclude disseminated disease.

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