Mucosa-associated lymphoid tissue lymphomas usually present clinically as parotid enlargement, sometimes bilateral [217]. There is often a history of Sjogren's syndrome, but not always. Cases have been reported in post-transplant patients [101]. General lymphadenopa-thy and bone marrow involvement are unusual in MALT lymphoma, and such a presentation favours a diagnosis of nodal lymphoma.

The histopathology of MALT lymphoma is intimately linked with that of LESA from which it develops - the risk of lymphoma in LESA has been estimated at approximately 4-7% [91]. Histological criteria alone cannot identify exactly when a clonal B-cell population emerges in LESA, and in practice the process can be considered not so much a sharp change from one (benign) entity to another (malignant) one, but rather as a spectrum of lymphoma gradually evolving from a purely inflammatory process (Table 5.5) [165]. It can be considered that MALT lymphoma begins as an antigen-driven lymphoid proliferation that progresses first to monoclonality and then, with the acquisition of secondary genetic changes, to MALT lymphoma [105].

The microscopic picture evolves with time [105]; the earliest morphologically recognisable feature of malignancy is the proliferation of marginal zone B-cells to form a distinct halo-like zone around the LELs of LESA. As the lymphoma evolves in a background of LESA marginal zones B cells expand, displace and then replace the follicles. Alternatively, they may colonise the germinal centres assuming a follicular-like architecture (pseudo-follicles) [106]. In addition, there may be foci of sclerosis, and infiltration by epithelioid histiocytes, which can form small granulomas.

Mucosa-associated lymphoid tissue lymphoma restricted to the salivary glands is a relatively indolent disease that is often curable with local treatment. Prognosis remains favourable even in the presence of other extranodal sites, including bone marrow [108].

Other primary non-MALT extranodal salivary lymphomas are rare, and are mainly T-cell neoplasms [29].

Nodal non-Hodgkin's lymphomas can involve the intra-salivary and adjacent lymph nodes, and they should be classified using the appropriate scheme [104, 108].

5.15 Other Tumours

A variety of soft tissue and other non-salivary neoplasms may rarely present as tumours of the salivary glands. These include solitary fibrous tumour, granular cell tumour, follicular dendritic cell sarcoma, inflammatory pseudotumour (inflammatory myofibroblastic tumour), primary malignant melanoma, primitive neu-roectodermal tumour (PNET) and teratoma.

5.16 Unclassified Tumours

The revised WHO classification defines this group as benign or malignant tumours that cannot be placed in any of the categories [171]. This designation may be unavoidable if only a small quantity of tissue is available for study.


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