Clinical features and management of non-gastrointestinal non-ocular extranodal mucosa associated lymphoid tissue (ENMALT) marginal zone lymphomas

Extranodal mucosa associated lymphoid tissue (ENMALT) marginal zone lymphomas may arise at any site of the body. The most frequent localizations other than gastrointestinal and eye are salivary gland, skin, lung and thyroid. These lymphomas usually arise in a setting of inflammation due to a persistent infection or autoimmune diseases such as Sjogren syndrome in salivary MALT lymphomas and Hashimoto's thryroiditis in thyroid lymphomas. They affect middle-aged patients with a female predominance when lymphoma arises in certain locations. Patients often present with localised stage I or II although disseminated disease may be present at diagnosis or relapse in a third of the cases. Biopsy of the affected site is mandatory to establish the diagnosis and a full work-up staging is recommended. The clinical course is indolent and prognosis is good despite that recurrences following response to therapy are frequent. Surgery, radiotherapy and/or Rituximab based regimens are effective in these lymphomas.