Mucosa-associated lymphoid tissue lymphoma of the salivary glands occurring in patients affected by Sjögren's syndrome: report of 6 cases.
OBJECTIVE
Mucosa-associated lymphoid tissue (MALT) lymphoma of the salivary glands occurring in 6 patients affected by primary Sjögren's syndrome is reported.


METHODS
Clinical findings, histologic type, stage, treatment and outcome of the 6 patients have been revised.


RESULTS
In all 6 cases the lymphoma was of the MALT type. Four patients had stage IE disease, 1 patient had stage IIE disease and 1 patient had stage IV disease. The patients received different treatments resulting in all cases in prolonged remission. After 7 years of complete remission 1 patient developed a diffuse large B-cell lymphoma.


CONCLUSIONS
MALT lymphoma of the salivary glands is an indolent disease. Though the best therapy of this lymphoproliferative disorder remains to be established, prolonged remission has been obtained in our cases with different therapeutic approaches. We review the literature regarding the relationship between Sjögren's syndrome and MALT lymphomas and study the mechanisms which may be involved in the transformation from a lymphoepithelial lesion into a neoplastic disorder.
Biasi
D
D
Dipartimento di Medicina Clinica e Sperimentale, Università di Verona, Italia.
Caramaschi
P
P
Ambrosetti
A
A
Carletto
A
A
Mocella
S
S
Randon
M
M
Bambara
L M
LM
D000328:Adult; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012468:Salivary Gland Neoplasms / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D012859:Sjogren's Syndrome / Q000150:complications* / Q000628:therapy; D016896:Treatment Outcome