Objective: To analyze the clinicopathologic features of chronic sclerosing submaxillaritis (CSS).
Methods: The clinical and pathological characteristics of 9 CSS were analyzed.
Results: In the 9 patients, there were 6 males and 3 females. The age of patients ranged from 51 - 77 years old. All of the tumors were located in the submandibular gland, presenting with painless and firm mass. Histologically, a well-defined mass lesion with extensive lymphocytes and plasma cells infiltration, preservation of lobular architecture, with acinar atrophy. The reactive hyperplasia of lymphoid follicles may be found in CSS. The phlebitis and obliterating phlebitis also formed. Immunohistochemistry showed evidence of diffuse infiltration of plasma cells. The mean number of IgG4-positive plasma cell per high-power field (HPF) was 186, mean value of the IgG4:IgG ratio was 0.71. Three of these 9 cases had manifestations of IgG4-associated systemic disease.
Conclusions: CSS is considered as a part of IgG4-related sclerosing diseases, recognition of which is very essential for a successful treatment. When diagnosis is made, it is necessary to ascertain whether lesion occurs within salivary gland only or in combination with outside IgG4-related sclerosing disease. The establishment of follow-up is also necessary. Some patients show good response to steroid therapy.