Sun Ke, Wang Li-jun, Yao Hong-tian, Xiang Hua, Ding Wei
Department of Pathology, The First Affiliated Hospital, College of Medicine, Zhejiang Univerisity, Hangzhou 310003, China.
Zhonghua Kou Qiang Yi Xue Za Zhi. 2012 Mar;47(3):153-6. doi: 10.3760/cma.j.issn.1002-0098.2012.03.006.
To analyze the clinicopathologic features of chronic sclerosing submaxillaritis (CSS).
The clinical and pathological characteristics of 9 CSS were analyzed.
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.
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.
分析慢性硬化性颌下腺炎（CSS）的临床病理特征。
分析9例CSS的临床及病理特点。
9例患者中，男性6例，女性3例。患者年龄51 - 77岁。所有肿瘤均位于颌下腺，表现为无痛性肿块且质地硬。组织学上，肿块界限清楚，有大量淋巴细胞和浆细胞浸润，小叶结构保留，但腺泡萎缩。CSS中可见淋巴滤泡反应性增生，还形成了静脉炎和闭塞性静脉炎。免疫组化显示浆细胞弥漫浸润。每高倍视野（HPF）IgG4阳性浆细胞的平均数为186，IgG4:IgG比值的平均值为0.71。这9例中有3例有IgG4相关系统性疾病的表现。
CSS被认为是IgG4相关硬化性疾病的一部分，认识到这一点对成功治疗至关重要。诊断时，有必要确定病变是否仅发生在唾液腺内或是否合并有其他IgG4相关硬化性疾病。建立随访也很有必要。一些患者对类固醇治疗反应良好。
