Titsinides Savvas, Nikitakis Nikolaos, Piperi Evangelia, Sklavounou Alexandra
Department of Oral Medicine and Pathology, School of Dentistry, National and Kapodistrian University of Athens, AthensGreece.
J Oral Maxillofac Res. 2017 Mar 31;8(1):e5. doi: 10.5037/jomr.2017.8105. eCollection 2017 Jan-Mar.
Sjögren's syndrome is a chronic systemic disease, characterized by lymphocytic infiltration and destruction mainly of the salivary and lacrimal glands, resulting in xerostomia and xeropthalmia. Sjögren's syndrome patients have a 44-fold excess risk for the development of non-Hodgkin's lymphoma particularly mucosa-associated lymphoid tissue (MALT) lymphoma, prevalently affecting the major salivary glands. In this report, a rare case of MALT lymphoma of minor salivary glands in a patient with Sjögren's syndrome is described. A review of the published cases of MALT lymphoma located in the minor salivary glands of patients with Sjögren's syndrome is provided.
In a 64-year-old female patient previously diagnosed with Sjögren's syndrome, an asymptomatic soft tissue mass at the palate was noticed, exhibiting rapid enlargement within one month. With a main differential diagnosis of salivary gland neoplasm or lymphoproliferative lesion, a partial biopsy was performed accompanied by proper immunohistochemical analysis.
A final diagnosis of MALT lymphoma was rendered and the patient was referred for further multidisciplinary evaluation. Gastric endoscopy and biopsy revealed a -negative gastric MALT lymphoma, while spleen involvement and bone marrow infiltration were also identified. Patient was classified as having stage IV disseminated disease and a standard chemotherapy protocol was administered; the treatment was well tolerated and resulted in complete remission.
This case emphasizes the need for close monitoring of patients with Sjögren's syndrome by oral medicine specialists, which, besides ensuring proper management of xerostomia and its sequelae, may also lead to early recognition of lymphoma development.
干燥综合征是一种慢性全身性疾病，主要特征为淋巴细胞浸润并破坏唾液腺和泪腺，导致口干和眼干。干燥综合征患者发生非霍奇金淋巴瘤尤其是黏膜相关淋巴组织（MALT）淋巴瘤的风险高出44倍，主要累及大唾液腺。本报告描述了1例干燥综合征患者发生小唾液腺MALT淋巴瘤的罕见病例，并对已发表的干燥综合征患者小唾液腺MALT淋巴瘤病例进行了综述。
在1例先前诊断为干燥综合征的64岁女性患者中，发现腭部有一无症状软组织肿块，1个月内迅速增大。主要鉴别诊断为唾液腺肿瘤或淋巴增殖性病变，进行了部分活检并辅以适当的免疫组化分析。
最终诊断为MALT淋巴瘤，患者被转诊进行进一步的多学科评估。胃镜检查及活检显示胃MALT淋巴瘤阴性，但也发现脾脏受累和骨髓浸润。患者被分类为IV期播散性疾病，给予标准化疗方案；治疗耐受性良好，实现了完全缓解。
本病例强调口腔医学专家对干燥综合征患者进行密切监测的必要性，这除了确保对口干及其后遗症进行适当管理外，还可能有助于早期识别淋巴瘤的发生。
