Cassidy Dermot T, McKelvie Penny, Harris Gerald J, Rose Geoffrey E, McNab Alan A
Orbital, Plastic and Lacrimal Clinic, Royal Victorian Eye and Ear Hospital, Melbourne, Australia.
Orbit. 2005 Dec;24(4):257-63. doi: 10.1080/01676830590922084.
To describe, for the first time, the occurrence of a large cyst in the orbital lobe of the lacrimal gland in association with marginal zone B-cell MALT lymphoma in three female patients, two of whom also had primary Sjögren's syndrome.
Retrospective case note review.
Three female patients, aged 35, 41 and 47 years, presented with progressive proptosis caused by an enlarging cyst within the orbital lobe of the lacrimal gland. Two of the patients had primary Sjögren's syndrome. Excision of the cyst and part of the lacrimal gland revealed marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type within the gland or wall of the cyst in all cases. The cysts were partially lined by ductular epithelium. One patient had systemic lymphoma, was treated with chemotherapy and remains in remission one year later; the other two had disease apparently confined to the lacrimal gland and are disease-free at 9 months and at three years after local irradiation, respectively.
To our knowledge, these are the first reported cases of cysts affecting the orbital lobe of the lacrimal gland in patients with B-cell MALT lymphoma of the gland. Although cystic changes have been described in other exocrine glands, lung and thymus in patients with Sjögren's syndrome, this association has also not been described in the lacrimal gland before.
首次描述3例女性患者泪腺眶叶出现大囊肿并伴有边缘区B细胞黏膜相关淋巴组织（MALT）淋巴瘤，其中2例还患有原发性干燥综合征。
回顾性病例记录 review。
3例女性患者，年龄分别为35岁、41岁和47岁，因泪腺眶叶内囊肿增大导致进行性眼球突出。其中2例患者患有原发性干燥综合征。切除囊肿及部分泪腺后，所有病例均显示囊肿内或囊肿壁存在黏膜相关淋巴组织（MALT）型边缘区B细胞淋巴瘤。囊肿部分内衬导管上皮。1例患者患有全身性淋巴瘤，接受化疗，1年后仍处于缓解期；另外2例患者的疾病显然局限于泪腺，分别在局部放疗后9个月和3年无疾病。
据我们所知，这些是首次报道的泪腺B细胞MALT淋巴瘤患者中影响泪腺眶叶的囊肿病例。虽然在干燥综合征患者的其他外分泌腺（如肺和胸腺）中已描述过囊性改变，但泪腺中的这种关联以前也未被描述过。
