36例良、恶性淋巴上皮病临床病理分析

目的：分析恶性淋巴上皮病（MLEL）、良性淋巴上皮病（BLEL）及与之相联系的黏膜相关淋巴组织型结外边缘区B细胞淋巴瘤（MALToma）的临床特点、病理学特征、发病机制、诊断、治疗及转归。方法：对13例MLEL、20例BLEL及3例MT进行免疫组化染色和HE染色观察，复习相关临床资料并随访。结果：MLEL的病理学特征为大量增生的淋巴组织中见成簇或条索状分布的肿瘤细胞，界限不清，核分裂像多见；免疫组化示CKpan全部阳性（13／13），Vim部分阳性（3／13），SMA部分阳性（2／13）；8例MLEL可随访资料中，术后1例死于复发，1例死于其他疾病，1例局部复发，5例未见复发或转移，随访3．5个月-4a。BLEL的病理学特点为腺实质萎缩，间质淋巴细胞浸润及形态温和的腺肌上皮岛；免疫组化示CKpan19例阳性，LCA17例阳性。UCHL-1、L2616例阳性。CK10 10例阳性．S-1002例阳性；12例可随访的BIEL中，2例术后复发诊断为MLEL，其余健在，随访3个月～6a不等。3例MT中。1例术后6个月复发，经再次手术并化疗后缓解；免疫组化L26、LCA、CD79、CD43均阳性；UCHL-1、CKpan、EMA均有2例阳性。结论：MLEL好发于腮腺，且女性多见，来源于唾液腺导管上皮。对已发生颈淋巴结转移的患者行原发灶一颈联合根治，术后放疗，少数MLEL可在BLEL基础上发生，故BLEL局部切除后应长期随访；MT为B细胞淋巴瘤，手术切除辅以适当化疗可获较好疗效。术中冷冻切片是本病目前最可靠的术中诊断手段。

Clinical and pathological analysis of 36 patients with benign and malignant lymphoepithelial lesions

PURPOSE: To analyze the clinical manifestation, diagnosis, treatment and prognosis of the patients with malignant lymphoepithelial lesion (MLEL), benign lymphoepithelial lesion (BLEL) and mucosa-associated lymphoid tissue lymphoma (MALToma), as well as their pathological characteristics and pathogenesis. METHODS: Thirteen patients with MLEL, 20 patients with BLEL, and 3 patients with MALToma were retrospectively reviewed with their clinical data, pathological data (hematoxylin and eosin stain, and immunohistochemistrical stain), and follow-up data. RESULTS: MLEL was characterized pathologically by clumps or strips of tumor cells with unclear margin and frequent nuclear mitosis among the large amounts of proliferated lymph tissues. Immunohistochemistrical results showed that all 13 cases were positive for CKpan, 3 out of 13 cases were positive for Vim, 2 out of 13 cases were positive for SMA; among the 8 patients who had been followed up for 3.5 months to 4 years, 1 patient died of local tumor recurrence, 1 patient died of other disease, 1 patient was alive with local tumor recurrence one year after primary surgery, and 5 patients were alive without tumor recurrence or metastasis. BLEL was characterized pathologically by atrophy of glandular substance, interstitial lymphocytes infiltration, and mild-shaped glandular myoepithelial islands. Immunohistochemistrical results showed that 19 cases were positive for CKpan, 17 cases were positive for LCA, 16 cases were positive for UCHL-1 and L26, 10 cases were positive for CK10, and 2 cases were positive for S-100; among the 12 patients who had been followed up for 3 months to 6 years, 1 patient died of other disease, 2 patients were alive with local tumor recurrence which was confirmed pathologically as MLEL after secondary surgical resection of the recurrent tumors, and the other patients were alive without tumor recurrence. Immunohistochemistrical results for MALToma showed that all 3 cases were positive for L26, LCA, CD79, and CD43, 2 cases were positive for either UCHL-1, or CKpan, or EMA; all 3 patients were still alive for at least 2 years, 1 patient had local tumor recurrence 6 months after primary surgery plus postoperative radiotherapy and then treated successfully by second surgery plus chemotherapy. CONCLUSION: From this study, MLEL is most often located in the parotid gland and more common in females than in males, it originates from salivary duct epithelium. Combined radical resection of primary lesion and neck dissection followed by postoperative radiotherapy is suggested for patients with MLEL and cervical lymph node metastasis. Local resection and close follow-up is suggested for patients with BLEL because of its potential transformation to MLEL. Surgical resection and proper postoperative chemotherapy is suggested for patients with MALToma, which is a subtype of B cell type lymphoma. Intraoperative frozen section is a reliable diagnostic method for these kinds of diseases. Supported by Shanghai Leading Academic Discipline Project(Grant No.Y0203).