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原发性泪腺导管腺癌伴多发淋巴结转移1例OA

Primary lacrimal duct adenocarcinoma with multiple lymph node metastases:A case report

中文摘要英文摘要

原发性泪腺导管腺癌(primary lacrimal duct adenocarcinoma,PLDC)是一种罕见的高度恶性泪腺上皮性肿瘤,约占所有泪腺上皮性肿瘤的2%,且该肿瘤容易发生淋巴结转移,预后较差.2024年9月,临沂市中心医院收治了1例以颈部淋巴结肿大为首发症状的75岁男性PLDC患者.影像学检查结果显示,左眼眶外上象限有最大径为3.1 cm的肿物,伴左侧颈部多发淋巴结转移.行左眼眶肿物切除术.病理学检查结果显示,肿瘤细胞呈腺样、筛状及实性排列,伴粉刺样坏死.免疫组织化学检查结果显示,人表皮生长因子受体2(human epidermal growth factor receptor 2,HER-2)为强阳性(3+),细胞角蛋白7(cytokeratin 7,CK7)、广谱细胞角蛋白(pan-cytokeratin,CKpan)、上皮膜抗原(epithelial membrane antigen,EMA)、GATA结合蛋白3(GATA-binding protein 3,GATA-3)和特异性囊肿病液体蛋白15(gross cystic disease fluid protein 15,GCDFP15)均为阳性,约40%的肿瘤细胞阳性表达雄激素受体(androgen receptor,AR),转录抑制因子 GATA 结合蛋白 1(transcriptional repressor GATA binding 1,TRPS1)、雌激素受体(estrogen receptor,ER)、孕激素受体(progesterone receptor,PR)、S-100、性别决定区Y框10(SRY-box transcription factor 10,SOX-10)、甲状腺转录因子-1(thyroid transcription factor-1,TTF-1)均为阴性.病理诊断为PLDC伴多发淋巴结转移.确诊后行白蛋白结合型紫杉醇+顺铂化疗5个周期,随访13个月未见复发.PLDC需与乳腺及肺部转移癌鉴别.TRPS1阴性有助于排除乳腺癌,AR阳性可能为潜在治疗靶点.通过对PLDC的临床特征、病理组织学形态、免疫表型特点的系统分析,为该肿瘤的早期诊断提供了影像学及病理学依据.同时,随访结果提示治疗方案有效,为进一步优化治疗方案、改善患者预后提供了临床参考数据.

Primary lacrimal duct adenocarcinoma(PLDC)is a rare and highly malignant epithelial tumor of the lacrimal gland,accounting for approximately 2%of all epithelial lacrimal gland tumors.This neoplasm has a strong propensity for lymph node metastasis and is generally associated with poor prognosis.In September 2024,a 75-year-old man presenting with cervical lymphadenopathy as the initial manifestation was admitted to Linyi Central Hospital and was subsequently diagnosed with PLDC.Imaging examination revealed a 3.1-cm mass located in the superolateral quadrant of the left orbit,accompanied by multiple metastatic lymph nodes in the left cervical region.The patient underwent surgical resection of the left orbital mass.Histopathological examination demonstrated that the tumor cells were arranged in glandular,cribriform,and solid patterns,accompanied by comedo-like necrosis.Immunohistochemical analysis showed strong positivity(3+)for human epidermal growth factor receptor 2(HER-2).Positive staining was observed for cytokeratin 7(CK7),pan-cytokeratin(CKpan),epithelial membrane antigen(EMA),GATA-binding protein 3(GATA-3),and gross cystic disease fluid protein 15(GCDFP15).Androgen receptor(AR)expression was observed in approximately 40%of tumor cells.The tumor was negative for transcriptional repressor GATA-binding protein 1(TRPS1),estrogen receptor(ER),progesterone receptor(PR),S-100,SRY-box transcription factor 10(SOX-10),and thyroid transcription factor-1(TTF-1).The pathological diagnosis was PLDC with multiple lymph node metastases.After confirmation of the diagnosis,the patient received 5 cycles of chemotherapy with albumin-bound paclitaxel plus cisplatin.No recurrence was observed during 13 months of follow-up.PLDC should be distinguished from metastatic breast and lung carcinomas.Negative TRPS1 expression is helpful for excluding breast carcinoma,whereas AR positivity may represent a potential therapeutic target.A systematic analysis of the clinical features,histopathological morphology,and immunophenotypic characteristics of PLDC provides imaging and pathological evidence for early diagnosis of this rare tumor.Moreover,the follow-up findings suggest potential therapeutic efficacy and provide valuable clinical evidence for optimizing therapeutic strategies and improving patient outcomes.

王波;叶涛;任永昌;张玉娜;朱德强;徐为为

临沂市中心医院病理科,临沂 276000临沂市中心医院病理科,临沂 276000临沂市中心医院病理科,临沂 276000临沂市中心医院病理科,临沂 276000临沂市中心医院病理科,临沂 276000临沂市中心医院呼吸与危重症医学科,临沂 276000

医药卫生

原发性泪腺导管腺癌淋巴结转移病理诊断鉴别诊断雄激素受体

primary lacrimal duct adenocarcinomalymph node metastasispathological diagnosisdifferential diagnosisandrogen receptor

《临床与病理杂志》 2026 (4)

657-661,5

10.11817/j.issn.2095-6959.2026.250800

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