Xia Fan, Ling Wenwu, Zhang Jing, Zhang Yifan, Ma Xuelei
Department of Biotherapy, Cancer Center, State Key Laboratory of Biotherapy Department of Neurosurgery Department of Radiology Department of Ophthalmology, West China Hospital, Sichuan University, Chengdu, China.
Medicine (Baltimore). 2018 Sep;97(37):e12198. doi: 10.1097/MD.0000000000012198.
Primary poorly differentiated lacrimal gland adenocarcinoma in the orbital region is an extremely rare type of neoplasm with only 1 related case in the literature. Its high grade of malignancy makes the timely data reported necessary. Hence, we present an extremely rare disease with biopsy results and recommendations on clinical treatment in an elderly male with Chinese descent.
A 66-year-old Chinese man presented with swelling in the left ocular region and eyeball proptosis. On physical examination, the patient had redness, tenderness, and swelling of the left eye. A surgical incision was noted on the left orbital region. Left eye movements were restricted.
Immunohistochemical examination revealed pan-cytokeratin (PCK, +), p63 (partial, +), cytokeratin 7 (CK7, +), cytokeratin 14 (CK14, +), epithelial membrane antigen (EMA, +), protein expressed by erythroblast transformation-specific related gene (ERG, -), S-100 (, -), Epstein-Barr virus-encoded small RNA (EBER, -), smooth muscle actin (SMA, -), and Ki-67 (with a proliferation index approximately 40%). After carefully reviewed the manifestations, imaging findings, and immunohistochemical evidences, a diagnosis of poorly differentiated adenocarcinoma of lacrimal gland was made.
Based on the gene sequencing results, we started the patient with an intensive PF chemotherapy including a combination of cisplatine, fluorouracil, and epirubicin. Two months later, radiotherapy was introduced to the therapy regimen.
The patient responded well to the treatment without severe adverse events. MRI scan showed remarkable remission.
This rare case report will help raise the awareness of high grade lacrimal gland cancer, and subsequently aid the diagnosis in future cases. Positive immunohistochemical markers of CK7, CK14, EMA, p63, and high proliferation index of Ki-67 can help establishing a diagnosis, and cisplatine-fluorouracil program is proved feasible. We share the difficulties we have encountered, hoping to improve patient care in the future.
眼眶区域原发性低分化泪腺腺癌是一种极其罕见的肿瘤类型，文献中仅有1例相关病例报道。其高度恶性使得及时报告相关数据很有必要。因此，我们报告了1例极为罕见的病例，介绍了1名老年华裔男性患者的活检结果及临床治疗建议。
一名66岁华裔男性，因左眼区域肿胀和眼球突出就诊。体格检查发现，患者左眼发红、压痛且肿胀。左侧眼眶区域可见手术切口。左眼活动受限。
免疫组化检查显示，全细胞角蛋白（PCK，阳性）、p63（部分阳性）、细胞角蛋白7（CK7，阳性）、细胞角蛋白14（CK14，阳性）、上皮膜抗原（EMA，阳性）、成红细胞转化特异性相关基因表达蛋白（ERG，阴性）、S-100（阴性）、爱泼斯坦-巴尔病毒编码小RNA（EBER，阴性）、平滑肌肌动蛋白（SMA，阴性）、Ki-67（增殖指数约为40%）。仔细审查临床表现、影像学检查结果及免疫组化证据后，诊断为低分化泪腺腺癌。
根据基因测序结果，我们对患者采用了强化PF化疗方案，包括顺铂、氟尿嘧啶和表柔比星联合使用。两个月后，放疗被纳入治疗方案。
患者对治疗反应良好，未出现严重不良事件。磁共振成像扫描显示病情明显缓解。
这份罕见病例报告将有助于提高对高级别泪腺癌症的认识，并有助于未来病例的诊断。CK7、CK14、EMA、p63免疫组化阳性标志物及Ki-67高增殖指数有助于确诊，顺铂-氟尿嘧啶方案被证明是可行的。我们分享了遇到的困难，希望未来能改善患者护理。
