Department of Pathology & Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN, USA.
Centro Clinico de Cabeza Y Cuello, Guatemala City, Guatemala.
Head Neck Pathol. 2020 Dec;14(4):1001-1012. doi: 10.1007/s12105-020-01172-w. Epub 2020 May 27.
Lymphoepithelial carcinoma of salivary glands (LECSG) are rare neoplasms, reported in endemic populations (southeastern Chinese) with a strong Epstein-Barr virus (EBV) association. A retrospective series comparing EBV status within an ethnically diverse population (endemic vs. non-endemic patients) has not been reported. Sixteen LECSG were equally distributed between males (n = 8) and females (n = 8) with a median age of 54 years (range 18 to 85 years) at initial diagnosis. Ten patients were white, 4 Asian, and 2 black. The patients typically presented with swelling or mass for an average of 11.6 months. Tumors affected only major salivary glands: parotid (n = 13); submandibular (n = 3). Tumors were an average of 2.9 cm (range 1.5 to 5.8 cm). Nine of 16 (56%) patients had cervical lymph node metastases at presentation. No patients had nasopharyngeal or oropharyngeal tumors. Microscopically, the tumors were widely infiltrative, characterized by large polygonal to spindled cells arranged in a syncytial, lattice-like network in a background of lymphoplasmacytic cells. The neoplastic cells showed an open-vesicular nuclear chromatin to a more basaloid-morphology, the latter showing hyperchromatic nuclei and less cytoplasm, while nearly all of the cases had associated lymphoepithelial lesions/sialadenitis. By in situ hybridization, 8 of 16 cases had a strong, diffuse EBER expression (4 of 4 Asians; 4 of 12 non-Asians), while with immunohistochemistry all cases tested were pan-cytokeratin, CK5/6 and p63 reactive; none of the cases tested were p16 reactive. All patients were managed with wide or radical excision, 4 with concurrent chemoradiation, and 6 with radiation alone. Distant metastasis (lung, brain, and bone) developed in 2 patients. Overall follow-up (mean 3.8 years) revealed 12 patients alive and 2 dead, none with evidence of disease (mean 4.3 years); one white male alive with disease at 1.9 years, and one Asian female dead of disease at 4.2 years; both of these latter patients had Group IV stage disease. High stage (Group IV) patients had a shorter mean survival than lower stage patients: 3.1 versus 4.8 years, respectively. In conclusion, LECSG are uncommon primary neoplasms. Concurrent lymphoepithelial lesions may help suggest a primary tumor. The tumors, irrespective of race or ethnicity, may express EBER. There is an overall good survival, perhaps better for EBV-negative patients and for those with lower stage disease.
涎腺淋巴上皮癌（LECSG）是罕见的肿瘤，仅见于地方性流行人群（中国东南部），与 EBV 强烈相关。在种族多样化的人群中（地方性与非地方性患者）比较 EBV 状态的回顾性系列研究尚未报道。16 例 LECSG 中男性（n=8）和女性（n=8）各占一半，中位年龄为 54 岁（18-85 岁）。10 例患者为白人，4 例为亚洲人，2 例为黑人。患者通常表现为肿胀或肿块，平均持续 11.6 个月。肿瘤仅影响大唾液腺：腮腺（n=13）；下颌下腺（n=3）。肿瘤平均为 2.9cm（1.5-5.8cm）。16 例患者中有 9 例（56%）在初诊时出现颈部淋巴结转移。无患者有鼻咽或口咽肿瘤。显微镜下，肿瘤广泛浸润，特征为大的多边形至梭形细胞，呈合胞体样，晶格样网络排列，背景为淋巴浆细胞。肿瘤细胞表现为开放的泡状核染色质，更具基底样形态，后者表现为染色质深染的核和较少的细胞质，而几乎所有病例均伴有淋巴上皮病变/唾液腺炎。通过原位杂交，16 例中有 8 例（4 例亚洲人；4 例非亚洲人）出现强烈、弥漫的 EBER 表达，而所有病例的免疫组化均为广谱细胞角蛋白、CK5/6 和 p63 阳性；无 p16 阳性病例。所有患者均行广泛或根治性切除术，4 例患者行同期放化疗，6 例患者仅行放疗。2 例患者发生远处转移（肺、脑和骨）。总随访（平均 3.8 年）显示 12 例患者存活，2 例患者死亡，均无疾病证据（平均 4.3 年）；1 例白人男性在 1.9 年时疾病仍存活，1 例亚洲女性在 4.2 年时因疾病死亡；这 2 例患者均为 IV 期疾病。高分期（IV 期）患者的平均生存时间短于低分期患者：分别为 3.1 年和 4.8 年。总之，LECSG 是罕见的原发性肿瘤。伴发的淋巴上皮病变可能有助于提示原发性肿瘤。无论种族或民族如何，肿瘤都可能表达 EBER。总体生存率较好，可能 EBV 阴性患者和低分期患者的预后更好。
