摘要：

目的 回顾分析1例以海绵窦综合征为首发症状的间变性大细胞淋巴瘤患者的临床病理学特点。方法与结果 女性患者，35岁。头面部疼痛1个月、视物成双7天、右上睑下垂4天；头部MRI 显示右侧海绵窦区异常信号影，考虑垂体腺瘤或海绵窦综合征可能。激素治疗无效后改行手术部分切除病灶。组织学形态可见肿瘤细胞呈弥漫浸润性生长、异型性明显，胞质丰富、粉染，胞核呈圆形、卵圆形、不规则形，部分胞核呈肾形。免疫组织化学染色肿瘤细胞胞膜表达CD30和CD56，胞膜和胞质表达上皮膜抗原，不表达间变性淋巴瘤激酶，Ki-67抗原标记指数达80%。术后一般情况较差，无法耐受放射治疗和药物化疗，术后3周死亡。结论 临床表现为海绵窦综合征的患者应警惕间变性大细胞淋巴瘤的可能，必要时行组织病理学检查，诊断过程中需注意与未分化型非角化性鼻咽癌、生殖细胞瘤、浆母细胞性淋巴瘤、恶性黑色素瘤和经典型霍奇金淋巴瘤等相鉴别。

关键词:  淋巴瘤, 大细胞, 间变性, 海绵窦, 免疫组织化学, 原位杂交

Abstract:

Objective A retrospective analysis of the clinical and pathological features of an anaplastic large cell lymphoma (ALCL) with cavernous sinus syndrome as its first symptom. Methods and Results A 35-year-old woman had head and facial pain for a month, diplopia for 7 d, right eyelid drooped for 4 d. Head MRI showed abnormal signals in right cavernous sinus, and pituitary adenoma or carvernous sinus syndrome were considered. Partial tumor resection was performed after hormone therapy was failed. Histological manifestations revealed that the tumor cells were diffuse and heterogeneous with abundant and pink stained cytoplasm, round, oval, irregular or kidney-shaped nuclei. Immunohistochemical staining showed that CD30 and CD56 were positive on the membrane, and epithelial membrane antigen (EMA) was positive on membrane and cytoplasm, and anaplastic lymphoma kinase (ALK) was negative. Ki-67 labeling index was 80%. Unfortunately, this patient died 3 weeks later after the operation, because of severe illness and intolerance to radiotherapy and chemotherapy. Conclusions The diagnosis of ALCL should be considered when cavernous sinus syndrome as the clinical symptom, and pathological examination should be performed if necessary. It should be differentiated from some tumors, such as undifferentiated non-keratinizing nasopharyngeal carcinoma, germinoma, plasmablastic lymphoma, malignant melanoma and Hodgkin's lymphoma (HL).

Key words:  Lymphoma, large-cell, anaplastic, Cavernous sinus, Immunohistochemistry, In situ hybridization