Gaal K, Sun N C, Hernandez A M, Arber D A
Department of Pathology at City of Hope National Medical Center, Duarte, California 91010, USA.
Am J Surg Pathol. 2000 Nov;24(11):1511-7. doi: 10.1097/00000478-200011000-00006.
Sinonasal natural killer (NK)/T-cell lymphomas are common in Asia and areas of South and Central America but are rarely seen in the United States, where they have not been as well characterized. Fifteen cases diagnosed in Southern California were studied with respect to histologic features, immunophenotype, Epstein-Barr virus EBER in-situ hybridization (EBV EBER-ISH), and T-cell receptor gamma chain (TCR-gamma) gene rearrangement. Although ethnic background was available for only seven patients, six were of Asian or Hispanic descent with only one non-Hispanic white known. Twelve presented as sinonasal lesions, but three were limited to the oropharynx. Most cases (11 of 15) demonstrated both necrosis and an angiodestructive pattern. All cases demonstrated cytoplasmic CD3 positivity (15 of 15), and were positive for both TIA-1 and granzyme B (14 of 14). Perforin was positive in 5 of 14. CD56 was expressed in 10 of 15 and CD8 in 3 of 15. EBV EBER-ISH was positive in 14 of 14 and TCR-gamma gene rearrangement was detected in 1 of 14 cases. None (0 of 14) were positive for CD16 or CD57. Although CD16-positive histiocytes were abundant, double-label EBER-ISH/IHC failed to identify CD16 expression on EBV-positive tumor cells. Three cases with pleomorphic large cell morphology showed focal CD30 positivity, raising the differential diagnosis of anaplastic large cell lymphoma, but all were ALK-1-negative and otherwise similar to the other cases of NK/T-cell lymphoma. Sinonasal NK/T-cell lymphomas in the United States most often occur in ethnic groups from areas of reported high frequency (Asia, Central and South America), although less commonly than in endemic populations, and are otherwise similar phenotypically. A combined approach, including immunohistochemistry, EBV EBER-ISH, and TCR gene rearrangement studies, is most helpful to arrive at the correct diagnosis.
鼻腔鼻窦自然杀伤（NK）/T细胞淋巴瘤在亚洲以及南美洲和中美洲地区较为常见，但在美国却很少见，在美国其特征尚未得到充分描述。对在南加州诊断出的15例病例进行了组织学特征、免疫表型、爱泼斯坦-巴尔病毒EBER原位杂交（EBV EBER-ISH）以及T细胞受体γ链（TCR-γ）基因重排方面的研究。尽管仅7例患者有族裔背景信息，其中6例为亚洲或西班牙裔血统，仅1例为非西班牙裔白人。12例表现为鼻腔鼻窦病变，但3例局限于口咽。大多数病例（15例中的11例）表现出坏死和血管破坏模式。所有病例均显示细胞质CD3阳性（15例中的15例），且TIA-1和颗粒酶B均为阳性（14例中的14例）。穿孔素在14例中的5例呈阳性。CD56在15例中的10例表达，CD8在15例中的3例表达。EBV EBER-ISH在14例中的14例呈阳性，TCR-γ基因重排在14例中的1例被检测到。14例中无一例（0例）CD16或CD57呈阳性。尽管CD16阳性组织细胞丰富，但双重标记的EBER-ISH/免疫组化未能在EBV阳性肿瘤细胞上识别出CD16表达。3例具有多形性大细胞形态的病例显示局灶性CD30阳性，这增加了间变性大细胞淋巴瘤的鉴别诊断难度，但所有病例ALK-1均为阴性，且在其他方面与其他NK/T细胞淋巴瘤病例相似。在美国，鼻腔鼻窦NK/T细胞淋巴瘤最常发生于来自报告高发地区（亚洲、中美洲和南美洲）的族裔群体，尽管其发生率低于流行地区人群，且在表型上其他方面相似。包括免疫组化、EBV EBER-ISH和TCR基因重排研究在内的联合方法对于做出正确诊断最有帮助。
