Extranodal NK/T-cell Lymphoma, Nasal Type A Report of 73 Cases at MD Anderson Cancer Center
Extranodal NK/T-cell Lymphoma, Nasal Type A Report of 73 Cases at MD Anderson Cancer Center
复制标题
MD 安德森癌症中心结外 NK/T 细胞淋巴瘤，鼻 A 型 73 例报告
DOI：
10.1097/pas.0b013e31826731b5
复制
发表时间：
2013-01-01
影响因子：
5.6
通讯作者：
Yin, C. Cameron
中科院分区：
文献类型：
--
作者：
Li, Shaoying;Feng, Xiaoli;Yin, C. Cameron
结外NK/ t细胞淋巴瘤，鼻型（ENKTL）是罕见的在美国。我们报告了73例ENKTL患者，包括49例男性和24例女性（中位年龄46岁）。鼻/上呼吸道疾病63例；10例鼻外疾病累及皮肤、小肠、会厌、睾丸、肾上腺、肾脏和乳房。68例患者获得了完整的分期数据：44例I/II期，24例IV期。69例中有15例（22%）有淋巴结病，10/63例有骨髓受累。组织学上，67/73（92%）表现为坏死，48/70（69%）表现为血管中心/血管破坏性生长模式。肿瘤细胞分布广泛：中等大小（n = 34），大小混合（n = 21），大（n = 13），小（n = 5）。Epstein-Barr病毒编码的小RNA原位杂交在所有病例中均呈阳性。免疫组织化学研究显示细胞毒性标志物（100%），T-bet (96%), CD2 (96%), CD3 (93%), CD56（90%）和ETS-1（64%）的表达。46%的病例Ki-67为bb0 = 60%。已知有64例患者接受治疗；单纯化疗14例，单纯放疗8例，放化疗联合42例。中位生存期为4.2年，5年总生存期为46%（中位随访3.8年）。鼻外病变、高国际预后指数评分和高增殖率与较差的预后相关。我们的结论是，美国的ENKTL病例与亚洲和其他国家报告的病例相似。没有血管中心/血管破坏模式和存在淋巴结病，这些特征在文献中没有得到重视，但在相当一部分患者中出现。国际预后指数评分、疾病解剖部位和增殖率在该患者队列中具有预后价值。
Extranodal NK/T-cell lymphoma, nasal type (ENKTL) is uncommon in the United States. We report 73 patients with ENKTL, including 49 men and 24 women (median age, 46 y). Sixty-three patients had nasal/upper aerodigestive tract disease; 10 had extranasal disease involving skin, small intestine, epiglottis, testis, adrenal glands, kidney, and breast. Complete staging data were available for 68 patients: 44 stage I/II and 24 stage IV. Fifteen of 69 (22%) had lymphadenopathy and 10/63 had bone marrow involvement. Histologically, 67/73 (92%) showed necrosis, and 48/70 (69%) had an angiocentric/angiodestructive growth pattern. The neoplastic cells showed a wide spectrum: medium sized (n = 34), mixed small and large (n = 21), large (n = 13), and small (n = 5). In situ hybridization for Epstein-Barr virus-encoded small RNA was positive in every case. Immunohistochemical studies showed expression of cytotoxic markers (100%), T-bet (96%), CD2 (96%), CD3 (93%), CD56 (90%), and ETS-1 (64%). Ki-67 was >= 60% in 46% cases. Therapy was known for 64 patients; 14 received only chemotherapy, 8 radiation alone, and 42 received combined radiation and chemotherapy. Median survival was 4.2 years, and 5-year overall survival was 46% (median follow-up, 3.8 y). Extranasal disease, high International Prognostic Index score, and high proliferation rate correlated with poorer prognosis. We conclude that ENKTL cases in the United States are similar to those reported in Asia and other countries. Absence of the angiocentric/angiodestructive pattern and presence of lymphadenopathy, features underemphasized in the literature, occurred in appreciable subsets of patients. The International Prognostic Index score, anatomic site of disease, and proliferation rate had prognostic value in this patient cohort.