McKelvie Penelope A, Climent Fina, Krings Gregor, Hasserjian Robert P, Abramson Jeremy S, Pilch Ben Z, Harris Nancy Lee, Ferry Judith A, Zukerberg Lawrence R, Sohani Aliyah R
The James Homer Wright Pathology Laboratories of the Massachusetts General Hospital and Department of Pathology, Harvard Medical School, Boston, MA, USA.
Department of Anatomical Pathology, St Vincent's Hospital, Melbourne, Australia.
Histopathology. 2016 Oct;69(4):667-79. doi: 10.1111/his.12990. Epub 2016 Jun 21.
Extranodal NK/T cell lymphoma, nasal type (ENKTCL) is usually composed of medium- to large-sized lymphoid cells showing prominent angiotrophism and tumour cell necrosis. We report 13 cases composed predominantly of small lymphocytes diagnosed in the United States and Western Europe.
Patients included seven females and six males aged 17-75 years. Ten presented with sinonasal and three with buccal disease. Nine had stage IE/IIE and four had stage IV disease. In five of seven patients with multiple biopsies at different time-intervals, the lymphoma was misinterpreted as representing chronic inflammation on an earlier biopsy. In all cases morphology showed a dense infiltrate of small lymphoid cells with minimal cytological atypia. Necrosis, angioinvasion and angiodestruction were each seen in 17%, 22% and 17% of biopsies. Median Ki67 was 5%. Four patients died of lymphoma 4-16 months after diagnosis, including three of four patients with stage IV disease; seven (54%) are alive with no evidence of disease at a median of 39 months; one patient with stage IV disease is alive at 10 months and one recurred at 17 months.
In sinonasal biopsies with predominantly small lymphocytic infiltrates with admixed chronic inflammation, focal hypercellularity, focal surface ulceration or microscopic bone invasion by small lymphoid cells should alert pathologists to the possibility of small-cell predominant ENKTCL. Awareness of the full histological spectrum of ENKTCL, particularly in non-endemic areas, is important in avoiding a delay in diagnosis and ensuring timely initiation of therapy.
鼻型结外NK/T细胞淋巴瘤（ENKTCL）通常由中等至大细胞大小的淋巴样细胞组成，具有显著的血管侵袭性和肿瘤细胞坏死。我们报告了在美国和西欧诊断的13例主要由小淋巴细胞组成的病例。
患者包括7名女性和6名男性，年龄在17 - 75岁之间。10例表现为鼻窦疾病，3例表现为颊部疾病。9例为IE/IIE期，4例为IV期。在7例不同时间间隔进行多次活检的患者中，有5例在早期活检时淋巴瘤被误诊为慢性炎症。所有病例的形态学表现为小淋巴样细胞密集浸润，细胞学异型性极小。活检标本中坏死、血管侵犯和血管破坏的发生率分别为17%、22%和17%。Ki67中位数为5%。4例患者在诊断后4 - 16个月死于淋巴瘤，其中包括4例IV期患者中的3例；7例（54%）存活，在中位39个月时无疾病证据；1例IV期患者在10个月时存活，1例在17个月时复发。
在鼻窦活检中，若主要为小淋巴细胞浸润并伴有慢性炎症、局灶性细胞增多、局灶性表面溃疡或小淋巴样细胞的显微镜下骨侵犯，病理学家应警惕小细胞为主型ENKTCL的可能性。认识ENKTCL的完整组织学谱，特别是在非流行地区，对于避免诊断延迟和确保及时开始治疗很重要。
