Department of Pathology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India.
Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India.
J Cancer Res Ther. 2022 Jul-Sep;18(4):1137-1143. doi: 10.4103/jcrt.JCRT_226_20.
Extranodal natural-killer/T-cell lymphoma, nasal type (ENKTL), is a rare, aggressive, predominantly extranodal non-Hodgkin lymphoma (NHL) of putative natural-killer (NK) cell and rarely T-cell origin, always associated with Epstein-Barr virus (EBV) infection and characterized by highly distinctive histopathological features with predilection for the upper aerodigestive tract. While the nasal cavity is the prototypical site, less frequently extranasal ENKTL can also occur. The objective of this case series is to study the immunomorphological features of ENKTL from a tertiary cancer centre as the data are sparse from India despite it being a distinct entity with characteristic clinicopathological features.
We identified 11 cases of ENKTL from the departmental archives between January 2015 and June 2018. The clinicopathological and immunohistochemistry (IHC) findings of these tumors were analyzed. EBV encoded RNA (EBER) in situ hybridization (EBER-ISH) for EBV was done in eight cases.
The disease was more common in males (male: female ratio 1.8:1) with the mean age of 45 years (range 31-65 years). Sinonasal region was the most common site with 9 cases and skin and penis were involved in one case each. The patient with penile involvement on further investigations was found to have occult nasal involvement, Histomorphological features such as angiocentricity/angioinvasion was seen in seven cases (63.6%) and significant necrosis was present in all 11 cases (100%). All cases were uniformly positive for cytoplasmic CD3 and CD56 with high Ki67 proliferating index and EBER-ISH test for EBV was positive in all the eight cases.
ENKTL is an aggressive NHL and should be differentiated from other T- and B-cell lymphomas as the prognosis and therapy differ. Nasal biopsies showing predominant necrosis and atypical lymphoid cells with angiocentricity must raise the suspicion of ENKTL and should be confirmed by immunomorphological and molecular studies.
结外鼻型自然杀伤（NK）/T 细胞淋巴瘤（ENKTL）是一种罕见的侵袭性、主要为结外非霍奇金淋巴瘤（NHL），起源于 NK 细胞，偶尔来源于 T 细胞，始终与 EBV 感染相关，具有高度独特的组织病理学特征，好发于上呼吸消化道。虽然鼻腔是典型部位，但较少见的结外 ENKTL 也可能发生。本病例系列研究的目的是研究来自三级癌症中心的 ENKTL 的免疫形态学特征，因为尽管它是一种具有特征性临床病理特征的独特实体，但来自印度的数据仍然很少。
我们在 2015 年 1 月至 2018 年 6 月期间从部门档案中确定了 11 例 ENKTL 病例。分析了这些肿瘤的临床病理和免疫组织化学（IHC）发现。对 8 例进行了 EBV 编码 RNA（EBER）原位杂交（EBER-ISH）以检测 EBV。
该疾病在男性中更为常见（男：女比例为 1.8:1），平均年龄为 45 岁（范围 31-65 岁）。鼻-鼻窦区是最常见的部位，有 9 例，皮肤和阴茎各有 1 例受累。进一步检查发现阴茎受累的患者有隐匿性鼻内受累。7 例（63.6%）存在血管中心性/血管侵袭性，11 例（100%）均存在显著坏死。所有病例均为 CD3 和 CD56 细胞质阳性，Ki67 增殖指数高，EBER-ISH 检测 EBV 均为阳性。
ENKTL 是一种侵袭性 NHL，应与其他 T 和 B 细胞淋巴瘤区分开来，因为预后和治疗方法不同。鼻活检显示主要坏死和具有血管中心性的非典型淋巴样细胞时，必须怀疑 ENKTL，并通过免疫形态学和分子研究来证实。
