Suvarna Nischitha N, Monappa Vidya
Department of Pathology, Kasturba Medical College Manipal, Manipal Academy of Higher Education, Manipal, Karnataka, India.
Iran J Pathol. 2024 Spring;19(2):250-258. doi: 10.30699/IJP.2024.2018132.3229. Epub 2024 Jan 29.
BACKGROUND & OBJECTIVE: Primary extranodal lymphoma (pENL) is a malignant lymphoid neoplasm that presents with the main bulk of disease at an extranodal site. The incidence of primary pENL has risen sharply in recent years due to the advent of better diagnostic modalities. Diagnosing pENL can be challenging due to its morphological overlap with other tumors native to the site of origin. This study aims to establish the anatomic distribution, clinical presentations, possible etiologic correlations, and histological subtypes of pENL in a tertiary care center located in South India.
This is a retrospective study of 109 patients with pENL (69 males, 40 females, M: F = 1.7:1) over 5 years (October 2012 to September 2017). The tumors were reclassified according to WHO classification of Haematolymphoid tumors, 5th edition, 2022.
pENL constituted 109/481 cases (22.6%) of all NHL cases, with the highest incidence in 7th decade. The gastrointestinal tract (39%) was the predominant site involved, followed by head and neck (26%). Diffuse large B cell lymphoma (DLBCL) was the most common histomorphological variant followed by Follicular lymphoma (FL). The majority of the patients were immunocompetent (89%) and presented with stage IV disease (31.1%) at diagnosis.
This study presents an overview of the diverse distribution patterns of both common and rare pENL within a tertiary care center. The accurate diagnosis of pENL necessitates the elimination of secondary extranodal involvement. It is important to note that the accurate diagnosis of pENL requires careful evaluation and exclusion of other possible causes.
原发性结外淋巴瘤（pENL）是一种恶性淋巴肿瘤，其主要病变位于结外部位。近年来，由于更好的诊断方式的出现，原发性pENL的发病率急剧上升。由于其形态学与起源部位的其他肿瘤重叠，诊断pENL可能具有挑战性。本研究旨在确定位于印度南部的一家三级医疗中心中pENL的解剖分布、临床表现、可能的病因相关性及组织学亚型。
这是一项对109例pENL患者（69例男性，40例女性，男:女 = 1.7:1）进行的为期5年（2012年10月至2017年9月）的回顾性研究。肿瘤根据2022年第5版世界卫生组织血液淋巴肿瘤分类进行重新分类。
pENL占所有非霍奇金淋巴瘤病例的109/481例（22.6%），在第7个十年发病率最高。胃肠道（39%）是主要受累部位，其次是头颈部（26%）。弥漫性大B细胞淋巴瘤（DLBCL）是最常见的组织形态学变异型，其次是滤泡性淋巴瘤（FL）。大多数患者免疫功能正常（89%），诊断时表现为IV期疾病（31.1%）。
本研究概述了三级医疗中心内常见和罕见pENL的不同分布模式。pENL的准确诊断需要排除继发性结外受累。需要注意的是，pENL的准确诊断需要仔细评估并排除其他可能的病因。
