Qadri S K, Shah A, Hamdani N H, Baba K M
Department of Pathology, Sher-I-Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India.
Department of Surgery, Sher-I-Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India.
Indian J Cancer. 2016 Apr-Jun;53(2):300-303. doi: 10.4103/0019-509X.197718.
Lymphomas are among the dominant solid tumors in children and primary gastrointestinal lymphomas (PGILs), although rare, are the most common extranodal lymphomas and the most common malignancies affecting the gastrointestinal tract in children. This study was undertaken to analyze childhood PGIL with reference to clinical presentation, anatomic distribution, histopathologic, and immunohistochemical (IHC) characteristics.
In this 12 year combined retrospective and prospective descriptive study, all the cases of PGIL in children were selected according to Dawson's criteria.
A total of 11 cases were found which included 9 boys and 2 girls (male:female - 4.5:1) ranging in age from 1 to 14 years (mean 6.6 years). Abdominal pain (81.8%) and intestinal obstruction (63.6%) were the most common presenting features. Grossly, most of the lesions were ulcero-infiltrative (72.7%) and involved the terminal part of the ileum (36.4%) and ileocecal region (27.3%) most commonly. Histopathologically and IHC, all the cases were high-grade lymphomas of diffuse large B-cell type except for one case of mucosa-associated lymphoid tissue lymphoma. No case of Burkitt's lymphoma was found.
PGILs are an important cause of morbidity and mortality in children worldwide with considerable variation in their clinicopathological features and treatment modalities. Only some studies are available in literature for comparison. Further studies are required to define the genetic and molecular basis of the different histopathological pattern found in our setting.
淋巴瘤是儿童主要的实体瘤之一，原发性胃肠道淋巴瘤（PGIL）虽罕见，但却是最常见的结外淋巴瘤以及儿童胃肠道最常见的恶性肿瘤。本研究旨在分析儿童PGIL的临床表现、解剖分布、组织病理学及免疫组化（IHC）特征。
在这项为期12年的回顾性与前瞻性相结合的描述性研究中，所有儿童PGIL病例均根据道森标准进行选择。
共发现11例病例，包括9名男孩和2名女孩（男:女 = 4.5:1），年龄在1至14岁之间（平均6.6岁）。腹痛（81.8%）和肠梗阻（63.6%）是最常见的临床表现。大体上，大多数病变为溃疡浸润性（72.7%），最常累及回肠末端（36.4%）和回盲部（27.3%）。组织病理学和免疫组化结果显示，除1例黏膜相关淋巴组织淋巴瘤外，所有病例均为弥漫性大B细胞型高级别淋巴瘤。未发现伯基特淋巴瘤病例。
PGIL是全球儿童发病和死亡的重要原因，其临床病理特征和治疗方式存在显著差异。文献中仅有一些研究可供比较。需要进一步研究来明确我们所观察到的不同组织病理学模式的遗传和分子基础。
