Liu Yao, Xing Hui, Liu Yue-Ping
Department of Pathology, The Fourth Hospital of Hebei Medical University, Shijiazhuang 050000, Hebei Province, China.
Department ofPathology, The Fourth Hospital of Hebei Medical University, Shijiazhuang 050000, Hebei Province, China.
World J Clin Cases. 2021 Nov 6;9(31):9542-9548. doi: 10.12998/wjcc.v9.i31.9542.
Pediatric-type follicular lymphoma (PTFL) is a unique pathological type in the 4 edition of hematopoiesis and lymphoid tissue tumor classification revised by World Health Organization. It is unique in clinical practice and seldom seen in adult. PTFL mainly occurs in the head and neck lymph nodes. Most of the cases are short of fever, night sweat, weight loss, and other B symptoms which substitute for lymphadenopathy as the main symptom. PTFL can be disposed of surgical resection and it can achieve long-term tumor-free survival, and it has an excellent outcome.
Two cases of PTFL were reported and their clinicopathological features, differential diagnosis, therapy and prognosis were discussed. PTFL showed gray-brown tough texture in general performance. The histological manifestations of PTFL were similar to that of adult-follicular lymphoma (FL). Under low power microscope, the structure of lymph nodes was destroyed in different degree, the follicles were closely arranged, expanded and irregular, and the mantle zone became thin or disappeared. In addition, the "starry sky phenomenon" could be seen. At high magnification, the follicles were mainly composed of single medium-sized central cells, and some of them mainly consisted of centroblastic cells to characterize scattered chromatin and inconspicuous nucleoli. Immunohistochemical showed the tumor cells expressed CD20, PAX5, CD79a and CD10, BCL6, FOXP-1, which were limited in germinal center; Ki-67 was highly expressed in germinal center. CD21 and CD23 showed nodular and expanded follicular dendritic cells. Immunoglobulin gene rearrangement was positive for IGH and IGK. The two patients underwent surgical resection with no complications. After discharge, the two patients with a close review for 18 mo and 5 mo respectively and showed no evidence of recurrence.
PTFL in adult is generally supposed to be extremely rare. PTFL displayed characteristic morphological, immunophenotypic, and molecular biological changes which are a kind of neoplasm with satisfactory prognosis after surgical excision.
儿童型滤泡性淋巴瘤（PTFL）是世界卫生组织修订的第4版造血与淋巴组织肿瘤分类中的一种独特病理类型。它在临床实践中较为独特，在成人中很少见。PTFL主要发生于头颈部淋巴结。大多数病例缺乏发热、盗汗、体重减轻等B症状，以淋巴结肿大为主要症状。PTFL可行手术切除，能实现长期无瘤生存，预后良好。
报告2例PTFL，并对其临床病理特征、鉴别诊断、治疗及预后进行讨论。PTFL大体表现为灰棕色质地坚韧。PTFL的组织学表现与成人滤泡性淋巴瘤（FL）相似。低倍镜下，淋巴结结构不同程度破坏，滤泡紧密排列、扩大且不规则，套区变薄或消失。此外，可见“星空现象”。高倍镜下，滤泡主要由单一中等大小的中心细胞组成，部分主要由中心母细胞组成，染色质分散，核仁不明显。免疫组化显示肿瘤细胞表达CD20、PAX5、CD79a、CD10、BCL6、FOXP-1，局限于生发中心；Ki-67在生发中心高表达。CD21和CD23显示结节状且扩大的滤泡树突状细胞。免疫球蛋白基因重排IGH和IGK阳性。2例患者均行手术切除，无并发症。出院后，2例患者分别密切随访18个月和5个月，均无复发迹象。
成人PTFL一般认为极为罕见。PTFL表现出特征性的形态、免疫表型和分子生物学改变，是一种手术切除后预后良好的肿瘤。
