Hou W H, Wei P, Xie J L, Zheng Y Y, Zhang Y L, Zhou X G
Department of Pathology, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China.
Zhonghua Bing Li Xue Za Zhi. 2018 Mar 8;47(3):158-162. doi: 10.3760/cma.j.issn.0529-5807.2018.03.002.
To investigate clinicopathological features and prognosis of tonsillar mantle cell lymphoma(TMCL). Clinical data of 25 patients with TMCL at Beijing Friendship Hospital, Capital Medical University from 2002 to 2016 were included. All the cases were reviewed microscopically. Various immunohistochemical stains were performed using the MaxVision two-step method. IgH/CCND1 gene fusion was detected by fluorescent in situ hybridization(FISH). Additionally, randomly selected 40 cases of non-tonsil MCL of the same period were compared. Among all mantle cell lymphomas (MCL), TMCL accounted for 5.6%(25/449). The median age of the patients was 60 years(range: 44-82 years) with a M∶F ratio of 5.3 to 1.0. The main symptoms were sore throat and foreign body sensation and patients usually presented with enlargement or mass of tonsil. At the early stage of the disease, 18 cases(72.0%) were clinically misdiagnosed as tonsillitis. Lymph node involvement was present in 76.0%(19/25) of the patients. There were 4 cases(16.0%)with current splenic involvement, 11 cases(44.0%) with pharyngeal focal recidivism, and 3 cases(12.0%) with involvement of other non-lymphoid organs. Morphologically, tonsillar architectures were effaced at various degrees. Eighteen MCL cases showed classical type and 7 cases were blastoid variant. All tumors were positive for CD20 and cyclin D1. 92.0%(23/25) tumors showed weakly positive or positive expression for CD5. FISH test that IgH/CCND1 gene fusion was positive in two CD5 negative classical cases. 18 patients(72.0%) had a median follow-up time of 26 months(range: 6-81 months). The difference of survival rate between stage Ⅰ-Ⅱ and stage Ⅲ-Ⅳ patients was not statistically significant(>0.05). Compared with NTMCL, TMCL was found to have higher proportion of stage Ⅰ-Ⅱ disease (χ(2)=12.789, <0.01), lower the proportion of non-lymphatic organ involvement (χ(2)=8.125, <0.01), and better prognosis (χ(2)=4.351, =0.037). The incidence of TMCL is low and prone to be misdiagnosed as tonsillitis. Patients with TMCL are more likely at stage Ⅰ-Ⅱ at presentation and the prognosis is better than that of NTMCL.
探讨扁桃体套细胞淋巴瘤（TMCL）的临床病理特征及预后。纳入首都医科大学附属北京友谊医院2002年至2016年收治的25例TMCL患者的临床资料。所有病例均进行显微镜复查。采用MaxVision两步法进行多种免疫组化染色。采用荧光原位杂交（FISH）检测IgH/CCND1基因融合情况。另外，随机选取同期40例非扁桃体套细胞淋巴瘤（MCL）病例进行对比。在所有套细胞淋巴瘤（MCL）中，TMCL占5.6%（25/449）。患者中位年龄为60岁（范围：44 - 82岁），男女比例为5.3∶1.0。主要症状为咽痛和异物感，患者多表现为扁桃体肿大或肿物。疾病早期，18例（72.0%）临床误诊为扁桃体炎。76.0%（19/25）的患者有淋巴结受累。4例（16.0%）有脾脏受累，11例（44.0%）有咽部局部复发，3例（12.0%）有其他非淋巴器官受累。形态学上，扁桃体结构不同程度被破坏。18例MCL病例表现为经典型，7例为母细胞变异型。所有肿瘤CD20和细胞周期蛋白D1均呈阳性。92.0%（23/25）的肿瘤CD5呈弱阳性或阳性表达。FISH检测发现，2例CD5阴性的经典型病例中IgH/CCND1基因融合呈阳性。18例患者（72.0%）中位随访时间为26个月（范围：6 - 81个月）。Ⅰ - Ⅱ期和Ⅲ - Ⅳ期患者生存率差异无统计学意义（>0.05）。与非扁桃体MCL相比，TMCLⅠ - Ⅱ期疾病比例更高（χ² = 12.789，<0.01），非淋巴器官受累比例更低（χ² = 8.125，<0.01），预后更好（χ² = 4.351，=0.037）。TMCL发病率低，易误诊为扁桃体炎。TMCL患者初诊时多为Ⅰ - Ⅱ期，预后优于非扁桃体MCL。
