扁桃体套细胞淋巴瘤临床病理学特点及预后

Clinicopathologic characteristics and prognositic indicators of tonsillar mantle cell lymphoma

目的探讨扁桃体套细胞淋巴瘤（TMCL）的临床病理学特点及预后。方法回顾性分析2002年1月至2016年1月北京友谊医院诊断的25例TMCL患者的临床资料，光镜观察肿瘤形态，免疫组织化学采用MaxVision染色法，2例采用荧光原位杂交技术（FISH）进行IgH／CCNDl融合基因检测。选取同期40例非扁桃体套细胞淋巴瘤（NTMCL）进行对比，以观察两组患者临床病理学特征和生存率差异。结果TMCL占同期套细胞淋巴瘤的5．6％（25／449）。男女比为5．3：1．0，中位年龄60岁（44．82岁）。主要症状为咽痛及异物感，体检示扁桃体肿大或质硬肿块，早期临床误诊为扁桃体炎18例（72．0％）。伴有淋巴结受累的患者19例（76．0％），脾脏受累4例（16．0％），咽部局灶性累犯11例（44．0％），其他非淋巴器官受累3例（12．O％）。病变扁桃体组织结构破坏，18例经典型和7例母细胞变异型均由形态单一的淋巴样细胞构成。25例肿瘤细胞阳性或强阳性表达CD20、cyclinD1，92．0％（23／25）的病例弱阳性或阳性表达CD5。肿瘤细胞不表达CD3、CD23、CDIO、bcl-6。2例CD5阴性的经典型病例FISH检测IgH／CCNDl融合基因为阳性。18例（72．0％）获得随访，中位随访时间26个月（6—81个月），Ⅰ～Ⅱ期和Ⅲ～Ⅳ期患者总生存率差异无统计学意义（P〉0．05）。与NTMCL比较，TMCL患者就诊时Ⅰ～Ⅱ期比例更高（x2=12．789，P〈0．01），非淋巴器官侵犯比例更低（X2=8．125，P〈0．01），预后更好（X2=4．351，P=0．037）。结论TMCL发病率低，临床易诊为扁桃体炎，就诊时Ⅰ～Ⅱ期患者比例高，较NTMCL预后更好。

Objective To investigate elinicopathologieal features and prognosis of tonsillar mantle cell lymphoma（TMCL）. Methods 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 immunohistoehemieal 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. Results 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, tonsil]ar 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 cyelin 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（ P〉0. 05 ）. Compared with NTMCL, TMCL was found to have higher proportion of stage Ⅰ～Ⅱ disease （X2 = 12. 789, P〈0. 01 ）, lower the proportion of non-lymphatic organ involvement （ X2 = 8. 125, P 〈 0. 01 ）, and better prognosis （X2 = 4. 351, P = 0. 037 ）. Conclusion 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.