摘要目的 探讨胃肠道套细胞淋巴瘤(mantle cell lymphoma, MCL)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38例胃肠道MCL患者的临床资料,观察肿瘤组织学形态和免疫表型,荧光原位杂交( FISH)技术检测IgH/CCND1融合基因.同时,选取同期60例非胃肠道 MCL进行比较,观察两组患者临床病理学特征和生存率差异.结果38例胃肠道MCL患者中,男性23例,女性15例,中位年龄62岁(范围35~78岁),＞60岁者占55.3% (21/38).病程＜6个月者占81.1%(30/37).起病时主要表现为腹痛、腹泻、纳差和便血.有B症状者占32.4%(12/37).肿瘤最常累及回盲部(57.9%,22/38),其次是直肠(36.8%,14/38)、乙状结肠(28.9%,11/38),胃占18.4%(14/38).内镜下表现为黏膜息肉样病变33例(86.8%,33/38),其中多发者22例(66.7%,22/33).局部隆起型肿物5例(13.2%,5/38). Ann Arbor分期:Ⅰ期3例(7.9%, 3/38),Ⅱ期4例(10.5%,4/38),Ⅳ期31例(81.6%,31/38).肿瘤累及腹腔及腹膜后淋巴结者占45.7%(16/35),累及浅表淋巴结者占41.7%(15/36).合并其他结外侵犯者占17.1%(6/35).脾脏肿大者占23.5%(8/34). 38例均为经典MCL,肿瘤由形态单一的异型淋巴细胞构成,浸润破坏正常黏膜组织结构.所有病例肿瘤细胞表达 CD20 和 CD5,97.4%(37/38)的病例表达 cyclin D1,92% (23/25)的病例表达SOX11. 1例cyclin D1阴性的病例FISH检测结果阳性. 28例(73.7%,28/38)获得随访,中位随访时间25个月(3~79个月).Ⅰ~Ⅱ期和Ⅲ~Ⅳ期患者总生存率差异无统计学意义(3年总生存率80.0%:69.1%,P=0.546).胃肠道MCL与非胃肠道MCL患者总生存率差异无统计学意义(3年总生存率71.7%:72.5%,P=0.935).单因素分析结果显示年龄＞60岁和脾脏肿大对总生存率的影响差异具有统计学意义(P＜0.05).结论 胃肠道MCL患者多以胃肠道症状起病,好发于结直肠,多发息肉样病变多见,年龄＞60岁和伴有脾脏肿大者预后差.预后与非胃肠道MCL患者无差异.

abstractsObjective To investigate the clinicopathologic features and prognosis of gastrointestinal mantle cell lymphoma ( GI-MCL). Methods Clinical data of 38 GI-MCL patients diagnosed at Beijing Friendship Hospital from January 2002 to January 2016 were retrospectively reviewed morphologically and immunophenotypically. IgH/CCND1 gene fusion was assessed by fluorescent in situ hybridization ( FISH). For comparison, 60 cases of non-GI-MCL were randomly selected to extract the differences inclinicopathological features and patient survival between the two groups. Results Of 38 patients with GI-MCL, the median age was 62 years (range: 35-78 years, 23 males and 15 females), of which patients of 60 years of age or older accounted for 55.3%. Patients with clinical course of less than 6 months accounted for 81.1% ( 30/37 ). The main symptoms included abdominal pain, diarrhea, anorexia and hematochezia. Those with B symptoms accounted for 32.4% ( 12/37 ). The tumor most often involved lleocecal region (57.9%,22/38), followed by rectum (36.8%,14/38) and sigmoid colon (28.9%, 11/37), and the stomach accounted for 18.4% ( 14/38 ). Endoscopic polypoid lesions were found in 33 cases (86.8%,33/38), of which 22 cases (66.7%,22/33) were multiple. Five cases (13.2%,5/38) presented with local protuberant neoplasm. According to Ann Arbor staging, 3 cases (7.9%,3/38) were at stage Ⅰ, 4 cases (10.5%,4/38) were at stage Ⅱ, and 31 cases (81.6%,31/38) were at stage Ⅳ. The number of patients with tumor involvement of abdominal and retroperitoneal lymph nodes accounted for 45.7%(16/35), including 41.7%(15/36) involving the superficial lymph node, 17.1%(6/35) involving extranodal sites, and 23.5%(8/34) having splenomegaly. All of the 38 cases were classic MCL, and the tumor was composed of uniform lymphoid cells and effacing normal mucosal structure. All tumors were positive for CD20 and CD5. 97.4% ( 37/38) tumors were positive for cyclin D1, and 92.0% ( 23/25) tumors were positive for SOX11. FISH test was positive in 1 case of cyclin D1 negative tumor. Twenty-eight patients (73.7%) had a median follow-up of 25.0 months (range:3-79 months). The 3-year survival rate for stage Ⅰ-Ⅱ and stage Ⅲ-Ⅳ of patients were 80.0% and 69.1%, respectively (P＞ 0.05). The 3-year survival rate for GI-MCL and non-GI-MCL patients were 71.7% and 72.5%, respectively (P＞0.05).Single factor analysis showed that age of ＞60 years and splenomegaly were correlated with a worse overall survival rate (P＜0.05). Conclusions Gastrointestinal malaise is the most common presenting symptom in GI-MCL patients. GI-MCL more commonly involves colorectum with more frequent multiple polypoid lesions. Patients of age ＞60 years and with splenomegaly have poor prognosis. There is no difference in the prognosis between GI-MCL and non-GI-MCL patients.