结肠滤泡树突状细胞肉瘤伴多发转移临床病理分析

Clinicopathological Analysis of Colon Follicular Dentritic Cell Sarcomawith Metastasis

目的探讨结肠滤泡树突状细胞肉瘤(FDCS)的临床及病理特点。方法回顾性分析我院收治的1例结肠FDCS伴多发转移患者的临床资料。患者,女,62岁。因"左中上腹部肿物伴疼痛2 a,加重5 d"于2015年9月8日入院。体检示左侧腹部可触及一大小约10 cm×18 cm肿块,质硬,活动度差,轻压痛。实验室检查无明显异常。CT平扫示左中上腹部见一大小约7.6 cm×9.5 cm团块状不规则形软组织密度影,边界不清。临床诊断为左侧腹腔占位。结果经术前准备于2015年9月10日在气管插管全身麻醉下行剖腹探查术。术中所见:无腹水,盆腔及腹腔、肝脏、肠管表面未见结节样转移,肿物位于左上腹,上至肾上极水平,外侧与侧腹膜粘连,内侧侵犯空肠起始部系膜与屈氏韧带起始部及空肠起始部粘连,下至脐水平以下2 cm;大小为15 cm×13 cm×8 cm,不规则突起,肿物质硬,部分囊实性突起,活动差。遂行左半结肠切除术+腹腔巨大肿物切除术+肠粘连松解术。术后病理示:结肠符合滤泡树突状细胞肉瘤/肿瘤,肠系膜结节及大网膜结节肿瘤浸润/转移。2015年10月21日患者术后于我院行"环磷酰胺+长春新碱+依托泊苷+醋酸泼尼松+表柔比星"方案化疗1周期,之后患者病情稳定,随访至2015年11月15日未见复发。结论结肠FDCS较少见,临床特点并不特异,主要诊断依据病理特点及免疫组化特异性标志。治疗以手术切除肿瘤为主,必要时可辅以放、化疗,但远期生存获益并不理想,对其治疗方法还需不断探索。

Objective To investigate the clinical and pathological features of colonfollicular dentritic cell sarcoma （FDCS）. Methods The clinical data of a patient with colon FDCS were retrospectively analyzed. The patient, female, 62-year-old was admitted tothe hospital on September 8,2015for the complaint that there was a mass with pain for 2 years in midsection and the pain got worse in recently 5 days. A 10 cm × 8 cm in diameter, hard,poor activityTumor was found inleft abdomen by physical examination. Abdominal tenderness was positive and laboratoryexaminations were es- sentially normal. CT scan showed a 7.6 cm× 9.5 cm in diameter, irregular, soft tissue density mass with noclearmargin in the left midsection. The clinical diagnosis was the space oceupyinglesion of left abdomen. Results After an active preoperative preparation, the patient underwent exploratory laparotomy underendotracheal general anesthesia on September 10,2015. No ascites and no nodular metastaseswas found in pelvic cavity, abdominal cavity,liver and the surface of bowel. The tumor measuring 15 cm ×13 cm ×8cm in diameter was found in the left midsection,up to the level of the renalupper pole, adhered with the side peritoneum on the outside ,infringed the mesentery of initial part of jejunum and adhered with the initial part of Treitz and jejunum on the inside, down to the level ot2 em lower than umbilical. The pa- tient accepted the Left hemicoleetomy ＋ resection of abdominal large neoplasm ＋ enterolysis. Postoperative pathologi- cal examinationindicatedthat the tumor in colon was follicular dentritic cell sarcoma/tumor, with obviousmetastasis in mesenteric nodes and omentum nodules. The patient accepted 1 cycle of chemotherapy using＂ cyclophosphamide ＋ vin- cristine ＋ etoposide ＋ prednisone acetate ＋ epirubicin＂ in our hospital on October 21,2015. The patient was cur- rently in stable condition and noreeurrenee was observed till November 15,2015. Conclusion Colon FDCS is a very rare disease and Clinical characteristic is not specific. The diagnosis relies on the results of pathological charcteristie and Imnmnohistochemical examination. Surgical resection of tumor is the main therapy for FDCSand the radiation and chemotherapy can be combined when necessary, but the long-term survival is poor, so we need to explore the therapy of FDCS continuously.