Clinical features and management of primary retroperitoneal diffuse large B-cell lymphoma
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摘要: 目的:总结原发性腹膜后弥漫大B细胞淋巴瘤(diffused large B-cell lymphoma, DLBCL)的诊治经验。方法:回顾性分析2012年9月—2019年10月我院收治的6例原发性腹膜后DLBCL的临床特征、治疗和预后。其中男4例,女2例;年龄42~73岁,平均62.17岁;左侧3例,右侧1例,双侧2例。5例首诊表现为腰腹痛,1例无症状体检发现。CT或MRI影像学检查提示腹膜后肿瘤。其中CT检查5例,MRI检查1例;单发肿块4例,多发结节样2例;肿瘤最大径5.5~17.8 cm,平均10.98 cm。本组均否认既往淋巴瘤病史,3例术前诊断为腹膜后恶性肿瘤,因穿刺活检出血或肠道损伤风险大,行剖腹探查无法完整切除肿瘤,行腹膜后肿瘤切取活检术,1例术前诊断为左肾上腺皮质癌并左肾侵犯,行左腹膜后肿瘤及左肾切除术,1例诊断为腹膜后恶性肿瘤,行CT引导下腹膜后肿物穿刺活检,1例诊断为腹膜后恶性肿瘤并结肠侵犯,行无痛肠镜活检。5例行静脉化疗,1例行腹膜后病变区域局部放疗。结果:术后或活检病理诊断均为腹膜后DLBCL,Ann Abor分期Ⅱ期4例(2例累及肠道,1例累及肾脏,1例累及输尿管),Ⅳ期2例。IPI评分1分1例,2分3例,4分2例。3例行利妥昔单抗联合环磷酰胺+表柔比星+长春新碱+泼尼松(R-CHOP)方案化疗;2例因经济困难未使用利妥昔单抗,行CHOP方案化疗;1例确诊1个月后因病情进展死亡,未行化疗。术后或活检后随访1~22个月,平均9.83个月。本组4例死于病情进展;2例存活,其中1例放化疗后完全缓解,另1例术后辅助化疗中。结论:原发性腹膜后DLBCL临床罕见,临床表现不典型,确诊需依靠病理检查,手术或穿刺活检是获取病理组织的重要方式。治疗首选R-CHOP方案静脉化疗,本病预后不良。Abstract: Objective: To summarize the clinical experience of primary retroperitoneal diffuse large B-cell lymphoma.Methods: From September 2012 to October 2019, 6 cases of primary retroperitoneal diffuse large B-cell lymphoma treated in our institute were analyzed retrospectively, including 4 males and 2 females. The mean age was 62.17 years old, ranged from 42 to 73. There were 3 cases in left side, 1 in right side and 2 in both sides. Five cases were hospitalized with backache or abdominal pain and 1 had no symptom. Imaging examination showed the retroperitoneal mass with maximum diameter ranged from 5.5 to 17.8 cm, 10.98 cm on average. No patient was found history of lymphoma. Five cases were diagnosed as retroperitoneal tumor and 1 case was diagnosed as left adrenocortical carcinoma preoperatively. Three cases underwent open surgery for biopsy, one received resection of left kidney and retroperitoneal tumor, one was diagnosed by colonoscopy and one by CT guided needle biopsy. Five received chemotherapy and one was taken radiotherapy.Results: Pathological diagnoses of 6 cases were diffuse large B-cell lymphoma with 4 in stage Ⅱ and 2 in stage Ⅳ by Ann Abor standard. The IPI scores were one point in 1, two points in 3 and four points in 2.Two patients underwent CHOP regimen(cyclophosphamide, doxorubicin, vincristine, prednisone), 3 cases received CHOP regimen combined with rituximab and one took no chemotherapy. Follow-up after surgery ranged from 1 to 22 months, 9.83 months on average. Four patients died of disease progression. One showed complete response and survived after chemotherapy followed by radiotherapy. One still received chemotherapy.Conclusion: The primary retroperitoneal diffuse large B-cell lymphoma was rare with no special clinical symptom. Pathology and immunohistochemistry are essential for diagnosis. Surgery and needle biopsy are critical for diagnosis. R-CHOP chemotherapy is recommended but the prognosis is poor.
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Key words:
- retroperitoneal neoplasms /
- lymphoma /
- large B-cell /
- diffuse /
- prognosis
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