原发性胆总管非霍奇金淋巴瘤1 例并文献复习
| 作者: |
1施显茂,
1吴飞翔,
1陈洁,
1叶甲舟,
1白涛,
2吴圣明,
1黎乐群
1 广西医科大学附属肿瘤医院肝胆外科，广西 南宁 530021 2 广西医科大学附属肿瘤医院临床病理科，广西 南宁 530021 |
| 通讯: |
黎乐群
Email: lilequngx@163.com |
| DOI: | 10.3978/.2018.08.002 |
| 基金: | 国家科技重大专项基金资助项目（2012ZX10002010001009）；广西科学研究与技术开发基金资助项目（桂科攻14124003-4）；广西青年自然科学基金资助项目（2016GXNSFBA380091）；广西壮族自治区教育厅课题基金资助项目（2017KY0094）。 |
摘要
目的：探讨原发性胆总管非霍奇金淋巴瘤（NHL）的临床病理特征、诊治及预后。
方法：报道广西医科大学附属肿瘤医院收治的 1 例原发性胆总管 NHL 患者，并结合 1982—2017 年间国内外文献报道的 34 例原发性胆总管 NHL 患者资料进行分析。
结果：该患者为男性，81 岁，行胰十二指肠切除术后病理结果证实原发性胆总管 NHL（弥漫性大 B 细胞淋巴瘤），术后未行全身化疗及局部放疗等辅助抗肿瘤治疗，4 个月后复查未见肿瘤复发。该例加上报道的 34 例患者中男 21 例（60.0%），女 14 例（40.0%）；发病年龄 4~81 岁，中位年龄 57 岁；临床表现有黄疸（88.6%）和腹痛（40.0%），体表淋巴结肿大（11.4%），伴发热（11.4%）；部分影像学表现为胆总管占位或者胆总管壁增厚狭窄；4 例行全身化疗，10 例行手术切除，16 例行手术切除联合全身化疗，1 例行手术切除联合局部放疗，3 例行手术切除联合全身化疗及局部放疗，1 例治疗方案未知。大部分患者治疗后预后较好，有 1 例生存期超过 72 个月；单纯手术切除患者与单独化疗或其他联合治疗患者的生存期差异无统计学意义（均 P>0.05）。
结论：原发性胆总管 NHL 为临床罕见疾病，缺乏典型的临床表现，实验室及影像学检查无明显特异性，故其临床诊断困难，确诊依赖于术后病理学与免疫组织化学，选择合适的治疗方案是延长患者生存期的关键。
关键词：
胆管肿瘤 / 诊断；胆管肿瘤 / 治疗；淋巴瘤，非霍奇金；预后
方法：报道广西医科大学附属肿瘤医院收治的 1 例原发性胆总管 NHL 患者，并结合 1982—2017 年间国内外文献报道的 34 例原发性胆总管 NHL 患者资料进行分析。
结果：该患者为男性，81 岁，行胰十二指肠切除术后病理结果证实原发性胆总管 NHL（弥漫性大 B 细胞淋巴瘤），术后未行全身化疗及局部放疗等辅助抗肿瘤治疗，4 个月后复查未见肿瘤复发。该例加上报道的 34 例患者中男 21 例（60.0%），女 14 例（40.0%）；发病年龄 4~81 岁，中位年龄 57 岁；临床表现有黄疸（88.6%）和腹痛（40.0%），体表淋巴结肿大（11.4%），伴发热（11.4%）；部分影像学表现为胆总管占位或者胆总管壁增厚狭窄；4 例行全身化疗，10 例行手术切除，16 例行手术切除联合全身化疗，1 例行手术切除联合局部放疗，3 例行手术切除联合全身化疗及局部放疗，1 例治疗方案未知。大部分患者治疗后预后较好，有 1 例生存期超过 72 个月；单纯手术切除患者与单独化疗或其他联合治疗患者的生存期差异无统计学意义（均 P>0.05）。
结论：原发性胆总管 NHL 为临床罕见疾病，缺乏典型的临床表现，实验室及影像学检查无明显特异性，故其临床诊断困难，确诊依赖于术后病理学与免疫组织化学，选择合适的治疗方案是延长患者生存期的关键。
Primary non-Hodgkin’s lymphoma of the common bile duct: a report of one case and literature review
CorrespondingAuthor:LI Lequn Email: lilequngx@163.com
Abstract
Objective: To investigate the clinicopathologic features, diagnosis and treatment as well as prognosis of primary non-Hodgkin’s lymphoma (NHL) of the common bile duct.
Methods: The data of a patient with primary NHL of the common bile duct treated in the Affiliated Tumor Hospital of Guangxi Medical University, and together with the data of 34 cases of primary NHL of the common bile duct reported from 1982 to 2017 at home and abroad were analyzed.
Results: The patient was an 81-year-old male, who was diagnosed as primary NHL (diffuse large B cell lymphoma) of the common bile duct by postoperative pathological findings after undergoing pancreatoduodenectomy. No adjuvant anti-tumor therapy such as systemic chemotherapy and regional radiotherapy was performed after operation, and no recurrence was noted 4 months later. Of this patient plus the reported 34 patients, 21 cases (60.0%) were males and 14 cases (40.0%) were females; the age at onset ranged from 4 to 81 years, with a median age of 57 years; the clinical manifestations included jaundice (88.6%) and abdominal pain (40.0%), enlarged superficial lymph nodes (11.4%) and concomitant fever (11.4%); imaging manifestations in some cases showed space occupying lesion in the common bile duct or thickening and stenosis of the common bile duct wall; four cases underwent systemic chemotherapy, 10 cases underwent surgical resection, 16 cases underwent surgical resection combined with systemic chemotherapy, 1 case underwent surgical resection combined with local radiotherapy, 3 cases underwent surgical resection combined with systemic chemotherapy and local radiotherapy, and treatment regimen in 1 case was unknown. The majority of cases had a passable prognosis after treatment, and one case survived more than 72 months; there were no significant differences in survival times between patients undergoing surgical resection alone and those undergoing chemotherapy alone or other combined treatments (all P>0.05).
Conclusion: Primary NHL of the common bile duct is a rare condition in clinical practice, lacks typical clinical manifestations, and the laboratory tests and imaging examinations have no obvious specificity. So, its clinical diagnosis is difficult, and definite diagnosis depends on postoperative pathology and immunohistochemistry. Selection of appropriate treatment options is critical for improving the survival of the patients.
Keywords：
Bile Duct Neoplasms/diag; Bile Duct Neoplasms/ther; Lymphoma
Non-Hodgkin; Prognosis
Methods: The data of a patient with primary NHL of the common bile duct treated in the Affiliated Tumor Hospital of Guangxi Medical University, and together with the data of 34 cases of primary NHL of the common bile duct reported from 1982 to 2017 at home and abroad were analyzed.
Results: The patient was an 81-year-old male, who was diagnosed as primary NHL (diffuse large B cell lymphoma) of the common bile duct by postoperative pathological findings after undergoing pancreatoduodenectomy. No adjuvant anti-tumor therapy such as systemic chemotherapy and regional radiotherapy was performed after operation, and no recurrence was noted 4 months later. Of this patient plus the reported 34 patients, 21 cases (60.0%) were males and 14 cases (40.0%) were females; the age at onset ranged from 4 to 81 years, with a median age of 57 years; the clinical manifestations included jaundice (88.6%) and abdominal pain (40.0%), enlarged superficial lymph nodes (11.4%) and concomitant fever (11.4%); imaging manifestations in some cases showed space occupying lesion in the common bile duct or thickening and stenosis of the common bile duct wall; four cases underwent systemic chemotherapy, 10 cases underwent surgical resection, 16 cases underwent surgical resection combined with systemic chemotherapy, 1 case underwent surgical resection combined with local radiotherapy, 3 cases underwent surgical resection combined with systemic chemotherapy and local radiotherapy, and treatment regimen in 1 case was unknown. The majority of cases had a passable prognosis after treatment, and one case survived more than 72 months; there were no significant differences in survival times between patients undergoing surgical resection alone and those undergoing chemotherapy alone or other combined treatments (all P>0.05).
Conclusion: Primary NHL of the common bile duct is a rare condition in clinical practice, lacks typical clinical manifestations, and the laboratory tests and imaging examinations have no obvious specificity. So, its clinical diagnosis is difficult, and definite diagnosis depends on postoperative pathology and immunohistochemistry. Selection of appropriate treatment options is critical for improving the survival of the patients.