Markopoulos Konstantinos, Bührer Emanuel, Banz Yara, Dawson Heather, Engelbrecht Swantje, Essig Manfred, Misselwitz Benjamin
Department of Gastroenterology and Internal Medicine Tiefenauspital, Bern, Switzerland.
Department of Medical Oncology, Inselspital, Bern University Hospital, Bern, Switzerland.
J Gastrointest Oncol. 2022 Oct;13(5):2583-2607. doi: 10.21037/jgo-22-74.
Marginal zone lymphoma can be accompanied by symptoms of small intestinal disease including abdominal pain and malabsorption. However, the best diagnostic approach for suspected marginal zone lymphoma is unknown and intestinal biopsies are frequently negative. We describe the case of a patient with symptoms of small bowel involvement where marginal zone lymphoma could only be detected upon peripheral lymph node resection. To assess the clinical variability of intestinal marginal zone lymphoma as a rare clinical entity, a scoping review with systematic literature research was performed.
A 57-year-old man presented with a 10-year history of postprandial abdominal pain, systemic inflammation and recent weight loss. Endoscopies and a surgical small bowel specimen revealed non-specific findings. Flow cytometry from the bone marrow was highly suspicious for marginal zone lymphoma. A 2-F-fluorodeoxyglucose-positron emission tomography/computed tomography (2-F-FDG-PET/CT) showed hypermetabolic lymph nodes on both sides of the diaphragm. Cervical lymph node dissection finally confirmed marginal zone lymphoma. Immunochemotherapy yielded lasting oncological remission and resolved symptoms. We searched PubMed, Embase and Ovid MEDLINE for additional case reports limited to the last 25 years. Five primary search terms combined using "AND" were used freely and as controlled vocabulary. Additional studies were identified by reviewing the reference lists of included articles.
Our review revealed 52 cases of marginal zone lymphoma with small intestinal manifestation. Patients presented with abdominal pain, bowel obstruction, weight loss or gastrointestinal bleeding. Diagnosis was mainly established by surgery (73%). The most frequent endoscopic findings were mucosal erosions and ulcerations. A 2-F-FDG-PET/CT was positive in 9/15 patients. Treatment included rituximab, chemotherapy, surgery and/or radiation resulting in clinical remission in 82% of cases.
Diagnostic workup for suspected small intestinal marginal zone lymphoma is challenging, necessitating a multidisciplinary approach. Endoscopy, imaging including 2-F-FDG-PET/CT and small bowel resection or dissection of hypermetabolic lymph nodes can be useful. If marginal zone lymphoma is suspected vigorous diagnostic efforts are justified since remission can be achieved in most patients. Our review highlights the variable clinical presentation of this underdiagnosed disease and adds systematic data to the literature.
边缘区淋巴瘤可伴有小肠疾病症状，包括腹痛和吸收不良。然而，疑似边缘区淋巴瘤的最佳诊断方法尚不清楚，且肠道活检结果常为阴性。我们描述了一例有小肠受累症状的患者，该患者仅在切除外周淋巴结后才检测到边缘区淋巴瘤。为评估肠道边缘区淋巴瘤作为一种罕见临床实体的临床变异性，我们进行了一项系统文献研究的范围综述。
一名57岁男性，有10年餐后腹痛、全身炎症及近期体重减轻的病史。内镜检查和手术获取的小肠标本显示为非特异性结果。骨髓流式细胞术高度怀疑为边缘区淋巴瘤。2-氟-脱氧葡萄糖-正电子发射断层扫描/计算机断层扫描（2-F-FDG-PET/CT）显示双侧膈肌旁淋巴结代谢活跃。颈部淋巴结清扫最终确诊为边缘区淋巴瘤。免疫化疗使肿瘤长期缓解并缓解了症状。我们在PubMed、Embase和Ovid MEDLINE上搜索了过去25年内的其他病例报告。使用“AND”组合的五个主要检索词作为自由词和控制词汇使用。通过查阅纳入文章的参考文献列表确定了其他研究。
我们的综述共纳入52例有小肠表现的边缘区淋巴瘤病例。患者表现为腹痛、肠梗阻、体重减轻或胃肠道出血。诊断主要通过手术确定（73%）。最常见的内镜检查结果为黏膜糜烂和溃疡。15例患者中有9例2-F-FDG-PET/CT呈阳性。治疗包括利妥昔单抗、化疗、手术和/或放疗，82%的病例实现了临床缓解。
疑似小肠边缘区淋巴瘤的诊断工作具有挑战性，需要多学科方法。内镜检查、包括2-F-FDG-PET/CT在内的影像学检查以及小肠切除或代谢活跃淋巴结清扫可能有用。如果怀疑边缘区淋巴瘤，积极的诊断努力是合理的，因为大多数患者可以实现缓解。我们的综述强调了这种诊断不足疾病的可变临床表现，并为文献增添了系统数据。
