Clarunis, Department of Visceral Surgery, University Centre for Gastrointestinal and Liver Diseases, St. Clara Hospital and University Hospital, Basel, Switzerland.
Department of General Surgery, Cantonal Hospital Lucerne, Spitalstrasse, CH-6000, Lucerne 16, Switzerland.
Int J Colorectal Dis. 2020 Feb;35(2):343-346. doi: 10.1007/s00384-019-03484-y. Epub 2019 Dec 12.
The sarcoid-lymphoma syndrome is a rare condition where both entities co-exist in the same patient. Overlapping clinical manifestations, imaging findings, and pathological characteristics pose both a diagnostic as well as therapeutic challenge, especially in the setting of life-threatening complications. The objective of this case report is to highlight rare complications caused by abdominal sarcoidosis as well as a subsequently diagnosed non-Hodgkin lymphoma.
A 35-year-old man presented with massive upper gastrointestinal bleeding caused by a large retroperitoneal mass. Following several endoscopic and radiologic interventions, successful hemostasis could only be achieved by an emergency Whipple procedure. Biopsies of the mass showed an abdominal manifestation of sarcoidosis and corticosteroid therapy was started. The patient required several additional emergency surgeries due to a complicated bronchoperitoneal fistula, splenic abscess, perforation of the sigmoid colon, small bowel leakage, and repetitive intra-abdominal bleeding.
Histopathological findings finally revealed a concurrent lymphoma, as clinically suspected earlier. Ultimately, polychemotherapy was administered.
A concurrent lymphoma can lead to emergency surgery and should be considered when sarcoidosis treatment does not improve symptoms, lymphadenopathy occurs, or hematologic changes persist under therapy.
肉瘤样-淋巴瘤综合征是一种罕见的疾病,两种病症同时存在于同一患者体内。重叠的临床表现、影像学发现和病理学特征既带来了诊断上的挑战,也带来了治疗上的挑战,尤其是在危及生命的并发症的情况下。本病例报告的目的是强调由腹部肉瘤样病引起的罕见并发症以及随后诊断的非霍奇金淋巴瘤。
一名 35 岁男性因巨大的腹膜后肿块引起大量上消化道出血而就诊。在进行了几次内镜和放射学干预后,仅通过紧急胰十二指肠切除术才能实现有效的止血。肿块的活检显示出腹部肉瘤样病的表现,并开始使用皮质类固醇治疗。由于复杂的支气管-腹膜瘘、脾脓肿、乙状结肠穿孔、小肠漏和反复的腹腔内出血,该患者需要进行多次额外的急诊手术。
最终的组织病理学发现证实了之前临床怀疑的同时存在的淋巴瘤。最终,进行了多化疗。
当肉瘤样病治疗不能改善症状、出现淋巴结病或在治疗下血液学改变持续存在时,应考虑同时存在淋巴瘤,这可能导致急诊手术。