Okuda Kenichi Vinzenz, Laass Martin, Schuchardt Katrin, Lange Björn Sönke, Knöfler Ralf, Fitze Guido, Woessmann Wilhelm, Suttorp Meinolf
Dept. of Pediatrics, Univ. Hospital "Carl Gustav Carus", Technical University, Dresden, Germany.
Div. of Pediatric Gastroenterology, Dept. of Pediatrics, Univ. Hospital "Carl Gustav Carus", Technical University, Dresden, Germany.
Klin Padiatr. 2018 Apr;230(3):138-141. doi: 10.1055/a-0586-4045. Epub 2018 Apr 4.
Burkitt lymphoma (BL) in children often presents with abdominal localization. Intestinal perforations have been described mainly during treatment. We report on a three-year-old patient with abdominal BL who was diagnosed with a duodenocolonic fistula.
A three-year-old boy presented with diarrhea, crampy abdominal pain, and a four-week history of loss of appetite and weight. Ultrasound and MRI detected a colonic tumor forming a duodenocolonic fistula which was verified by gastroduodenoscopy. A surgical biopsy revealed BL. The stage III BL with low LDH was treated with four courses of BFM-type short-pulse chemotherapy. After two courses of chemotherapy the patient developed a mechanic ileus. A segmental resection of a short segment of the colon at the right flexure carrying the residual tumor mass with cicatricial stenosis and fistula followed by colonic end to end anastomosis and covering of the fistula by omentum major were carried out without complication. 15 days after surgery, two additional courses of chemotherapy could be administrated and the boy is in ongoing remission and free of any symptoms with a follow-up interval of 18 months.
Duodeonocolonic fistula at presentation in a child with abdominal BL is extremely rare. Delayed surgery after size of the tumor bulk has been reduced by chemotherapy might represent a risk adapted approach. However, due to limited experience with duodenocolonic fistulas even in larger pediatric lymphoma trials any decision has to be based on the problems to be faced in individual cases.
儿童伯基特淋巴瘤(BL)常表现为腹部局限性病变。肠穿孔主要在治疗期间被描述过。我们报告一例三岁腹部BL患儿,其被诊断为十二指肠结肠瘘。
一名三岁男孩出现腹泻、腹部绞痛,并有四周食欲减退和体重减轻的病史。超声和磁共振成像检测到一个形成十二指肠结肠瘘的结肠肿瘤,经胃十二指肠镜检查得以证实。手术活检显示为BL。低乳酸脱氢酶水平的III期BL采用四个疗程的BFM型短脉冲化疗。化疗两个疗程后,患儿出现机械性肠梗阻。对右结肠弯曲处携带残余肿瘤块、伴有瘢痕性狭窄和瘘管的一小段结肠进行了节段性切除,随后进行结肠端端吻合,并将大网膜覆盖在瘘管上,手术无并发症。术后15天,又进行了两个疗程的化疗,该男孩目前处于持续缓解状态,无任何症状,随访间隔为18个月。
腹部BL患儿初诊时出现十二指肠结肠瘘极为罕见。在通过化疗使肿瘤体积缩小后延迟手术可能是一种风险适应性方法。然而,由于即使在大型儿科淋巴瘤试验中,十二指肠结肠瘘的经验也有限,任何决策都必须基于个别病例所面临的问题。