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一例罕见的不明原因发热病例:肝脏炎性肌纤维母细胞瘤。病例报告及文献综述

A rare case of fever of unknown origin: inflammatory myofibroblastic tumor of the liver. Case report and review of the literature.

作者信息

Kruth Jens, Michaely Henrik, Trunk Markus, Niedergethmann Marco, Rupf Ann-Katrin, Krämer Bernhard K, Göttmann Uwe

机构信息

3rd Department of Medicine (Hematology and Oncology), University Hospital Mannheim, University of Heidelberg, Mannheim, Germany.

出版信息

Acta Gastroenterol Belg. 2012 Dec;75(4):448-53.

Abstract

We present the case of a rare cause of fever of unknown origin (FUO). FUO is challenging for patients as well as for physicians as there are more than 200 differential diagnoses of FUO (1,2). Pointing out a diagnosis often requires numerous noninvasive and invasive procedures that sometimes even fail to explain the fever. Our patient was admitted twice to our hospital due to remitting fever rising up to 40 degrees C without any subjective discomfort. At the first presentation no clinical focus could be identified. This included the examination of multiple blood and urine cultures, serology, autoimmune serology, transesophageal echocardiography, CT-scan of the lung and the abdomen, and bone scintigraphy. Elevated C-reactive protein (268 mg/l) decreased spontaneously and fever disappeared after 4 weeks. However, the patient was re-admitted 4 months later with identical symptoms. Multiple blood and urine cultures, serology, bone marrow examination, CT-scan of the lung and the abdomen, esophago-gastro-duodenoscopy and colonoscopy still showed no pathological findings. MRI-scan of the abdomen identified a liver tumor of 3.3 cm in diameter in segment 6 without typical signs of an adenoma, focal nodular hyperplasia or hepatocellular carcinoma. Biopsy of the suspect liver lesion revealed an inflammatory myofibroblastic tumor (inflammatory pseudotumor). After surgical resection of the tumor elevated inflammation markers as C-reactive protein normalized and fever disappeared. One year after surgery no more episodes of fever re-occurred. An inflammatory myofibroblastic tumor of the liver can be a rare cause of fever of unknown origin. MRI-scan can be an additional imaging tool to identify previously not recognized liver tumors.

摘要

我们报告一例不明原因发热(FUO)的罕见病因。FUO对患者和医生来说都是一项挑战,因为FUO有200多种鉴别诊断(1,2)。明确诊断往往需要进行大量的非侵入性和侵入性检查,有时甚至无法解释发热原因。我们的患者因弛张热高达40摄氏度且无任何主观不适,两次入住我院。首次就诊时未发现临床病灶。检查包括多次血培养和尿培养、血清学检查、自身免疫血清学检查、经食管超声心动图检查、肺部和腹部CT扫描以及骨闪烁显像。C反应蛋白升高(268mg/l)后自发下降,发热在4周后消失。然而,患者在4个月后因相同症状再次入院。多次血培养和尿培养、血清学检查、骨髓检查、肺部和腹部CT扫描、食管胃十二指肠镜检查和结肠镜检查仍未发现病理结果。腹部MRI扫描在肝6段发现一个直径3.3cm的肝肿瘤,无腺瘤、局灶性结节性增生或肝细胞癌的典型征象。对可疑肝病灶进行活检,结果显示为炎性肌纤维母细胞瘤(炎性假瘤)。肿瘤手术切除后,C反应蛋白等炎症标志物升高的情况恢复正常,发热消失。术后一年未再出现发热发作。肝脏炎性肌纤维母细胞瘤可能是不明原因发热的罕见病因。MRI扫描可作为一种辅助成像工具,用于识别先前未被发现的肝脏肿瘤。

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