Abstract：
Leiomyosarcomas of large vessels are rare. It is a malignant tumour and the vast majority of these tumours arose from the inferior vena cava. We report a rare case of portal vein leiomyosarcoma, in a 56-years-old female patient admitted for chronic abdominal pain with abdominal mass in the right hypochondrium all evolving in a context of deterioration in general condition. We performed an abdominopelvic CT scan and then a MRI with contrast agent which objectified a large tissue mass containing areas of necrosis at the level of the duodeno-pancreatic compartment communicating at a large angle with the portal trunk over its entire length from the hepatic hilum to the spleno-mesenteric confluence responsible for a portal cavernoma downstream. This is associated with multiple secondary nodular tissue hepatic lesions. We also noted a respect for the fatty border separating the mass of the duodenal tract and the head of the pancreas, and also the absence of dilation of the pancreatic ducts making a pancreatic origin unlikely. To eliminate a duodenal origin of the mass we performed an upper digestive endoscopy which came back without any abnormality. An ultrasound-guided trans parietal biopsy of a secondary hepatic lesion was done and the pathological result of which speaks of a secondary hepatic lesion of a leiomyosarcoma.
摘要：
大血管平滑肌肉瘤是罕见的。这是一种恶性肿瘤，这些肿瘤绝大多数来自下腔静脉。我们报道了一例罕见的门静脉平滑肌肉瘤，在一名56岁的女性患者中，该患者因慢性腹痛而入院，右侧软骨下腹部肿块均在一般状况恶化的背景下发展。我们进行了腹骨盆CT扫描，然后用造影剂进行了MRI扫描，该造影剂在十二指肠胰室水平上形成了一个大的组织块，该组织块包含坏死区域，该坏死区域在从肝门到肝门的整个长度上与门静脉主干成较大角度连通。脾-肠系膜汇合，负责下游的门静脉海绵瘤。这与多个继发性结节性组织肝病变有关。我们还注意到对十二指肠和胰头的脂肪边界的尊重，也没有扩张的胰管，使胰腺起源的可能性不大。为了消除肿块的十二指肠起源，我们进行了上消化道内窥镜检查，结果没有任何异常。对继发性肝病变进行了超声引导的经顶叶活检，其病理结果说明平滑肌肉瘤的继发性肝病变。
