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罕见血管肿瘤导致的呼吸困难:肝下下腔静脉平滑肌肉瘤

Dyspnea due to an uncommon vascular tumor: leiomyosarcoma of the infrahepatic vena cava inferior.

作者信息

Gafarli Samra, Igna Dorian, Wagner Mathias, Nistor Adriana, Glanemann Matthias, Stange Barbara

机构信息

Department of General-, Visceral-, Vascular- and Pediatric Surgery, Saarland University, Kirrberger Straße 100, D-66421, Homburg, Saar, Germany.

Department of Pathology, Saarland University, Homburg, Saar, Germany.

出版信息

Surg Case Rep. 2020 Jun 16;6(1):136. doi: 10.1186/s40792-020-00896-9.

Abstract

BACKGROUND

Leiomyosarcoma (LMS) of the inferior vena cava (IVC) is a rare malignancy that originated from the smooth muscle tissue of the vascular wall. Diagnoses, as well as, treatment of the disease are still challenging and to date, a radical surgical resection of the tumor is the only curative approach.

CASE REPORT

We report on the case of a 49-year old male patient who presented with suddenly experienced dyspnea. Besides bilateral pulmonary arterial embolism, a lesion close to the head of the pancreas was found using CT scan, infiltrating the infrahepatic IVC. Percutaneous ultrasound-guided biopsy revealed a low-grade LMS. Intraoperatively, a tumor of the IVC was observed without infiltration of surrounding organs or distant metastases. Consequently, the tumor was removed successfully, by en-bloc resection including prosthetic graft placement of the IVC. Histological workup revealed a completely resected (R0) moderately differentiated LMS of the IVC.

CONCLUSION

LMS of the infrahepatic IVC is an uncommon tumor, which may present with dyspnea as its first clinical sign. Patients benefit from radical tumor resection. However, due to the poor prognosis of vascular LMS, a careful follow-up is mandatory.

摘要

背景

下腔静脉平滑肌肉瘤(LMS)是一种罕见的恶性肿瘤,起源于血管壁的平滑肌组织。该疾病的诊断和治疗仍然具有挑战性,迄今为止,肿瘤的根治性手术切除是唯一的治愈方法。

病例报告

我们报告一例49岁男性患者,该患者突然出现呼吸困难。除双侧肺动脉栓塞外,通过CT扫描发现胰腺头部附近有一个病变,侵犯肝下下腔静脉。经皮超声引导下活检显示为低级别LMS。术中观察到下腔静脉肿瘤,无周围器官浸润或远处转移。因此,通过包括下腔静脉人工血管置换的整块切除成功切除了肿瘤。组织学检查显示为下腔静脉完全切除(R0)的中度分化LMS。

结论

肝下下腔静脉LMS是一种罕见肿瘤,可能以呼吸困难作为其首发临床症状。患者可从肿瘤根治性切除中获益。然而,由于血管LMS预后较差,必须进行仔细的随访。

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