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肾血管平滑肌脂肪瘤作为急性腹膜后出血的病因

[Renal angiomyolipoma as a cause of acute retroperitoneal hemorrhage].

作者信息

Furuset A S, Bjerklund Johansen T E, Majak B

机构信息

Kirurgisk avdeling Telemark sentralsjukehus, Porsgrunn.

出版信息

Tidsskr Nor Laegeforen. 1997 Nov 30;117(29):4215-7.

PMID:9441463
Abstract

Angiomyolipoma is a mesenchymal tumor, usually found in the kidney. The pleomorphic appearance and involvement of regional lymph nodes may simulate malignancy, but angiomyolipomas are classified as benign. Renal angiomyolipomas are found in approximately 40% of tuberous sclerosis patients and are usually small, bilateral and asymptomatic nodules. Spontaneous rupture and retroperitoneal haemorrhage may be experienced with larger tumours. Although findings by ultrasonography and computerized tomography are specific for this lesion, histological examination is necessary for a final diagnosis. Small asymptomatic angiomyolipomas should be followed up with sequential CT scans and be removed by enucleation or partial nephrectomy when they reach a size of more than 4 cm in diameter. If the patient suffers from massive bleeding, it is very difficult to preserve the kidney. We report on a 61-year old women who experienced acute retroperitoneal haemorrhage. She was given ten blood transfusions before she was operated on with en bloc removal of the left kidney, an angiomyolipoma with a diameter of 10 cm and a large haematoma.

摘要

血管平滑肌脂肪瘤是一种间叶组织肿瘤,通常见于肾脏。其多形性外观及区域淋巴结受累情况可能类似恶性肿瘤,但血管平滑肌脂肪瘤被归类为良性肿瘤。约40%的结节性硬化症患者存在肾血管平滑肌脂肪瘤,且通常为小的、双侧性且无症状的结节。较大的肿瘤可能会出现自发性破裂和腹膜后出血。尽管超声检查和计算机断层扫描结果对该病变具有特异性,但最终诊断仍需组织学检查。小的无症状血管平滑肌脂肪瘤应通过连续CT扫描进行随访,当直径超过4厘米时可通过剜除术或部分肾切除术切除。如果患者发生大量出血,则很难保留肾脏。我们报告了一名61岁女性,她经历了急性腹膜后出血。在接受手术整块切除左肾(一个直径10厘米的血管平滑肌脂肪瘤和一个大血肿)之前,她接受了十次输血。

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