Tsuda N, Chowdhury P R, Hayashi T, Anami M, Iseki M, Koga S, Matsuya F, Kanetake H, Saito Y, Horita Y
Department of Pathology, Nagasaki University Hospital, Japan.
Pathol Int. 1997 Nov;47(11):778-83. doi: 10.1111/j.1440-1827.1997.tb04457.x.
Primary renal angiosarcoma is very rare. To our knowledge, only 15 cases have been reported to date. A 77-year-old Japanese man with a unilateral kidney presented with massive hematuria followed by renal failure. A renal tumor was suspected and a left nephrectomy was performed. The histopathological diagnosis was angiosarcoma of the kidney. A hemorrhagic tumor measuring 10 x 5 cm and clotted blood was found in the medullary area. The atypical tumor cells had a sinusoidal and solid appearance, and showed immunohistochemically positive reactions for some of the endothelial markers. The patient died about 21 months after the nephrectomy and the autopsy revealed massive metastases to the liver and retroperitoneum. One of the differential diagnoses of the case was angiomyolipoma, because the tumor cells were relatively bland in their histological appearance with entrapped fat cells in the pelvic area. Fifteen case reports with titles that included the term 'hemangiosarcoma/angiosarcoma', 'hemangioendothelioma/endothelioma' or 'vascular sarcoma' of the kidney were reviewed and compared to the present case.
原发性肾血管肉瘤非常罕见。据我们所知，迄今为止仅报道过15例。一名77岁的日本单侧肾男性患者出现大量血尿，随后发展为肾衰竭。怀疑有肾肿瘤并进行了左肾切除术。组织病理学诊断为肾血管肉瘤。在髓质区域发现一个大小为10×5 cm的出血性肿瘤及凝血块。非典型肿瘤细胞呈窦状和实性外观，免疫组化显示对某些内皮标志物呈阳性反应。患者在肾切除术后约21个月死亡，尸检显示肝脏和腹膜后有大量转移。该病例的鉴别诊断之一是血管平滑肌脂肪瘤，因为肿瘤细胞在组织学外观上相对温和，盆腔区域有包绕的脂肪细胞。我们回顾并比较了15篇标题中包含“血管肉瘤/血管内皮肉瘤”“血管内皮瘤/内皮瘤”或“血管肉瘤”的肾病例报告与本病例。
