Lee Tsu-Yee Joseph, Lawen Joseph, Gupta Rekh
Department of Pathology, QEII Health Science Center, Dalhousie University, Halifax, Nova Scotia, Canada.
Can J Urol. 2007 Feb;14(1):3471-6.
In adults renal cell carcinoma (RCC) accounts for over 85% of all diagnosed renal cancers. A much more rare and aggressive malignant tumor of the kidney is angiosarcoma (AS) with less than 25 cases described internationally. Both RCC and AS have similar radiological appearances and thus require histological evaluation for definitive diagnosis. We present a case of renal AS in a 63-year old male who was initially radiologically diagnosed as RCC, and review the current renal AS literature.
The current English literature from 1981 and onwards on renal AS was reviewed and compared to our current case.
The median age and sex of patients with renal AS at presentation was 63 years old (mean 61 years) and common in males with a left kidney predominance. Symptoms included flank pain, palpable mass, and hematuria with imaging suggestive of RCC. Hematogenous metastatic spread often occurred with median survival time of 3.5 months from time of diagnosis (mean 5.8 months). Histologically, the tumors have classical features of angiosarcoma with numerous blood-filled vascular spaces lined by plump pleomorphic endothelial cells with CD31 and CD34 staining positivity. Overall treatment was radical nephrectomy with radiation therapy for local control and metastases. The use of chemotherapy was not consistent.
Although RCC accounts for the majority of malignant renal tumors, the poor prognosis of AS and its similar radiological appearance to RCC imparts the importance of histological evaluation and the potential radiological mimicry of AS.
在成人中，肾细胞癌（RCC）占所有确诊肾癌的85%以上。一种更为罕见且侵袭性更强的肾脏恶性肿瘤是血管肉瘤（AS），国际上报道的病例不足25例。RCC和AS具有相似的放射学表现，因此需要进行组织学评估以明确诊断。我们报告一例63岁男性肾AS病例，该患者最初经放射学诊断为RCC，并回顾当前关于肾AS的文献。
回顾了1981年及以后关于肾AS的英文文献，并与我们当前的病例进行比较。
肾AS患者就诊时的中位年龄和性别为63岁（平均61岁），男性常见，以左肾为主。症状包括胁腹痛、可触及肿块和血尿，影像学表现提示RCC。常发生血行转移，从诊断时起中位生存时间为3.5个月（平均5.8个月）。组织学上，肿瘤具有血管肉瘤的典型特征，有许多充满血液的血管腔，内衬丰满的多形性内皮细胞，CD31和CD34染色呈阳性。总体治疗为根治性肾切除术，并辅以放射治疗以控制局部病变和转移。化疗的使用并不一致。
尽管RCC占恶性肾肿瘤的大多数，但AS预后较差且其放射学表现与RCC相似，这凸显了组织学评估的重要性以及AS可能存在的放射学模拟表现。
