Primary renal angiosarcoma
A 67-year-old gentleman presented with dull aching left loin pain since the last two months. He also had malaise, loss of appetite and significant loss of weight. There was no hematuria, headache, hemoptysis or bone pain. There was no history of previous exposure to radiation or agents like thorotrast and vinyl chloride but he had a sixty pack year history of smoking. Physical examination revealed pallor and a large ballotable mass in the left flank.\nOn evaluation, his hemoglobin was 5.7 gm% and creatinine was 1.72 mg%. Liver function tests and chest X-ray were normal. Contrast-enhanced computed tomogram revealed a 13 × 12 × 10 cm, predominantly hypodense left upper and inter-polar renal mass with few areas of enhancement, abutting the posterior abdominal wall, psoas and spleen []. After optimization, he underwent open radical nephrectomy through a subcostal transperitoneal approach. Intraoperatively, the left renal mass was seen to involve the entire kidney and infiltrate the splenic capsule, diaphragm and the psoas muscle.\nGrossly, the tumor measured 13 × 10 × 7 cm. Sectioning revealed a hemorrhagic surface admixed with areas of necrosis. The posterior aspect showed a raw area with breach of capsule and attachment to remnants of diaphragm. The tumor was not seen to infiltrate the renal sinus or hilum [].\nMicroscopy showed renal parenchyma infiltrated by an extensively hemorrhagic and focally necrotic tumor []. Scanty viable tumor, composed of clusters of spindle to polygonal-shaped cells with moderately pleomorphic and mitotically active nuclei was seen. Some tumor cells were seen to line small spaces containing RBCs []. On immunohistochemistry, the tumor cells were diffusely positive for CD31, CD34 and vimentin and were negative for pancytokeratin and EMA [Figures and ]. These features are consistent with angiosarcoma. Ki proliferative index was high (>80%).\nHe had an uneventful postoperative recovery and was started on paclitaxel-based chemotherapy. Paclitaxel was given in a dose of 115 mg intravenously, weekly for 3 weeks, followed by one week rest. The next cycle was started on day 28 and a total of 3 cycles are planned.