Primary angiosarcoma of the kidney: case report and comprehensive literature review

Abstract

<jats:title>Abstract</jats:title><jats:sec id="j_med-2019-0048_s_007_w2aab3b7d289b1b6b1aab1c16b1Aa"><jats:title>Purpose</jats:title><jats:p>Angiosarcoma (AS) is an aggressive malignant tumour of endothelial origin, most frequently compelling the skin. The kidney is a rare site of primary occurrence of AS with a quite challenging diagnosis and poor prognosis, mainly due to its raw metastasising power. We described our first case of AS treatment. A comprehensive literature review in this field is also carried out.</jats:p></jats:sec><jats:sec id="j_med-2019-0048_s_008_w2aab3b7d289b1b6b1aab1c16b2Aa"><jats:title>Patients and methods</jats:title><jats:p>We report the case of a 56-years-old man with radiological appearance of an 11 cm poor vascularised left renal mass. The pre-operative imaging ruled out distant metastases and inferior vena cava thrombosis. Thus, after radioembolization, we performed a transperitoneal open radical nephrectomy with regional lymphadenectomy with the removal of the middle portion of the ileo-psoas muscle, followed by two lines of adjuvant chemotherapy. We analysed all the papers concerning primary kidney AS until December 2018.</jats:p></jats:sec><jats:sec id="j_med-2019-0048_s_009_w2aab3b7d289b1b6b1aab1c16b3Aa"><jats:title>Results</jats:title><jats:p>Estimated intraoperative blood loss was 200 mL with an operative time of 100 minutes. No postoperative complications were recorded, with the patient discharge in the 8th postoperative day. The pathological assessment showed a pT3a N0, M0 tumour compatible with AS of the kidney. An immune-histochemical study showed a vascular positive for CD31, CD34, Fli-1 with a Ki-67/Mib 1 of 50%. One month after surgery the patient began adjuvant chemotherapy with gemcitabine. After 45 days from surgery, a Computed Tomography scan showed lung and liver metastases with disease relapse in the left kidney lodge. The response to the first-line treatment was excellent, unlike the second-line, encountering a chemo-refractory disease. The cancer-specific survival was of 15 months. We included 66 cases in our review.</jats:p></jats:sec><jats:sec id="j_med-2019-0048_s_010_w2aab3b7d289b1b6b1aab1c16b4Aa"><jats:title>Conclusion</jats:title><jats:p>This is the first case of treatment of kidney AS performed at our Institute. Our findings added new information about the unclear biology and progression of this subset of tumours, demonstrating an abysmal prognosis.</jats:p></jats:sec>

CT Findings of Primary Renal Angiosarcoma

“…With respect to imaging findings, the tumors have been described as an extensive hemorrhagic or necrotic mass with variable peripheral enhancement similar to our series, or a large necrotic mass [2-6, 8, 9]. However, there are very few useful imaging features, suggestive of primary renal angiosarcomas.…”

“…Angiosarcoma is an aggressive malignant neoplasm originating from endothelial cells of the blood and lymphatic vessels [6, 7]. Among the various malignant tumors that can occur in the kidney, primary angiosarcomas are extremely rare, with only about 68 cases reported to date in the literature published in 2019 [8]. Although the presence of several risk factors, such as thorotrast, arsenic, polyvinyl chloride, radiotherapy, and chronic lymphedema, have been reported in angiosarcomas arising at other sites in the body, there is no evidence of a direct relationship between these predisposing factors and primary angiosarcomas of the kidney [3-5].…”

Primary cardiac angiosarcoma: a case report

“…Approximately 60% of lesions arise in the skin, soft tissue, liver, spleen, bones, and breasts, whereas the heart and kidneys are less frequently affected. 4 Primary cardiac angiosarcoma (PCAS) accounts for 33% of all primary malignant cardiac neoplasms, 5 and the disease has a male predominance (incidence ratio of 2–3:1), 6 , 7 and the onset age is 30 to 50 years old. The lesion mostly originates in the right atrium and atrial septum, whereas right ventricle and left cardiac system involvement is relatively rare.…”

Vascular Neoplasms and Related Lesions of Kidney

“…Angiosarcoma is a malignant vascular neoplasm which may occur in any location of the body, including the skin, soft tissue, breast, bone and visceral organs including the liver. Primary renal angiosarcoma is very rare, with about 70 cases described in the literature to date [49][50][51][52][53][54][55][56][57]. Most of these cases are seen in older men, with a peak incidence in the sixth to seventh decades and a pronounced male predilection (male-to-female ratio of 7:1) [49].…”

“…CT imaging demonstrates a hypodense mass with varying levels of contrast enhancement ranging from none, to heterogeneous, to intense peripheral, to diffuse enhancement [53,54,58,59]. Central areas of necrosis or hemorrhage may be detected [55].…”