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      Primary renal angiosarcoma mimicking urothelial carcinoma – A case report and literature reviews

      case-report

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          Abstract

          Angiosarcoma (AS) is a rare aggressive tumor originating from endothelial cells. We reported a 66-year-old female with primary renal angiosarcoma (PRA) who presented as urothelial carcinoma with hematuria and dysuria. Based on ureterorenoscopic tumor biopsy, the initial diagnosis suggested low-grade non-invasive urothelial carcinoma. However, the specimen retrieved from nephroureterectomy confirmed the diagnosis of primary renal angiosarcoma.

          Primary renal angiosarcoma could uncommonly present as urothelial carcinoma in renal pelvis. Surgical resection remains to be the most effective therapy but there is no consensus about adjuvant therapies. The overall prognosis of primary renal angiosarcoma is dismal.

          Highlights

          • Primary renal angiosarcoma is a rare disease that could mimic urothelial carcinoma.

          • Surgical resection is considered to be main treatment.

          • The effectiveness osf adjuvant therapies remain to be clarified.

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          Most cited references5

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          Clinical and Pathologic Features of Primary Angiosarcoma of the Kidney.

          Primary angiosarcoma of the kidney is extremely rare; hence, relatively little is known regarding its clinicopathologic features and prognosis. Herein, we review the literature on primary renal angiosarcoma with emphasis on the clinical and pathologic features.
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            Clinicopathological features of primary angiosarcoma of the kidney: a review of 62 cases

            Angiosarcoma (AS) is a malignant tumor occurring in less than 2% of soft tissue sarcomas. Primary involvement of the kidney is rare, its pathogenesis remains largely unknown and it has overlapping features with other tumors of the kidney. The objective of this paper is to review the case reports of primary AS of the kidney in the literature. The search terms were primary AS of the kidney, primary renal AS and primary renal hemangiosarcoma. The total cohort of the cases reviewed was 62. The mean age of presentation was 61 years old with a predilection for the male sex. Metastatic disease at the time of diagnosis accounted for 44.9% (22/49) of the cases reported and 44.4% (12/27) of patients with non-metastatic disease at diagnosis, subsequently developed metastasis. Primary AS of the kidney is a rare malignant tumor with a poor prognosis. Local recurrence and distant metastasis is common. Primary AS of the kidney shares similar clinical presentation with other renal tumors and imaging does not allow for tumor specific diagnosis. Histopathological examination and immunohistochemistry is very important for the confirmation of the diagnosis. Current treatment options include a variable combination of surgery, radiotherapy and chemotherapy.
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              Epithelioid angiosarcoma of the kidney: A case report and literature review

              Epithelioid angiosarcoma (EAS) is a rare disease which presents a great diagnostic challenge. The present study reports a case of EAS in the kidney in a 75-year-old male who presented with gross hematuria. An abdominal computed tomography scan revealed space-occupying lesions of the right kidney and renal cell carcinoma was suspected. Histological examination of the resected specimens showed pleomorphic epithelioid cells with vesicular nuclei, prominent nucleoli and eosinophilic cytoplasm that lined irregular vascular spaces. Immunohistochemical staining revealed that the tumor cells were positive for AE1/AE3, cytokeratin (CK) 7, vimentin, cluster of differentiation (CD) 31 and E-cadherin, but showed no staining for CD10, CD34, factor VIII, CK20, carcinoembryonic antigen or desmin. Based on the histopathological and immunohistochemical findings, the patient was diagnosed with epithelioid angiosarcoma. Postoperative radiation therapy was administered and no recurrence was observed six months after surgery.
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                Author and article information

                Contributors
                Journal
                Urol Case Rep
                Urol Case Rep
                Urology Case Reports
                Elsevier
                2214-4420
                09 September 2020
                January 2021
                09 September 2020
                : 34
                : 101407
                Affiliations
                [a ]Department of Urology, Kaohsiung Municipal Siaogang Hospital, Kaohsiung, Taiwan
                [b ]Department of Urology, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
                [c ]Department of Pathology, Kaohsiung Municipal Ta-Tung Hospital, Kaohsiung, Taiwan
                [d ]Department of Urology, Kaohsiung Municipal Ta-Tung Hospital, Kaohsiung, Taiwan
                [e ]Department of Urology, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan
                [f ]Graduate Institute of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
                Author notes
                []Corresponding author. Department of Urology, College of Medicine, Kaohsiung Medical University, Department of Urology, Kaohsiung Municipal Ta-Tung Hospital, Kaohsiung, No.68, Zhonghua 3rd Rd., Qianjin Dist., Kaohsiung City, 80145, Taiwan. spacejason69@ 123456yahoo.com.tw
                Article
                S2214-4420(20)30296-5 101407
                10.1016/j.eucr.2020.101407
                7596193
                33145170
                40cd9a00-7840-4293-824d-736410e5f1ba
                © 2020 The Authors

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 23 July 2020
                : 8 September 2020
                Categories
                Oncology

                angiosarcoma,urothelial carcinoma,nephroureterectomy,adjuvant chemotherapy,radiotherapy,pra, primary renal angiosarcoma,pa, primary angiosarcoma,ct, computed tomography,pet, positron emission tomography

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