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      Sporadic Hemangioblastoma of the Kidney: a rare renal tumor

      case-report

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          Abstract

          Abstract

          Hemangioblastoma is a benign and morphologically distinctive tumor that can occur sporadically or in association with von Hippel-Lindau disease in approximately 25% of the cases, and which involves the central nervous system in the majority of the cases. Rare occurrences of hemangioblastoma in peripheral nerves and extraneural tissues have been reported. This report describes one case of sporadic renal hemangioblastoma happened in a 16-year-old Chinese female patient, presenting with hematuria, and low back pain. Histologically, the tumors were circumscribed, and composed of sheets of large polygonal cells traversed by arborizing thin-walled blood vessels. The diagnosis of hemangioblastoma was confirmed by negative immunostaining for cytokeratin, and positive staining for α-inhibin, S100 and neuron-specific enolase (NSE). This benign neoplasm which can be mistaken for various malignancies such as renal cell carcinoma, epithelioid hemangiopericytoma and epithelioid angiomyolipoma, deserves wider recognition for its occurrence as a primary renal tumor.

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          Uncommon and recently described renal carcinomas.

          J. Srigley, Brett Delahunt (2009)
          Major consensus conferences held over a decade ago laid the foundations for the current (2004) WHO classification of renal carcinoma. Clear cell, papillary and chromophobe carcinomas account for 85-90% carcinomas seen in routine practice. The remaining 10-15% of carcinomas consist of rare sporadic and hereditary tumors, some of which had been long recognized, but many of which only emerged as distinct entities in the decade leading up to the WHO publication. Collecting-duct carcinoma is a rare, often lethal form of carcinoma. Medullary carcinoma associated with sickle cell trait, has emerged as a distinctive tumor showing some overlapping features with upper tract urothelial carcinoma. Mucinous tubular and spindle-cell carcinoma and tubulocystic carcinoma were earlier considered as patterns of low-grade collecting-duct carcinoma, but are now recognized as separate tumor entities. Carcinomas associated with somatic translocations of TFE3 and TFEB comprise a significant proportion of pediatric renal carcinomas. Oncocytoid renal carcinomas in neuroblastoma survivors was recognized as a unique tumor category in the WHO classification. Renal carcinoma associated with end-stage renal disease is now recognized as having distinct morphological patterns and behavior. In addition there is a group of rare recently described carcinomas, including clear cell papillary carcinoma, oncocytic papillary renal cell carcinoma, follicular renal carcinoma and leiomyomatous renal cell carcinoma. It behooves the surgical pathologist to not only be capable of diagnosing the common forms of renal cancer, but also to be aware of the rare types of renal carcinoma, many of which have emerged in recent years.
          Article 0 comments Cited 41 times – based on 0 reviews     Review now
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            Extraneural hemangioblastoma: a report of 5 cases.

            Jaime Rodriguez-Canales, Juan Rosai, Daisuke Nonaka (2007)
            Hemangioblastoma is a morphologically distinctive tumor that can occur sporadically or in association with von Hippel-Lindau disease, and which involves the central nervous system in the majority of the cases. Rare occurrences of hemangioblastoma in peripheral nerves and extraneural tissues have been reported. The histogenesis of this tumor remains uncertain. Various cell lineages such as vascular, glial, neural, fibrohistiocytic, and smooth muscle/myofibroblastic have been proposed for the so-called stromal cells, which are thought to represent the neoplastic component of these lesions. We report on 5 cases of hemangioblastoma arising in extraneural tissues. Two of the tumors were located in the presacral region, and one each in the maxilla, kidney, and adrenal glands. All 5 cases were morphologically indistinguishable from central nervous system hemangioblastoma. The existence of these cases suggests that the "stromal" cells of hemangioblastoma can demonstrate a variety of mature specific lineages, such as smooth muscle/myofibroblastic, or neuroendocrine, depending on the location and possibly the microenvironment.
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              Sporadic hemangioblastoma of the kidney: an underrecognized pseudomalignant tumor?

              Nancy Ip, Wui-hang Cheung, Qun Yuan … (2010)
              Hemangioblastoma is a benign tumor that can occur sporadically, or in association with von Hippel-Lindau disease in approximately one-quarter of the cases. Only exceptionally does it occur outside the central nervous system. This report describes 2 cases of sporadic renal hemangioblastoma, with 1 patient presenting with hematuria and polycythemia, and the other low back pain. Histologically, the tumors were circumscribed, and composed of sheets of large polygonal cells traversed by arborizing thin-walled blood vessels. Many of the tumor cells showed pleomorphic nuclei, but the mitotic figures were rare. The cytoplasm was eosinophilic, and occasionally finely vacuolated indicating the presence of lipid. The diagnosis of hemangioblastoma was confirmed by negative immunostaining for cytokeratin, and positive staining for α-inhibin, S100, and neuron-specific enolase. This benign neoplasm which can be mistaken for various malignancies such as renal cell carcinoma, epithelioid angiomyolipoma, adrenal cortical carcinoma, and paraganglioma, deserves wider recognition for its occurrence as a primary renal tumor.
              Article 0 comments Cited 11 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (nlm-ta): Diagn Pathol
                Journal ID (iso-abbrev): Diagn Pathol
                Title: Diagnostic Pathology
                Publisher: BioMed Central
                ISSN (Electronic): 1746-1596
                Publication date Collection: 2012
                Publication date (Electronic): 1 May 2012
                Volume: 7
                Page: 49
                Affiliations
                [1 ]Department of Pathology, the First Affiliated Hospital and College of Basic Medical Sciences, China Medical University, Shenyang, 110001, China
                [2 ]Institute of Pathology and Pathophysiology, China Medical University, Shenyang, 110001, China
                Article
                Publisher ID: 1746-1596-7-49
                DOI: 10.1186/1746-1596-7-49
                PMC ID: 3488519
                PubMed ID: 22548972
                SO-VID: 8dcb1594-f9b0-42d2-a59e-77a606b292ff
                Copyright © Copyright ©2012 Liu et al.; licensee BioMed Central Ltd.
                License:

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 13 March 2012
                Date accepted : 22 April 2012
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Pathology
                Keywords: von hippel-lindau disease,epithelioid angiomyolipoma,hemangioblastoma,renal cell carcinoma,epithelioid hemangiopericytoma
                Data availability:
                ScienceOpen disciplines: Pathology
                Keywords: von hippel-lindau disease, epithelioid angiomyolipoma, hemangioblastoma, renal cell carcinoma, epithelioid hemangiopericytoma

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