CASE REPORT Open AccessSporadic haemangioblastoma of the kidney withrhabdoid features and focal CD10 expression:report of a case and literature reviewWei-hua Yin 1 , Jian Li 1* and John KC Chan 2AbstractWe present here an intriguing case of sporadic renal haemangioblastoma occurring in a 61-year-old male. Thetumor consisted of nests of polygonal cells and abundant capillary networks. The neoplastic cells generally showedabundant eosinophilic cytoplasm and prominent eccentric nuclei, resembling the rhabdoid cells. Pronouncedintranuclear cytoplasmic pseudoinclusions were another significant feature seen. NSE, a-inhibin and S100 werepositive in tumor cells and particularly, focal CD10 expressions were observed. This is possibly the first reportedcase of a haemangioblastoma showing a rhabdoid phenotype and CD10 immunopositivity. Malignant rhabdoidtumor and renal cell carcinoma with rhabdoid features were probably the most challenging mimics need to bedifferentiated. The result of focal CD10 staining in our case may further lead to confusion with renal cell carcinoma.To avoid misdiagnosis, more considerations should be attached to the rare neoplasm.Virtual Slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1068858553657049Keywords: Haemangioblastoma, Kidney, CD10, Rhabdoid featuresBackgroundHaemangioblastoma is a slowly growing, highly vascularbenign tumor, corresponding to WHO grade I. It typicallyarises within the central nervous system (CNS), but mayoccasionally originate in unusual sites such as peripheralnerve, bone, soft tissue, skin, liver, lung and pancreas[1-3], and maybe associated with von Hippel-Lindau(VHL) disease.The kidney is another rare site for the development ofsporadic haemangioblastoma growth, and only four caseshave been reported in the English-language literature sofar [4-6]. The accurate diagnosis is often challengingwhen the tumor develops in this region. We describedherein the fifth case of this rare tumor, which notablydemonstrated a rhabdoid phenotype as well as unex-pected CD10 staining. In addition, the shared characteris-tics of renal haemangioblastomas (RHB) and theirdifferential considerations were also discussed in detail.Case presentationA 61-year-old man was admitted to our hospital for asolid mass found in the right kidney during a routinecheckup. Computed tomography showed that the masswas located in the superior pole. No remarkable symp-toms such as flank pain or urinary irritation werereported by the patient. He also had no familial historyor clinical evidences of VHL disease. Radical nephrect-omy was carried out, showing a 5.3 × 5.0 × 5.0 cm mass.It was grey to yellowish in color and well-demarcatedfrom the surrounding renal parenchyma. The patient hadan uneventful postoperative recovery and was well at12 months follow-up.Histopathological and Immunohistochemical findingsMicroscopically, the specimen consisted of nests of poly-gonal tumor cells and a prominent capillary network.Focal areas of necrosis were present. The stromashowed extensive fibrosis and hyalinization, whichamounted to approximately one third of the lesion(Figure 1A, B).* Correspondence: lijianhouma@yahoo.cn1 Department of Pathology, Peking University Shenzhen Hospital, No.1120Lianhua North Road, Shenzhen 518000, ChinaFull list of author information is available at the end of the articleYin et al. Diagnostic Pathology 2012, 7:39http://www.diagnosticpathology.org/content/7/1/39© 2012 Yin et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative CommonsAttribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction inany medium, provided the original work is properly cited.
