Abstract
We present here an intriguing case of sporadic renal haemangioblastoma occurring in a 61-year-old male. The tumor consisted of nests of polygonal cells and abundant capillary networks. The neoplastic cells generally showed abundant eosinophilic cytoplasm and prominent eccentric nuclei, resembling the rhabdoid cells. Pronounced intranuclear cytoplasmic pseudoinclusions were another significant feature seen. NSE, a-inhibin and S100 were positive in tumor cells and particularly, focal CD10 expressions were observed. This is possibly the first reported case of a haemangioblastoma showing a rhabdoid phenotype and CD10 immunopositivity. Malignant rhabdoid tumor and renal cell carcinoma with rhabdoid features were probably the most challenging mimics need to be differentiated. 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 SlidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1068858553657049
Highlights
Haemangioblastoma is a slowly growing, highly vascular benign tumor, corresponding to WHO grade I. It typically arises within the central nervous system (CNS), but may occasionally originate in unusual sites such as peripheral nerve, bone, soft tissue, skin, liver, lung and pancreas [1,2,3], and maybe associated with von Hippel-Lindau (VHL) disease. The kidney is another rare site for the development of sporadic haemangioblastoma growth, and only four cases have been reported in the English-language literature so far [4-6]
The majority of the tumor cells in our case showed rhabdoid features, which may be mistaken for other rhabdoid tumors that are known to occur in the kidney
The neoplastic cells in our case demonstrated dark-stained and coarse granular chromatin, and lack the discernable nucleoli as well as cytoplasmic inclusions. (ii) malignant rhabdoid tumors (MRTs) are devoid of the cytoplasmic lipid droplets and arborizing stromal vasculature, characterized by haemangioblastomas. (iii) Immunohistochemically, MRTs occasionally are focally positive for S100 and Neuron-specific enolase (NSE) [9], but a-inhibin staining was not shown
Summary
Haemangioblastoma is a slowly growing, highly vascular benign tumor, corresponding to WHO grade I It typically arises within the central nervous system (CNS), but may occasionally originate in unusual sites such as peripheral nerve, bone, soft tissue, skin, liver, lung and pancreas [1,2,3], and maybe associated with von Hippel-Lindau (VHL) disease. Computed tomography showed that the mass was located in the superior pole No remarkable symptoms such as flank pain or urinary irritation were reported by the patient. The coupled vessels were remained and were frequently dilated (Figure 1F) Reticular fibers enclosed both tumor cells and vasculature in the areas of reticular growth pattern, but barely surrounded the vessel walls in the regions of cellular growth pattern (Figure 2). There was no positive staining for AE1/AE3, CK8/18, CK19, gp200, calretinin, HMB-45, Melan-A, chromogranin, Desmin, Actin, Myoglobin and CD68 (Table 1)
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