Sporadic Hemangioblastoma of the Kidney: An Underrecognized Pseudomalignant Tumor?

Departments of *Pathology ¶Urology, Saint-Louis HospitalParis, France §Department of PathologySainte-Anne Hospital, Paris, France ∥Department of PathologyNancy University Hospital, France †Paris-Diderot University Paris- Paris 7 Paris, France ‡Inserm U728, Paris, France.
The American journal of surgical pathology (Impact Factor: 4.59). 03/2011; 35(4):623-4. DOI: 10.1097/PAS.0b013e31820f6d11
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    ABSTRACT: Hemangioblastoma is a benign, morphologically distinctive neoplasm of disputed histogenesis that typically occurs in the central nervous system either in the setting of von Hippel-Lindau disease or more often sporadically. Extraneural hemangioblastoma is exceptional and raises a challenging differential diagnosis. Herein, we report a primary renal hemangioblastoma occurring in 51-year-old woman without stigmata of von Hippel-Lindau disease. Histologically, the tumor was composed of sheets of polygonal epithelioid stromal cells with ample pale or eosinophilic, vacuolated cytoplasm in an arborizing capillary network. Tumor cells showed variable nuclear pleomorphism, intranuclear cytoplasmic invaginations, scattered hyaline globules, and psammoma-like calcifications. Some areas showed branching hemangiopericytoma-like vessels with tumor cells radiating from the wall, while other areas were edematous and hyalinized with sparse stromal cells and abundant reticular vessels. Immunohistochemically, the tumor cells reacted strongly and diffusely with antibodies to PAX8, CD10, α-inhibin, S100 protein, neuron-specific enolase, and vimentin, and they showed focal positivity with antibodies to epithelial membrane antigen and AE1/AE3. Tumor cells were negative for CK7, CK8/18, RCC antigen, synaptophysin, chromogranin, c-kit, D2-40, HMB45, melan-A, cathepsin K, SMA, desmin, CD31, CD34, and estrogen and progesterone receptors. Positive immunoreactivity for PAX8 is unexpected and contrasts to central nervous system (CNS) hemangioblastomas, which are essentially always negative for PAX8. This novel finding adds support to the hypothesis that the immunoprofile of extraneural hemangioblastoma varies with site of origin, perhaps as a result of tumor cell lineage and retention of organ-specific markers or acquisition of site-specific antigens due to local factors.
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    ABSTRACT: In this study, we presented an additional case of renal hemangioblastoma, which demonstrates PAX2 and focal CD10 expression. Histologically, the tumor consisted of sheets of oval or polygonal cells and a prominent vascular network. The tumor cells varied in size, and possessed pale or eosinophilic cytoplasm that sometimes contained sharply delineated fine vacuoles. The tumor cell nuclei with inconspicuous nucleoli showed moderate nuclear atypia and pleomorphism. Focal areas of stromal hyalinization and sclerosis were detected. On account of its strong or moderate immunoreactivity for the a-inhibin, S100, NSE, and EGFR, the diagnosis of renal hemangioblastoma was established. For further evidence of VHL deficiency, the tumor was subjected to VHL sequence analysis of all three exons and fluorescence in situ hybridization (FISH) detection for chromosome 3p deletion. None of the VHL gene mutations and chromosome 3p deletion was detected in the tumor. Because of several shared morphological and immunophenotypic features, renal hemangioblastoma may be underrecognized and should be included in the differential diagnosis of primary renal tumors, in particular clear cell renal cell carcinoma. The unexpected positive staining of PAX2 and CD10 in renal hemangioblastoma should be particular concerned. Using a combination of immunoprofile may be helpful to the differential diagnosis of these renal tumors.
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    ABSTRACT: The morphological features and immunohistochemical findings of two cases of clear cell renal cell carcinoma with extensive hemangioblastoma-like features are described. To the best of our knowledge, this is the first description of such a pattern, the differential diagnosis being with renal hemangioblastoma, a rare tumor that could be considered a diffuse hemangioblastoma-like change in a clear cell renal cell tumor. Even though the clinical significance and therapeutic implications are not yet known, the hemangioblastoma-like pattern could have favorable prognostic significance based on the fact that the renal hemangioblastomas described so far have benign behavior.
    European Urology 05/2014; 66(5). DOI:10.1016/j.eururo.2014.04.022 · 12.48 Impact Factor