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: 5.15). 03/2011; 35(4):623-4. DOI: 10.1097/PAS.0b013e31820f6d11
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    • "In such particular case, immunohistochemical staining might be the only solution. In contrast to hemangioblastoma, clear cell RCC is usually negative for α-inhibin, S100, and NSE and positive for AE1/AE3, EMA and CD10 [5,6]. And in this case, the tumor cells show striking morphologic mimicry of epithelioid angiomyolipoma. "
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    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. Virtual slides The virtual slide(s) for this article can be found here:
    Diagnostic Pathology 05/2012; 7(1):49. DOI:10.1186/1746-1596-7-49 · 2.60 Impact Factor
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    • "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 accurate diagnosis is often challenging when the tumor develops in this region. "
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    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 Slides The virtual slide(s) for this article can be found here:
    Diagnostic Pathology 04/2012; 7(1):39. DOI:10.1186/1746-1596-7-39 · 2.60 Impact Factor
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    ABSTRACT: Hemangioblastoma of the kidney is a rare, newly recognized tumor with morphological features similar to its cerebellar counterpart. There have been only 4 cases reported in the literature in English, all of them occurring in middle-aged to elderly patients. Here, we report a case of renal hemangioblastoma in a young adult without clinical evidence of von Hippel-Lindau disease. The tumor was composed of polygonal cells with mildly eosinophilic to clear cytoplasm and a rich vascular network. Immunohistochemical staining revealed a typical profile (positivity for α-inhibin, neuron-specific enolase and S100; negative results for epithelial membrane antigen, HMB-45, and Melan-A), which confirmed the diagnosis. Despite the similarity to renal-cell carcinoma in morphology, hemangioblastoma of the kidney is clinically indolent. Correct recognition of this pathological entity is important to avoid overdiagnosis and unnecessary clinical treatment.
    International Journal of Surgical Pathology 01/2012; 20(5):519-22. DOI:10.1177/1066896911434548 · 0.95 Impact Factor
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