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: To date, 13 cases of sporadic renal hemangioblastoma have been reported. In this article, we report such a case that might cause the diagnostic pitfall. A 37-year-old Japanese was found to have a renal mass by periodic medical check-up. He underwent radical nephrectomy. Macroscopically, the tumor was well-defined without fibrous capsule and the cut surface of the tumor exhibited light brown to gray-tan color without hemorrhage or necrosis. Microscopically, the tumor was made up of large polygonal to short spindle cells with eosinophilic cytoplasm with occasional vacuolization and abundant arborizing capillary network. Immunohistochemically, neoplastic cells showed diffuse positivity for inhibin-alpha, S-100 protein, vimentin, CA9, PAX2 and PAX8, but negativity for cytokeratin CAM5.2, alpha smooth muscle actin, Melanosome, Melan A, TFE3 and cathepsin K. In genetic analyses, this tumor showed no changes of VHL gene mutation, hypermethylation and loss of heterozygosity of chromosome 3p. Additionally, G-band karyotype and array comparative genomic hybridization studies showed a normal chromosome. In conclusion, the positivity for CA9, PAX2 and PAX8 in sporadic renal hemangioblastoma may cause the critical diagnostic pitfall in the differential diagnosis from clear cell renal cell carcinoma. Pathologists need to pay attention to systemic evaluation including macroscopic, microscopic and immunohistochemical findings. In some cases, molecular genetic study may be necessary.
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    ABSTRACT: Mesenchymal tumors of the kidney, although infrequently encountered, constitute a wide spectrum of lesions. The relative rarity of these tumors means that in some instances criteria to differentiate between benign and malignancy are currently incompletely defined. More recently a variety of novel stromal tumors have been characterized, with hemangioblastoma and myopericytoma being notable examples. The identification of a subset of spindle cell tumors as synovial sarcoma, on the basis of the presence of a characteristic genetic translocation, has facilitated the correct classification of a number of tumors previously labeled as fibrosarcoma, malignant fibrous histiocytoma, or more recently cystic embryonal sarcoma. In this review, we have detailed the spectrum of both benign and malignant stromal tumors of the adult kidney, described the gross and microscopic features, with an emphasis on immunoexpression and the differential diagnosis of each tumor type. Copyright © 2015 Elsevier Inc. All rights reserved.
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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:
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