A Rare Case of Sporadic Renal Hemangioblastoma

Background: Hemangioblastoma is a low grade highly vascular neoplasm mainly found in the cerebellar region between the fourth and fifth decades of life. The extra CNS locations are rare and even rarer when present as sporadic form, not associated with Von Hipple Lindau disease. To the best of our knowledge’s about 17 cases had been reported in kidney by review of literature till day. The main differential diagnosis is clear cell renal cell carcinoma and IHC study to confirm the diagnosis should be done to avoid the unnecessary treatment. Complete surgical resection provides excellent result and rare reports of recurrence after incomplete excision is reported.Case report: We report a 24 year old Iranian female presented with flank pain and single homogenous solid mass in left kidney on CT scan with no significant past medical and familial history especially related to Hipple Lindau disease. The patient underwent partial nephrectomy and the dissected encapsulated mass show homogenous brown cut surfaces which composed of hypo and hyper cellular areas of proliferation of closely packed variable sized thin walled vessels with solid areas of large stromal cells containing foamy cytoplasm on microscopic examination. Immunohistochemical study was done and the neoplastic stromal cells shows positive immunoreaction for vimentin, inhibin and SMA .8 months follow up the patient revealed no tumoral recurrence.Conclusion: sporadic Hemangioblastoma of the kidney is a rare neoplasm with uncertainly histogenesis that can mimic some most common tumors such as RCC, metastatic adrenal cortical carcinoma, epithelioid angiosarcoma and paraganglioma. Careful IHC study can strongly help in diagnosis and prevent the unnecessary treatment.

Keywords:

Hemangioblastoma , von-Hippel-Lindau (VHL) , renal cell carcinoma