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Review
. 2015 Dec;68(12):1020-5.
doi: 10.1136/jclinpath-2015-202900. Epub 2015 Jul 22.

Extraneural hemangioblastoma of the kidney: the challenge for clinicopathological diagnosis

Yong Wu  1 , Tao Wang  1 , Pei-Pei Zhang  1 , Xiaoqun Yang  1 , Jian Wang  1 , Chao-Fu Wang  1
Affiliations
Review

Extraneural hemangioblastoma of the kidney: the challenge for clinicopathological diagnosis

Yong Wu et al. J Clin Pathol. 2015 Dec.

Abstract

Background: Hemangioblastoma is a benign cerebellar tumour which may occur as a sporadic entity or in association with von Hippel-Lindau (VHL) disease in approximately 25% of cases. Renal hemangioblastoma (RH) is an extremely rare and newly recognised tumour. Here, we describe five cases of RH, one discovered by CT in an accident and the other four detected during routine examinations.

Methods: Five cases of renal hemangioblastoma retrieved from the Department of Pathology, Fudan University Shanghai Cancer Center were studied and the literatures were reviewed. Immunohistochemistry was used to differentiate and confirm this tumour.

Results: Pathological examination following tumour resection revealed RH in all cases, the first patient was also diagnosed with renal cell carcinoma (RCC), suggesting the possibility of VHL syndrome, but PCR sequencing analysis of the VHL gene confirmed no mutation in any of the three exons, implying sporadic disease. Histologically, the tumours were circumscribed, composed of sheets of oval or polygonal cells and a prominent vascular network. Tumour cells had pleomorphic nuclei, but mitotic figures were rare. The diagnosis of hemangioblastoma was confirmed by immunohistochemistry.

Conclusions: RH is very rare and is challenging to differentially diagnose. Distinguishing RCC and RH is difficult and each has a different prognosis, so differentiating between them is essential for avoiding over-diagnosis and unnecessary treatment.

Keywords: IMMUNOHISTOCHEMISTRY; KIDNEY; RENAL CANCER.

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Figures

Figure 1
Figure 1
Histological features. (A) Most tumours were solid and were traversed by arborising thin-walled blood vessels. (B) Tumours were composed of plump spindle cells with palely eosinophilic cytoplasm. (C) The microvacuolated cells often mimicked lipoblasts or renal cell carcinoma and were mildly atypical. (D) Abundant anastomosing thin blood vessels surrounding bland-appearing stromal cells. Under a high-power field, scattered tumour cells have highly pleomorphic nuclei.
Figure 2
Figure 2
Blood vessels often had ectasia or pericytomatous configurations.
Figure 3
Figure 3
In most cases, tumour cells expressed inhibin (A) and vimentin (B).
Figure 4
Figure 4
Tumour cells were positive for neuron-specific enolase (A) and S100 protein (B).
Figure 5
Figure 5
EMA was focally positive in stromal cells in some cases.
Figure 6
Figure 6
Most tumours except case 1 were negative for CK (A), CK7 (B), CK8 (C), PAX8 (D), AE1/AE3 (E) and HMB45 (F).
Figure 7
Figure 7
CD34 stains highlighted the capillary network but not the tumour cells.
See this image and copyright information in PMC

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