Sporadic renal haemangioblastoma: Case report and review of the literature

  • Authors:
    • Yadong Wang
    • Can Wei
    • Lisha Mou
    • Qiang Zhang
    • Zhiwen  Cui
    • Xianxin Li
    • Jiongxian Ye
    • Yongqing Lai
  • View Affiliations

  • Published online on: September 27, 2012     https://doi.org/10.3892/ol.2012.942
  • Pages: 360-362
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Abstract

Haemangioblastoma is a benign tumour which generally occurs in a relatively restricted area of the central nervous system. Renal haemangioblastoma are extremely rare. We report a rare case of renal haemangioblastoma occurring in a 61-year-old male with a solid mass, which was detected during a routine examination. The patient was asymptomatic and abdominal computed tomography (CT) revealed a solid mass in the right kidney. No definitive preoperative diagnosis could be established. Surgical resection of the tumour revealed sporadic renal haemangioblastoma by pathological examination. The patient was followed up at 1 year without any problems. We also present a supplementary review of previously published cases and literature.

Introduction

Haemangioblastoma is a slowly growing, highly vascular solid mass of uncertain histogenesis, also known as capillary haemangioblastoma (1). It typically occurs within the central nervous system (CNS), predominantly in the cerebellum (2). Renal haemangioblastoma is an extremely uncommon disease with a poorly established diagnosis due to a lack of descriptions of typical symptoms. To the best of our knowledge, there are 6 cases reported previously (Table I) (3,4). In the present study, we report the case of a patient with haemangioblastoma involving the kidney, which may be mistaken for other renal tumours, in particular renal cell carcinoma (RCC). The study was approved by the ethics committee of Peking University Shenzhen Hospital, Shenzhen, China. Written informed patient consent was obtained from the patient.

Table I

Reported cases of renal haemangioblastoma.

Table I

Reported cases of renal haemangioblastoma.

CaseAuthorYearAge (years)/genderChief complaintSize (cm)Clinical featuresVHL (yes/no)Immunohistochemical stainingFollow-up (months) prognosis
1Nonaka200771/femaleAsymptomatic6.8Right renal upper poleNoS100+, SMA+, MSA+, calponin+, vimentin+108, without disease
2Ip201058/maleHaematuria and polycythaemia5.5Right renal upper poleNoS100+, NSE+, α-inhibin+24, alive
3Ip201055/femaleLow back pain3.5Right renal upper poleNoS100+, NSE+, α-inhibin+48, alive
4Verine201164/maleOther disease3.2Left renal upper poleNoCA-IX+, S100+, NSE+, α-inhibin+12, no recurrence
5Wang201229/maleOther disease2.7Right renalNoS100+, NSE+, α-inhibin+20, without disease
6Liu201216/femaleHaematuria1.2Left renal upper poleNoAE1/AE3, S100+, NSE+, α-inhibin+6, no recurrence
7Present201261/maleCheckup6.5Right renal upper poleNoS100+, NSE+, α-inhibin+12, no recurrence

[i] VHL, von Hippel-Lindau; NSE, neuron-specific enolase.

Case report

A 61-year-old male, who was found to have a right renal tumour during a routine examination, was admitted to our department for further examination on April 20, 2011. The patient was asymptomatic with a normal appetite, no abdominal pain and no weight changes. The patient had no urinary, respiratory or cardiovascular symptoms, no constitutional symptoms and had not previously undergone surgery. His family history was unremarkable. Physical examination revealed a well-developed and well-nourished male. The patient was afebrile and had a pulse of 66 beats per min, temperature 36.7°C, blood pressure 120/60 mmHg and respiration 20 per min. The chest was clear to percussion and auscultation and no masses were palpable on abdominal examination.

Laboratory examination revealed that haemoglobin was 12.3 g/dl, white blood cell count was 7.21×109/l, with 61.4% granulocytes. Glucose was 5.88 mmol/l, blood urea nitrogen was 8.25 mmol/l and serum creatinine was 82.2 μmol/l. Liver function tests and serum electrolytes were within normal limits. Urinalysis was unremarkable. Chest X-ray was normal. A non-contrast computed tomography (CT) scan of the kidneys revealed a 6.5×6.2-cm round hypodense mass [48.2–54.2 Hounsfield units (HU)] in the upper pole of the right kidney (Fig. 1A and B). A contrast-enhanced CT revealed a heterogeneously enhanced mass with a non-enhanced hypodense region in the centre (Fig. 1C and D).

A right radical nephrectomy was performed on April 25, 2011. Tumour invasion to the adjacent tissue was not observed. Grossly, the tumour was a solid and well-encapsulated mass 6.5 cm in diameter. Microscopic examination revealed polygonal cells diffusely positive for α-inhibin, neuron-specific enolase (NSE) and S100 and confirmed renal haemangioblastoma. There was no clinical evidence of von Hippel-Lindau (VHL) disease. Magnetic resonance imaging (MRI) revealed no other tumours.

The patient was discharged on the fifth postoperative day after an uncomplicated post-surgical recovery. No evidence of recurrence or residual disease appeared on CT scans at follow-up (1 year).

Discussion

Capillary haemangioblastoma is a benign tumour of uncertain histogenesis that generally occurs in a relatively restricted area of the CNS (2). In microscopic views, the tumours may exhibit significant nuclear pleomorphism, mimicking carcinoma or other malignancies (1,2). A majority of cases arise sporadically, whereas 25% are a manifestation of VHL disease (5). This tumour typically occurs within the CNS, predominantly in the cerebellum (2). Haemangioblastoma has also been rarely reported in sites outside of the CNS, including peripheral nerves, liver, lung, pancreas, retroperitoneum, kidney, pancreas, bladder, soft tissues of the ankle and popliteal fossa and nasal skin, usually in the setting of known VHL disease (6).

There are only a few studies concerning sporadic haemangioblastoma occurring outside the central nervous system. Sporadic renal haemangioblastoma are extremely rare, with only 6 cases reported previously (3,4). We describe the 7th sporadic renal haemangioblastoma. The diagnosis in the present case was based on the presence of typical morphology and immunophenotype (S100+, NSE+, α-inhibin+) (5).

CT scanning, with and without the administration of contrast material, is necessary to take full advantage of the contrast enhancement characteristics of highly vascular renal parenchymal tumours. In general, any renal mass that enhances with intravenous administration of contrast material on CT scanning by more than 15 HU should be considered an RCC until proven otherwise (7). In this patient, a contrast-enhanced CT revealed a heterogeneously enhanced mass (more than 20 HU; Fig. 1). The mass was thus highly suggestive of RCC. Until now, characteristic imaging features of renal haemangioblastoma remained unknown (3,5).

Haemangioblastoma is likely to be an underrecognised tumour of the kidney as it mimics numerous tumour types morphologically and is usually not considered in the differential diagnosis (8). A correct diagnosis is important for patients as haemangioblastoma is a benign disease, unlike malignant RCC (3). It is not necessary for patients with sporadic renal haemangioblastoma to receive further treatment (e.g., molecular targeted therapy) and their follow-up is different from that of patients with RCC (3).

We report a solid lesion of the kidney found to be a sporadic renal haemangioblastoma. Our review of published studies revealed only 6 cases reported previously. Haemangioblastoma is likely to be an underrecognised tumour of the kidney, as it mimics a number of tumour types morphologically and is usually not considered in the differential diagnosis. A correct diagnosis is important to avoid overdiagnosis and unnecessary clinical treatment.

Acknowledgements

This study was supported by the National Natural Science Foundation of China (No. 81101922).

References

1 

Karabagli H, Karabagli P, Alpman A and Durmaz B: Congenital supratentorial cystic hemangioblastoma. Case report and review of the literature. J Neurosurg. 107(Suppl 6): 515–518. 2007.PubMed/NCBI

2 

Hussein MR: Central nervous system capillary haemangioblastoma: the pathologist's viewpoint. Int J Exp Pathol. 88:311–324. 2007.PubMed/NCBI

3 

Wang CC, Wang SM and Liau JY: Sporadic hemangioblastoma of the kidney in a 29-year-old man. Int J Surg Pathol. Jan 23–2012.(Epub ahead of print).

4 

Liu Y, Qiu XS and Wang EH: Sporadic hemangioblastoma of the kidney: a rare renal tumor. Diagn Pathol. May 1–2012.(Epub ahead of print).

5 

Ip YT, Yuan JQ, Cheung H and Chan JK: Sporadic hemangioblastoma of the kidney: an underrecognized pseudomalignant tumor? Am J Surg Pathol. 34:1695–1700. 2010.PubMed/NCBI

6 

Verine J, Sandid W, Miquel C, Vignaud JM and Mongiat-Artus P: Sporadic hemangioblastoma of the kidney: an underrecognized pseudomalignant tumor? Am J Surg Pathol. 35:623–624. 2011. View Article : Google Scholar : PubMed/NCBI

7 

Zagoria RJ and Dyer RB: The small renal mass: detection, characterization, and management. Abdom Imaging. 23:256–265. 1998. View Article : Google Scholar : PubMed/NCBI

8 

Nonaka D, Rodriguez J and Rosai J: Extraneural hemangioblastoma: a report of 5 cases. Am J Surg Pathol. 31:1545–1551. 2007. View Article : Google Scholar : PubMed/NCBI

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Spandidos Publications style
Wang Y, Wei C, Mou L, Zhang Q, Cui Z, Li X, Ye J and Lai Y: Sporadic renal haemangioblastoma: Case report and review of the literature. Oncol Lett 5: 360-362, 2013.
APA
Wang, Y., Wei, C., Mou, L., Zhang, Q., Cui, Z., Li, X. ... Lai, Y. (2013). Sporadic renal haemangioblastoma: Case report and review of the literature. Oncology Letters, 5, 360-362. https://doi.org/10.3892/ol.2012.942
MLA
Wang, Y., Wei, C., Mou, L., Zhang, Q., Cui, Z., Li, X., Ye, J., Lai, Y."Sporadic renal haemangioblastoma: Case report and review of the literature". Oncology Letters 5.1 (2013): 360-362.
Chicago
Wang, Y., Wei, C., Mou, L., Zhang, Q., Cui, Z., Li, X., Ye, J., Lai, Y."Sporadic renal haemangioblastoma: Case report and review of the literature". Oncology Letters 5, no. 1 (2013): 360-362. https://doi.org/10.3892/ol.2012.942