Haemangioblastoma versus metastatic renal cell carcinoma

Haemangioblastoma usually involves the cerebellum or spinal cord. Both haemangioblastoma and renal clear cell carcinoma may occur as components of the von Hippel-Lindau syndrome. Since both are highly vascular clear cell tumours, distinguishing haemangioblastoma from metastatic renal cell carcinoma may be problematic.

Immunohistochemistry

 

 

CNS haemangioblastoma

renal clear cell carcinoma

 

Renal cell carcinoma marker

0/711

28/551

Inhibin A

20/222, 25/253, 66/674

5/212, 0/193, 16/344

CD10

0/711, 0/222, 0/674

46/541, 21/212, 27/344, 85%8

EMA

0/72, 0/63, 24/674

34/344

NSE

7/72, 9/93

usually negative

Aquasporin 1

67/674, 26/266, 10/107

13/344, 78%8

AE1/AE3

0/674

30/344

D2-40

13/674, 23/235

12/344, 0/285

     

A combination of Aquasporin 1 and AE1/AE3 allows the differentiation of haemangioblastoma from renal clear cell carcinoma4.

Cytogenetics

Both sporadic haemangioblastoma and sporadic renal cell carcinoma may show loss of the VHL tumour suppressor gene on the short arm of chromosome 34.

References

1 Ingold B, Wild PJ, Nocito A, et al. Renal cell carcinoma marker reliably discriminates central nervous system haemangioblastoma from brain metastases of renal cell carcinoma. Histopathology 2008; 52:674-81

2 Jung SM,Kuo TT. Immunoreactivity of CD10 and inhibin alpha in differentiating hemangioblastoma of central nervous system from metastatic clear cell renal cell carcinoma. Mod Pathol 2005; 18:788-94 FULL TEXT

3 Hoang MP,Amirkhan RH. Inhibin alpha distinguishes hemangioblastoma from clear cell renal cell carcinoma. Am J Surg Pathol 2003; 27:1152-6

4 Weinbreck N, Marie B, Bressenot A, et al. Immunohistochemical markers to distinguish between hemangioblastoma and metastatic clear-cell renal cell carcinoma in the brain: utility of aquaporin1 combined with cytokeratin AE1/AE3 immunostaining. Am J Surg Pathol 2008; 32:1051-9

5 Roy S, Chu A, Trojanowski JQ, et al. D2-40, a novel monoclonal antibody against the M2A antigen as a marker to distinguish hemangioblastomas from renal cell carcinomas. Acta Neuropathol 2005; 109:497-502

6 Chen Y, Tachibana O, Oda M, et al. Increased expression of aquaporin 1 in human hemangioblastomas and its correlation with cyst formation. J Neurooncol 2006; 80:219-25

7 Longatti P, Basaldella L, Orvieto E, et al. Aquaporin 1 expression in cystic hemangioblastomas. Neurosci Lett 2006; 392:178-80

8 Mazal PR, Stichenwirth M, Koller A, et al. Expression of aquaporins and PAX-2 compared to CD10 and cytokeratin 7 in renal neoplasms: a tissue microarray study. Mod Pathol 2005; 18:535-40

This page last revised 19.7.2008

©SMUHT/PW Bishop