J Korean Neurosurg Soc.  2013 Nov;54(5):415-419. 10.3340/jkns.2013.54.5.415.

Meningeal Supratentorial Hemangioblastoma in a Patient with Von Hippel-Lindau Disease Mimicking Angioblastic Menigioma

• Kim H
• Park IS
• Jo KW

• KMID: 2138358
• DOI: http://doi.org/10.3340/jkns.2013.54.5.415

Abstract

Hemangioblastomas are sporadic tumors found in the cerebellum or spinal cord. Supratentorial hemangioblastomas are rare, and those with meningeal involvement are extremely rare and have been reported in only approximately 130 patients. Here, we report the case of a 51-year-old female patient with supratentorial meningeal hemangioblastoma detected 5 years after surgical resection of an infratentorial hemangioblastoma associated with von Hippel-Lindau disease. Patients with von Hippel-Lindau syndrome are at risk for developing multiple hemangioblastomas, with new tumor formation and growth and possible meningeal infiltration. Regular lifelong follow-up in at-risk patients is recommended and should include the differential diagnosis of dural-based tumors such as angioblastic meningioma and metastatic renal cell carcinoma.

Keyword

Angioblastic meningioma; Hemangioblastoma; Supratentorial; Von Hippel-Lindau disease

MeSH Terms

Carcinoma, Renal Cell
Cerebellum
Diagnosis, Differential
Female
Hemangioblastoma*
Humans
Meningioma
Middle Aged
Spinal Cord
von Hippel-Lindau Disease*

Figure

• 

  Fig. 1 Initial digital subtraction angiography (DSA) and magnetic resonance image (MRI). A : Lateral vertebral artery (VA) DSA shows a highly vascular tumor nodule supplied by the posterior inferior cerebellar artery. B and C : Lateral internal carotid artery (ICA) and external carotid artery (ECA) DSA show no vascular abnormality. D : No abnormal findings are observed in the supratentorial region on an enhanced T1-weighted image. DSA and MRI images at 5 years after the operation. E : Lateral VA DSA shows complete resection of the cerebellar tumor after the previous surgery. F : Left ICA DSA shows pial blood supply to the rim of the tumor. G : Left ECA DSA shows tumor stain from the middle meningeal artery. H : A 4.0×3.6×4.0-cm strongly enhanced dural-based tumor and peritumoral edema are observed in the left frontal lobe on an enhanced T1-weighted image.

• 

  Fig. 2 A : Photomicrograph shows large vessels, lipid-containing vacuoles, and abundant vascular cells adhered to the dura basement (hematoxylin and eosin staining, ×100). B and C : Immunohistochemical staining for epithelial membrane antigen and glial fibrillary acidic protein is negative. D and E : Staining for neuron-specific enolase and vimentin is positive.

Reference

1. Agostinelli C, Roncaroli F, Galassi E, Bernardi B, Acciarri N, Tani G. Leptomeningeal hemangioblastoma. Case illustration. J Neurosurg. 2004; 101(1 Suppl):122. PMID: 16206984.

2. Ammerman JM, Lonser RR, Dambrosia J, Butman JA, Oldfield EH. Long-term natural history of hemangioblastomas in patients with von Hippel-Lindau disease : implications for treatment. J Neurosurg. 2006; 105:248–255. PMID: 17219830.

3. Becker I, Paulus W, Roggendorf W. Histogenesis of stromal cells in cerebellar hemangioblastomas. An immunohistochemical study. Am J Pathol. 1989; 134:271–275. PMID: 2916647.

4. Böhling T, Plate KH, Haltia MJ, Alitalo K, Neumann HP. Von Hippel-Lindau disease and capillary haemangioblastoma. In : Kleihues P, Cavenee WK, editors. World Health Organization classification of tumours Pathology and genetics, tumours of the nervous system. Lyon: IARC Press;2000.

5. Choi DH, Kim HS, Mok JH, Kim DH, Lee KC, Lee YB. Supratentorial meningeal hemangioblastoma : case report. J Korean Neurosurg Soc. 1998; 27:1299–1303.

6. Commins DL, Hinton DR. Cytologic features of hemangioblastoma : comparison with meningioma, anaplastic astrocytoma and renal cell carcinoma. Acta Cytol. 1998; 42:1104–1110. PMID: 9755665.

7. Cosar M, Hatiboglu MA, Iplikcioglu AC, Ozcan D. Parasagittal leptomeningeal hemangioblastoma--case report. Neurol Med Chir (Tokyo). 2006; 46:294–297. PMID: 16794350.

8. Elguezabal A, Díaz ML, Landeyro J, Gené M, Boutayeb L, Escosa M, et al. [Solid-cystic supratentorial hemangioblastoma affecting the falx cerebri. Report of a case]. Neurocirugia (Astur). 2010; 21:401–404. PMID: 21042692.

9. Grant JW, Gallagher PJ, Hedinger C. Haemangioblastoma. An immunohistochemical study of ten cases. Acta Neuropathol. 1988; 76:82–86. PMID: 3394496.

10. Hussein MR. Central nervous system capillary haemangioblastoma : the pathologist's viewpoint. Int J Exp Pathol. 2007; 88:311–324. PMID: 17877533.

11. Iplikçioğlu AC, Yaradanakul V, Trakya U. Supratentorial haemangioblastoma : appearances on MR imaging. Br J Neurosurg. 1997; 11:576–578. PMID: 11013633.

12. Ishwar S, Taniguchi RM, Vogel FS. Multiple supratentorial hemangioblastomas. Case study and ultrastructural characteristics. J Neurosurg. 1971; 35:396–405. PMID: 5167344.

13. Iyigun OL, Cogluk C, Aydin K, Yildiz L, Rakunt C, Celik F. Supratentorial leptomeningeal hemangioblastoma mimicking a meningioma without von Hippel-Lindau complex. Turk Neurosurg. 2004; 14:25–27.

14. Jang H. Supratentorial leptomeningeal hemangioblastoma : case report. Yeungnam Univ J Med. 2007; 24:S770–S774.

15. Kaloostian P, Taylor C. Supratentorial dural-based haemangioblastoma in a Native American patient without Von Hippel Lindau Syndrome. J Surg Case Rep. 2012; 11.

16. Kim HS, Park SH, Cho BM, Kim DH, Oh SM. Supratentorial hemangioblastma, occurred after total removal of recurrent cerebellar hemangioblastoma - Case Report -. J Korean Neurosurg Soc. 2001; 30:348–351.

17. Lee JY, Dong SM, Park WS, Yoo NJ, Kim CS, Jang JJ, et al. Loss of heterozygosity and somatic mutations of the VHL tumor suppressor gene in sporadic cerebellar hemangioblastomas. Cancer Res. 1998; 58:504–508. PMID: 9458097.

18. Lee KR, Kishore PR, Wulfsberg E, Kepes JJ. Supratentorial leptomeningeal hemangioblastoma. Neurology. 1978; 28:727–730. PMID: 566874.

19. Mills SA, Oh MC, Rutkowski MJ, Sughrue ME, Barani IJ, Parsa AT. Supratentorial hemangioblastoma : clinical features, prognosis, and predictive value of location for von Hippel-Lindau disease. Neuro Oncol. 2012; 14:1097–1104. PMID: 22723428.

20. Murali R, Jones WI, Ma Wyatt J. A 57-year-old man with a dural-based parietal lobe tumor. Brain Pathol. 2007; 17:460–463. 474PMID: 17919131.

21. Peyre M, David P, Van Effenterre R, François P, Thys M, Emery E, et al. Natural history of supratentorial hemangioblastomas in von Hippel-Lindau disease. Neurosurgery. 2010; 67:577–587. discussion 587. PMID: 20647972.

22. Richard S, David P, Marsot-Dupuch K, Giraud S, Béroud C, Resche F. Central nervous system hemangioblastomas, endolymphatic sac tumors, and von Hippel-Lindau disease. Neurosurg Rev. 2000; 23:1–22. discussion 23-24. PMID: 10809480.

23. Richard S, Graff J, Lindau J, Resche F. Von Hippel-Lindau disease. Lancet. 2004; 363:1231–1234. PMID: 15081659.

24. Richmond BK, Schmidt JH 3rd. Congenital cystic supratentorial hemangioblastoma. Case report. J Neurosurg. 1995; 82:113–115. PMID: 7815112.

25. Rivera E, Chason JL. Cerebral hemangioblastoma. Case report. J Neurosurg. 1966; 25:452–454. PMID: 5951226.

26. Sharma RR, Cast IP, O'Brien C. Supratentorial haemangioblastoma not associated with Von Hippel Lindau complex or polycythaemia : case report and literature review. Br J Neurosurg. 1995; 9:81–84. PMID: 7786433.

27. Sherman JH, Le BH, Okonkwo DO, Jane JA. Supratentorial dural-based hemangioblastoma not associated with von Hippel Lindau complex. Acta Neurochir (Wien). 2007; 149:969–972. discussion 972. PMID: 17558459.

28. Takei H, Bhattacharjee MB, Rivera A, Dancer Y, Powell SZ. New immunohistochemical markers in the evaluation of central nervous system tumors : a review of 7 selected adult and pediatric brain tumors. Arch Pathol Lab Med. 2007; 131:234–241. PMID: 17284108.

29. Takeuchi H, Hashimoto N, Kitai R, Kubota T. A report of supratentorial leptomeningeal hemangioblastoma and a literature review. Neuropathology. 2008; 28:98–102. PMID: 18181838.

30. Theunissen PH, Debets-Te Baerts M, Blaauw G. Histogenesis of intracranial haemangiopericytoma and haemangioblastoma. An immunohistochemical study. Acta Neuropathol. 1990; 80:68–71. PMID: 2113758.

31. Vortmeyer AO, Gnarra JR, Emmert-Buck MR, Katz D, Linehan WM, Oldfield EH, et al. von Hippel-Lindau gene deletion detected in the stromal cell component of a cerebellar hemangioblastoma associated with von Hippel-Lindau disease. Hum Pathol. 1997; 28:540–543. PMID: 9158701.

32. Wanebo JE, Lonser RR, Glenn GM, Oldfield EH. The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease. J Neurosurg. 2003; 98:82–94. PMID: 12546356.

33. Zeitlin H. Hemangioblastomas of meninges and their relation to Lindau's disease. J Neuropathol Exp Neurol. 1942; 1:14–23.