Cerebellar Hemangioblastoma: Four Case Reports and Review of the Literature

Year 2015, Volume: 40 Issue: 1, 184 - 192, 10.09.2015

Sevgi Bakariş , Mürüvet Yüksel

https://doi.org/10.17826/cutf.73086

Cited By: 1

Abstract

Hemangioblastoma (HB) is a benign, slow-growing, highly vascular tumour of not well defined histological origin. These tumors make up about 1 to 2 percent of all intracranial neoplasms and occur primarily in the posterior fossa. Hemangioblastomas can occur sporadically but in about 20% to 30% cases, it is associated with von Hippel-Lindau (VHL) disease. Four cases of cerebellar haemangioblastoma, not associated with von Hippel-Lindau disease (sporadic haemangioblastomas), were presented and reviewed the relevant literature.Four hemangioblastomas of the central nervous system were examined with haematoxylin & eosin (H&E), reticulin stain and with a panel of antibodies including CD34, vimentin, NSE, S-100, CD99, CD56, GFAP, cytoceratin, epithelial membrane antigen (EMA), CD10. Of the 4 patients in this study 1 was male and 3 were female. Their ages ranged from 46 years to 60 years with a mean age of 54.75 years. All of them were as cystic nodules about 2-3 cm in diameter. In the histopathological examination, the tumors sections showed large and vacuolated stromal cells and numerous arborizing capillary-size blood vessels. Some tumors showed atypical nuclei. Vimentin was strongly positive both stromal cells and blood veessels in all tumors. In 4 cases of HB, some stromal cells were positive for NSE and CD99. Three tumors were positive for S-100 and CD56, two tumors were focally positive for glial fibrillary acidic protein (GFAP). CD34 immunostaining highlighted the arborizing and complex vascular network, whereas the tumor stromal cells were negative. The stromal cells were negative for epithelial markers such as cytokeratin, EMA and CD10. Ki-67 index was less than 1% of the tumor cells. Hemangioblastoma, a rare, benign tumors of uncertain histogenesis, is characterized histologically by the presence of vacuolated, lipid containing cells and a well developed, fine capillary network. The main histological differential diagnosis of HB is metastatic clear cell carcinoma. Additionally, because of the cystic mural features, pilocytic astrocytomas of the cerebellum must be separated from haemangioblastomas

Keywords

hemangioblastoma, von Hippel-Lindau, cantral nervous system, histopathology

Serebellar Hemangioblastoma: Dört Olgu Sunumu ve Literatür Incelemesi

Abstract

Hemanjiyoblastoma; histolojik kökeni henüz tam olarak tanımlanmamış, iyi huylu, yavaş büyüyen ve oldukça damarsal bir tümördür. Bu tümörler tüm kafatası içi neoplazmaların %1-2’sini oluşturur ki genellikle posterior fossada meydana gelirler. Hemanjiyoblastomalar normalde belirli aralıklarla düzensiz şekillerde meydana gelmektedirler ancak bu oran von hippel-lindau (VHL)’li vakaların yaklaşık %20-30’unda gözlenmektedirler. Burada Von Hippel-Lindau ile ilişkili olmayan dört serebellar hemanjiyoblastoma vakasının sunumu ve ilgili literatürün derlemesi yapıldı. Merkezi sinir sisteminde yer alan 4 hemanjiyoblastoma vakası hematoksilin-eosin, retikülin boya ve CD34, vimentin, NSE, S-100, CD99, CD56, GFAP, sitokeratin, epitelyal membran antijeni (EMA) ve CD10 antibiyotiklerini içeren bir panel ile incelemeleri yapıldı. Üç kadın bir erkek toplam 4 hastanın yaş ortalaması 54.75 (46 -60) idi. Hastalarda 2-3 cm çapında sistik nodüller vardı. Histopatolojik incelemeler sonucunda tümör kesitleri bizlere, büyük ve vaküollenmiş stromal Olgu Sunumu / Case Report 184 Cilt/Volume 40 Yıl/Year 2015 Cerebellar Hemangioblastoma hücreleri ve çok sayıda dallanma gösteren ve bir kıl kadar ince kan damarları olduğunu gösterdi. Tümörlerin bazıları atipikal çekirdek oluşumunu ortaya çıkardı. Vimentin tümörlerin hem stromal hücrelerinde hem de kan damarlarında yüksek seviyelerde pozitiflik gösterdi. Dört HB vakasında bazı stromal hücreler NSE ve CD99 için pozitifdi. Üç tümör S- 100 ve CD56 için pozitifti. İki tümör odaksal olarak glial fibriler asidik protein (GFAP) için pozitifti. Tümör stromal hücrelerinin negatif olmalarının aksin CD34 immün boyama, dallanan ve kompleks vasküler bağlantının varlığını gösterdi. Stromal hücreler sitokeratin, EMA ve CD10 gibi epitelyal işaretçiler için negatifti. Ki-67 indeksi tümör hücrelerinin %1’inden daha azdı. Nadir, histogenezisi tam olarak belli olmayan, iyi huylu tümör olan hemanjiyoblastoma; vakuollenmiş, lipid içeren hücreler ve iyi gelişmiş kapiller bağlantıların varlığı ile karakterize edilmektedir. HB’nin temel histolojik olarak ayrımlı teşhisi; metastatik açık hücre karsinomasıdır. İlaveten, sistik mural özelliklerinden dolayı serebellumun pilositik astrositomları hemanjiyoblastomlardan ayrılması gerekmektedir

Keywords

Hemanjiyoblastoma, histopatoloji, merkezi sinir sistemi, von Hippel-Lindau

References

• vessels of the brain: angiomatous malformations and
• hemangioblastomas. Springfield: Charles C Thomas, 1928:105-9. 2. Lindau A. Discussion on vascular tumors of the brain
• and spinal cord. Proc R Soc Med. 1931;24:363–70. 3. Richard S, Martin S, David P. Von Hippel-Lindau 4. Resche F, Moisan JP, Mantoura J, de Kersaint-Gilly
• A, Andre MJ, Perrin-Resche I, et al.
• Haemangioblastoma, haemangioblastomatosis, and
• von Hippel-Lindau disease. Advances and technical
• standards in neurosurgery. 1993;20:197-304. Epub 1993/01/01. 5. Neumann HP, Eggert HR, Weigel K, Friedburg H,
• Wiestler OD, Schollmeyer P: Hemangioblastomas of
• the central nervous system. A 10- year study with
• special reference to von Hippel-Lindau syndrome. J
• Neurosurg. 1989,70:24-30. 6. Conway JE, Chou D, Clatterbuck RE, Brem H, Long
• DM, Rigamonti D: Hemangioblastomas of the central
• nervous system in von Hippel-Lindau syndrome and
• sporadic disease. Neurosurgery. 2001;48:55-63. 7. 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 8. Vater GE. Hemangioblastoma. In: Neuro-oncology.
• edn. Philadelphia PA: Elsevier Saunders. 2005:294- 300. 9. WHO Classification of Tumours of the Central
• Nervous System. 4 ed. Lyon: International Agency for
• Research on Cancer. 2007:309. 10. Ho VB1, Smirniotopoulos JG, Murphy FM, Rushing
• EJ Radiologic-pathologic correlation:
• hemangioblastoma. Am J Neuroradiol. 1992;13:1343- 52 11. Constans JP, Meder F, Maiuri F, Donzelli R,
• Spaziante R, de Divitiis E. Posterior fossa
• hemangioblastomas. Surg Neurol. 1986;25:269-75. 12. Jagannathan J, Lonser RR, Smith R, DeVroom HL,
• Oldfield EH. Surgical management of cerebellar hemangioblastomas in patients with von Hippel
• Lindau disease. Journal of neurosurgery.
• 2008;108:210-22. 13. Bohling T, Haltia M, Rosenlof K, Fyhrquist F:
• Erythropoietin in capillary hemangioblastomas. Acta
• Neuropathol (Berl) 1987;74:324-32 14. Kawamura J., Garcia J.H., Kamijyo Y. Cerebellar
• hemangioblastoma: histogenesis of stroma cells.
• Cancer. 1973;31:1528–40. 15. Del Basso De Caro M., De Stefano V., Bucciero A.
• et al. [Solitary capillary hemangioblastoma, cellular
• variant. Clinical, radiological, and anatomo
• pathological study of 2 cases]. Pathologica.
• 1995;8:518–21. 16. Deck H.N. and Rubinstein L.J. Glial Fibrillary Acidic
• Protein in Stromal cells of some capillary 26. Böhling T, Hatva E, Kujala M. Expression of growth
• hemangioblastomas: significance and possible
• implications of an immunoperoxidase study. Acta
• Neuropathol. (Berl.) 1981;54:173-81. 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
• research. 1998;58:504-8. 18. 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. Human pathology.
• 1997;28:540-43. 19. Plate KH, Vortmeyer AO, Zagzag D, et al. Von
• Hippel-Lindau disease and haemangioblastoma. In:
• Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, eds.
• WHO Classification of Tumours of the Central
• Nervous System. 4th ed. Lyon: IARC. 2007:215-7. 20. Chaudhry A.O., Montes M. and Cohn G.A. .
• Ultrastructure of cerebellar hemangioblastoma.
• Cancer 1978;42:1834-50. 21. Ho K.L. Ultrastructure of cerebellar capillary
• haemangioblastoma. 1. Weibel-palade bodies and
• stromal cell histogenesis. J. Neuropathol. Exp.
• Neurol. 1984;43:592-608. 22. Bohling T, Maenpaa A, Timonen T, Vantunen L,
• Paetau A, Haltia M. Different expression of adhesion molecules on stromal cells and endothelial cells of capillary hemangioblastoma. Acta neuropathologica.
• 1996;92:461-6. 23. Wizigmann-Voos S, Plate KH. Pathology, genetics
• and cell biology of hemangioblastomas. Histology
• and histopathology. 1996;11:1049-61. 24. Ishizawa K, Komori T, Hirose T. Stromal cells in
• hemangioblastoma: neuroectodermal differentiation
• and morphological similarities to ependymoma.
• Pathology international. 2005;55:377-85. 25. Bohling T, Turunen O, Jaaskelainen J, Carpen O,
• Sainio M, Wahlstrom T, et al. Ezrin expression in
• stromal cells of capillary hemangioblastoma. An
• immunohistochemical survey of brain tumors. The
• American journal of pathology. 1996;148:367-73.
• factors and growth factor receptors in capillary
• hemangioblastoma. J Neuropathol Exp Neurol
• 1996;55:522-7. 27. Becker I., Paulus W., Roggendorf W. (1989)
• Histogenesis of stromal cells in cerebellar
• hemangioblastomas. An immunohistochemica study.
• Am. J. Pathol. 1989;134:271–5. 28. Kepes J., Rengachary S.S. and Lee S.H. (1979).
• Astrocytes in hemangioblastomas of the central
• nervous system and their relationship with stromal
• cells. Acta Neuropathol. (Berl.) 1979;47:99-104. 29. Grossniklaus H.E., Thomas J.W., Vigneswaran N.,
• Jarrett W.H., III Retinal hemangioblastoma. A
• histologic, immunohistochemical, and ultrastructural
• evaluation. Ophthalmology 1992;99;140–45. 30. Nemes Z (1992) Fibrohistiocytic differentiation in
• capillary hemangioblastoma. Hum. Pathol.
• 1992;23:805–10. 31. McComb R.D., Eastman P.J., Hahan F.J. and
• Bennett D.R.. Cerebellar haemangioblastoma with
• prominent stromal astrocytosis: diagnostic and
• histogenetic considerations. Clin. Neuropathol.
• 1987;6:149-54. 32. Andrew S. and Gradwell E.. lmmunoperoxidase
• labelled antibody staining in differential diagnosis of
• central nervous system haemangioblastomas and
• central nervous system metastases of renal
• carcinomas. J. Clin. Pathol. 1986;30:917-19. 33. Gouldesbrough D.R., Bell J.E. and Gordon A. Use of immunohistochemical methods in the differential diagnosis between primary cerebellar haemangioblastoma and metastatic renal carcinoma.
• J. Clin. Pathol. 1988;41:861-65. 34. Zaucha R. & Welnicka-Jaskiewicz M. (1996)
• [Hemangioblastoma of the breast]. Pol. Arch. Med.
• Wewn. 1986;95:145–6. 35. Boyd A.S. & Zhang J. Hemangioblastoma arising in
• the skin. Am. J. Dermatopathol. 2001;23:482–84. 36. Fanburg-Smith J.C., Gyure K.A., Michal M., Katz D.,
• Thompson L.D. Retroperitoneal peripheral
• hemangioblastoma: a case report and review of the
• literature. Ann. Diagn. Pathol. 2000;4:81–7. 37. Tender G.C., Butman J.A., Oldfield E.H., Lonser
• R.R. (2004) Thoracic spinal nerve
• hemangioblastoma. Case illustration. J. Neurosurg.
• Spine 2004;1:142. 38. Rojiani A.M., Owen D.A., Berry K. et al. Hepatic hemangioblastoma. An unusual presentation in a patient with von Hippel-Lindau disease. Am. J. Surg.
• Pathol. 1991;15 81–6. 39. Poussa K, Sankila R, Haapasalo H, Kaariainen H,
• Pukkala E, Jaaskelainen J. Long-term prognosis of
• haemangioblastoma of the CNS: impact of von
• Hippel-Lindau disease. Acta Neurochir (Wien).
• 1999;141:1147-56. 40. de la Monte S.M. & Horowitz S.A.
• Hemangioblastomas: clinical and histopathological
• factors correlated with recurrence. Neurosurgery.
• 1989;25:695–8. 41. Hasselblatt M., Jeibmann A., Gerss J. et al. Cellular
• and reticular variants of haemangioblastoma
• revisited: a clinicopathologic study of 88 cases.
• Neuropathol. Appl. Neurobiol. 2005;31:618–22