Skip to main content
PMC home page
Journal of Clinical Pathology logoLink to Journal of Clinical Pathology
. 1985 Apr;38(4):417–421. doi: 10.1136/jcp.38.4.417

Histogenesis of haemangioblastomas: an immunocytochemical and ultrastructural study in a case of von Hippel-Lindau syndrome.

S M Ismail, B Jasani, G Cole
PMCID: PMC499168  PMID: 2859302

Abstract

The cerebellar, retinal, and one of the spinal haemangioblastomas in a case of von Hippel-Lindau syndrome were studied by immunocytochemistry and electron microscopy. The tumours were positive for neurone specific enolase and variably positive for somatostatin, pancreatic polypeptide, and bombesin. Electron microscopy of the cerebellar tumour showed secretory granules with an average diameter of 170 nm. This report is believed to be the first description of neurone specific enolase positivity and polypeptide hormones within the intervascular cells of haemangioblastomas. In the light of these findings it is suggested that haemangioblastomas are tumours of neuroectodermal origin, derived either from neural or neuroendocrine cells.

Full text

PDF
417

Images in this article

418Image
on p.418
419Image
on p.419
420Image
on p.420

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Andrioli G. C., Scanarini M., Iob I. Intrinsic haematopoietic activity of cerebellar haemangioblastomas. Ultrastructural study of three cases. Neurochirurgia (Stuttg) 1979 Jan;22(1):24–28. doi: 10.1055/s-0028-1090283. [DOI] [PubMed] [Google Scholar]
  2. Bishop A. E., Polak J. M., Facer P., Ferri G. L., Marangos P. J., Pearse A. G. Neuron specific enolase: a common marker for the endocrine cells and innervation of the gut and pancreas. Gastroenterology. 1982 Oct;83(4):902–915. [PubMed] [Google Scholar]
  3. CHAPMAN R. C., DIAZ-PEREZ R. Pheochromocytoma associated with cerebellar hemangioblastoma. Familial occurrence. JAMA. 1962 Dec 8;182:1014–1017. doi: 10.1001/jama.1962.03050490038008. [DOI] [PubMed] [Google Scholar]
  4. CRAMER F., KIMSEY M. W. The cerebellar hemangioblastomas; review of fifty-three cases with special reference to cerebellar cysts and the association of polycythemia. AMA Arch Neurol Psychiatry. 1952 Feb;67(2):237–252. [PubMed] [Google Scholar]
  5. Graber S. E., Krantz S. B. Erythropoietin and the control of red cell production. Annu Rev Med. 1978;29:51–66. doi: 10.1146/annurev.me.29.020178.000411. [DOI] [PubMed] [Google Scholar]
  6. Horton W. A., Wong V., Eldridge R. Von Hippel-Lindau disease: clinical and pathological manifestations in nine families with 50 affected members. Arch Intern Med. 1976 Jul;136(7):769–777. doi: 10.1001/archinte.136.7.769. [DOI] [PubMed] [Google Scholar]
  7. Hull M. T., Warfel K. A., Muller J., Higgins J. T. Familial islet cell tumors in Von Hippel-Lindau's disease. Cancer. 1979 Oct;44(4):1523–1526. doi: 10.1002/1097-0142(197910)44:4<1523::aid-cncr2820440452>3.0.co;2-0. [DOI] [PubMed] [Google Scholar]
  8. Hökfelt T., Johansson O., Ljungdahl A., Lundberg J. M., Schultzberg M. Peptidergic neurones. Nature. 1980 Apr 10;284(5756):515–521. doi: 10.1038/284515a0. [DOI] [PubMed] [Google Scholar]
  9. Ismail S. M., Cole G. Von Hippel-Lindau syndrome with microscopic hemangioblastomas of the spinal nerve roots. Case report. J Neurosurg. 1984 Jun;60(6):1279–1281. doi: 10.3171/jns.1984.60.6.1279. [DOI] [PubMed] [Google Scholar]
  10. Jakobiec F. A., Font R. L., Johnson F. B. Angiomatosis retinae. An ultrastructural study and lipid analysis. Cancer. 1976 Nov;38(5):2042–2056. doi: 10.1002/1097-0142(197611)38:5<2042::aid-cncr2820380529>3.0.co;2-o. [DOI] [PubMed] [Google Scholar]
  11. Jeffreys R. V., Napier J. A., Reynolds S. H. Erythropoietin levels in posterior fossa haemangioblastoma. J Neurol Neurosurg Psychiatry. 1982 Mar;45(3):264–266. doi: 10.1136/jnnp.45.3.264. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Jurco S., 3rd, Nadji M., Harvey D. G., Parker J. C., Jr, Font R. L., Morales A. R. Hemangioblastomas: histogenesis of the stromal cell studied by immunocytochemistry. Hum Pathol. 1982 Jan;13(1):13–18. doi: 10.1016/s0046-8177(82)80133-0. [DOI] [PubMed] [Google Scholar]
  13. Krieger D. T., Martin J. B. Brain peptides (first of two parts). N Engl J Med. 1981 Apr 9;304(15):876–885. doi: 10.1056/NEJM198104093041505. [DOI] [PubMed] [Google Scholar]
  14. MELMON K. L., ROSEN S. W. LINDAU'S DISEASE. REVIEW OF THE LITERATURE AND STUDY OF A LARGE KINDRED. Am J Med. 1964 Apr;36:595–617. doi: 10.1016/0002-9343(64)90107-x. [DOI] [PubMed] [Google Scholar]
  15. Mohan J., Brownell B., Oppenheimer D. R. Malignant spread of haemangioblastoma: report on two cases. J Neurol Neurosurg Psychiatry. 1976 Jun;39(6):515–525. doi: 10.1136/jnnp.39.6.515. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. OLIVECRONA H. The cerebellar angioreticulomas. J Neurosurg. 1952 Jul;9(4):317–330. doi: 10.3171/jns.1952.9.4.0317. [DOI] [PubMed] [Google Scholar]
  17. Probst A., Lotz M., Heitz P. Von Hippel-Lindau's Disease, syringomyelia and multiple endocrine tumors: a complex neuroendocrinopathy. Virchows Arch A Pathol Anat Histol. 1978 Jun 15;378(3):265–272. doi: 10.1007/BF00427365. [DOI] [PubMed] [Google Scholar]
  18. Royds J. A., Parsons M. A., Taylor C. B., Timperley W. R. Enolase isoenzyme distribution in the human brain and its tumours. J Pathol. 1982 May;137(1):37–49. doi: 10.1002/path.1711370105. [DOI] [PubMed] [Google Scholar]
  19. Sander S., Normann T., Mathisen W. Pheochromocytoma associated with von Hippel-Lindau's disease in a family. Scand J Urol Nephrol. 1970;4(3):259–263. doi: 10.3109/00365597009137605. [DOI] [PubMed] [Google Scholar]
  20. Schmechel D., Marangos P. J., Brightman M. Neurone-specific enolase is a molecular marker for peripheral and central neuroendocrine cells. Nature. 1978 Dec 21;276(5690):834–836. doi: 10.1038/276834a0. [DOI] [PubMed] [Google Scholar]
  21. Schmechel D., Marangos P. J., Zis A. P., Brightman M., Goodwin F. K. Brain endolases as specific markers of neuronal and glial cells. Science. 1978 Jan 20;199(4326):313–315. doi: 10.1126/science.339349. [DOI] [PubMed] [Google Scholar]
  22. Tapia F. J., Polak J. M., Barbosa A. J., Bloom S. R., Marangos P. J., Dermody C., Pearse A. G. Neuron-specific enolase is produced by neuroendocrine tumours. Lancet. 1981 Apr 11;1(8224):808–811. doi: 10.1016/s0140-6736(81)92682-9. [DOI] [PubMed] [Google Scholar]
  23. Vincent S. R., Johansson O., Hökfelt T., Meyerson B., Sachs C., Elde R. P., Terenius L., Kimmel J. Neuropeptide coexistence in human cortical neurones. Nature. 1982 Jul 1;298(5869):65–67. doi: 10.1038/298065a0. [DOI] [PubMed] [Google Scholar]

Articles from Journal of Clinical Pathology are provided here courtesy of BMJ Publishing Group

RESOURCES