Skip to main content
SpringerLink
Log in

Choroidal ganglioneuroma in neurofibromatosis

  • Published:
Graefe's Archive for Clinical and Experimental Ophthalmology Aims and scope Submit manuscript

Abstract

A twenty-one year old female with previously unsuspected neurofibromatosis presented for evaluation of a blind painful eye. Histopathologic examination of the enucleated specimen revealed choroidal thickening with ovoid bodies and proliferation of connective tissue with pigment-containing cells and ganglion-like cells. Electron microscopic study of the latter cell population revealed typical morphologic features of ganglion cells, including numerous electron-dense intracellular granules and an abundance of mitochondria and rough endoplasmic reticulum. These pathologic findings were interpreted as consistent with the diagnosis of choroidal ganglioneuroma occurring in the context of ocular neurofibromatosis. The literature concerning this unusual tumor is reviewed and the possible relationship of this lesion to neurofibromatosis and other disorders of neural cresent proliferation are briefly discussed.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Woog JJ, Albert DM, Solt LC, Hu DN, Wang WJ (in press) Neurofibromatosis of the eyelid and orbit. International Ophthalmology Clinics

  2. Kobrin JL, Blodi FC, Weingeist TA (1979) Ocular and Orbital Manifestations of Neurofibromatosis. Surv Ophthalmol 24: 45

    CAS  PubMed  Google Scholar 

  3. Bender BL, Ghatak NR (1978) Light and electron microscopic observations on a ganglineuroma. Acta Neuropathologic observations on a ganglioneuroma. Acta Neuropathologica (Berl) 42(1): 7–10

    CAS  Google Scholar 

  4. Kramer W (1970) Tumors of Nerves, in Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology, Vol 8, New York, American Elsevier Publishing Company, Inc., pp 459–465

    Google Scholar 

  5. Carpenter WB, Kennohan JW (1963) Retroperitoneal ganglioneuromas and neurofibromas. A clinicopathological study. Cancer 16: 788

    CAS  PubMed  Google Scholar 

  6. Loritz W (1870) Ein Fall von gangliosen Neurom (Ganglion). Virchows Arch Path Anat 49: 435–441

    Google Scholar 

  7. Cogan DC, Poppen JL, Hicks SP (1961) Ganglioneuroma of the chiasm and optic nerves. Arch Ophthalmol 65: 481

    CAS  PubMed  Google Scholar 

  8. McCrea WBE (1952) A case of ganglioneuroma of the retina. Trans Ophthalmol Soc UK 72: 235

    Google Scholar 

  9. Montepellier J, Tarlant P, Foissini J (1939) Ganglioneuroschwannospongioblastome de la cavite orbitawe. Ann d'oculistique 176: 18

    Google Scholar 

  10. McCrae D Jr, Shaw A (1979) Ganglioneuroma, heterochromia iridis, and Horner's syndrome. J Pediatr Surg 14(15): 612

    Google Scholar 

  11. Potter EL, Parrish JM (1942) Neuroblastoma, ganglioneuroma, and fibroneuroma in a stillborn fetus. Am J Path 18: 141

    Google Scholar 

  12. Chalten J, Voorhess ML (1967) Familial neuroblastoma — report of a kindred with multiple disorders. NEJM 277: 1230

    Google Scholar 

  13. Gorlin RJ, Sedan HO, Vickers RA, Cervenka J (1963) Multiple mucosal neuromas, pheochromocytoma, and medullary carcinoma of the thyroid — a syndrome. Cancer 22: 293

    Google Scholar 

  14. Bolande RP, Towler WF (Jul 1970) A possible relationship of neuroblastoma to von Recklinghausen's disease. Cancer 26: 162

    Google Scholar 

  15. Stout AP (1947) Ganglioneuroma of the sympathetic nervous system. SGO 84: 101–116

    Google Scholar 

  16. Dahl EV, Waugh JM, Dahlin DC (1953) GI ganglioneuromas — brief review with report of duodenal ganglioneuroma. Am J Pathol 33: 953

    Google Scholar 

  17. Sinclair JE, Yang YH (1961) Ganglioneuromata of the spine associated with von Recklinghausen's disease. J Neurosurg 18: 115

    Google Scholar 

  18. Gill BS (1965) Neurofibromatosis with ganglioneuromatosis of the pharynx. J Laryng 79: 1093

    CAS  PubMed  Google Scholar 

  19. Dublin WB (1954) Fundamentals of Neuropathology. Springfield, Illinois, Charles C. Thomas, p 685

    Google Scholar 

  20. Russell DS, Rubinstein LJ (1963) Pathology of tumors of the nervous system. Baltimore, William and Wilkins Co., pp 270–271

    Google Scholar 

  21. Knight M (1925) A critical survey of neoplasms of the choroid. Am J Ophthalmol 8: 791

    Google Scholar 

  22. Wolter JR (1965) Nerve fibrils in ovoid bodies. Arch Ophthalmol 73: 696

    CAS  PubMed  Google Scholar 

  23. Wheeler JM (1936) Plexiform neurofibromatosis involving the choroid, ciliary body, and other structures. Trans Am Ophthalmol Soc 34: 151

    Google Scholar 

  24. Wolter JR, Bryson JM, McKee TP (1965) Ganglioneuromelanocytosis of the choroid. Arch Ophthalmol 74: 353

    CAS  PubMed  Google Scholar 

  25. Plamondon M, Augen C (1953) Ganglioneurome melanique de la choroid oculaire au cours d'une maladie de von Recklinghausen. Union Medicale du Canada 82: 1368

    CAS  PubMed  Google Scholar 

  26. Berger L, Vaillancourt J (1934) Bull de 1'Ass. fr. p. l'etude du Cancer 23: 275

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Harvard Medical School, Massachusetts Eye and Ear Infirmary, Massachusetts

    J. J. Woog & D. M. Albert

  2. Massachusetts Eye and Ear Infirmary, 243 Charles Street, 02114, Boston, MA, USA

    D. M. Albert & J. Craft

  3. Mercy Hospital, Des Moines, Iowa, USA

    N. Silberman

  4. Minneapolis, Minnesota, USA

    D. Horns

Authors
  1. J. J. Woog
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. D. M. Albert
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. J. Craft
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. N. Silberman
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. D. Horns
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Woog, J.J., Albert, D.M., Craft, J. et al. Choroidal ganglioneuroma in neurofibromatosis. Graefe's Arch Clin Exp Ophthalmol 220, 25–31 (1983). https://doi.org/10.1007/BF02307012

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02307012

Keywords

Search

Navigation