Skip to main content

Selective Pathology of Intraocular Tumors

  • Chapter
  • First Online:
Oncological Surgical Pathology

Abstract

Retinoblastoma represents the most common intraocular malignant tumor in children and worldwide is the most common intraocular malignancy. Uveal melanomas are the most common malignant intraocular tumors in adults. This review chapter includes key clinical features and important histopathologic findings for diagnosis and prognosis of both entities.

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

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 219.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 279.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Nork TM, Schwartz TL, Doshi HM, Millecchia LL. Retinoblastoma. Cell of origin. Arch Ophthalmol. 1995;113:791–802.

    Article  CAS  Google Scholar 

  2. Albert DM. Wardrop Lecture, 1974. James Wardrop: a brief review of his life and contributions. Trans Ophthalmol Soc UK. 1974;94:892–908.

    PubMed  CAS  Google Scholar 

  3. Albert DM. Historic review of retinoblastoma. Ophthalmology. 1987;94:654–62.

    Article  CAS  Google Scholar 

  4. Gallie BL, Phillips RA, Ellsworth RM, et al. Significance of retinoma and phthisis bulbi for retinoblastoma. Ophthalmology. 1982;89:1393–9.

    Article  CAS  Google Scholar 

  5. McLean IW. Retinoblastoma, retinocytomas, and pseudoretinoblastomas. In: Spencer WH, editor. Ophthalmic pathology. An atlas and textbook. Philadelphia: American Academy of Ophthalmology/WB Saunders Company; 1990. p. 1332–438.

    Google Scholar 

  6. Zhang J, Schweers B, Dyer MA. The first knockout mouse model of retinoblastoma. Cell Cycle. 2004;3(7):952–9.

    Article  CAS  Google Scholar 

  7. Squire J, Gallie BL, Phillips RA. A detailed analysis of chromosomal changes in heritable and non- heritable retinoblastoma. Hum Genet. 1985;70:291–301.

    Article  CAS  Google Scholar 

  8. Classon M, Harlow E. The retinoblastoma tumor suppressor in development and cancer. Nat Rev Cancer. 2002;2:910–7.

    Article  CAS  Google Scholar 

  9. Skalet AH, Gombos DS, Gallie BL, Kim JW, Shields CL, Marr BP, Plon SE, Chevez-Barrios P. Screening children at risk for retinoblastoma: consensus report from the American Association of Ophthalmic Oncologists and Pathologists. Ophthalmology. 2018;125(3):453–8.

    Article  Google Scholar 

  10. Sastre X, Chantada GL, Doz F, Wilson MW, de Davila MT, Rodríguez-Galindo C, Chintagumpala M, Chévez-Barrios P, International Retinoblastoma Staging Working Group. Proceedings of the consensus meetings from the International Retinoblastoma Staging Working Group on the pathology guidelines for the examination of enucleated eyes and evaluation of prognostic risk factors in retinoblastoma. Arch Pathol Lab Med. 2009;133(8):1199–202. https://doi.org/10.1043/1543-2165-133.8.1199.

    Article  PubMed  Google Scholar 

  11. Hurwitz RL, Chévez-Barrios P, Chintagumpala M, Shields C, Shields J. Retinoblastoma. In: Pizzo PA, Poplack D, editors. Principles and practice of pediatric oncology. 6th ed. Philadelphia: Lippincott-Raven; 2002. p. 825–46.

    Google Scholar 

  12. Wong FL, Boice JD Jr, Abramson DH, et al. Cancer incidence after retinoblastoma: radiation dose and sarcoma risk. JAMA. 1997;278:1262–7.

    Article  CAS  Google Scholar 

  13. Chantada GL, Doz F, Orjuela M, Qaddoumi I, Sitorus RS, Kepak T, Furmanchuk A, Castellanos M, Sharma T, Chevez-Barrios P, Rodriguez-Galindo C, International Retinoblastoma Staging Working Group. World disparities in risk definition and management of retinoblastoma: a report from the International Retinoblastoma Staging Working Group. Pediatr Blood Cancer. 2008;50(3):692–4.

    Article  Google Scholar 

  14. Ghisoli ML, Zage PE, Herzog C, Chevez-Barrios P, Chintagumpala M, Hurwitz RL, Gombos DS. Multidisciplinary management of retinoblastoma: diagnosis, treatment, and future direction. Ophthalmic Oncol. 2011:179.

    Google Scholar 

  15. Chintagumpala M, Chevez-Barrios P, Paysse EA, Plon SE, Hurwitz R. Retinoblastoma: review of current management. Oncologist. 2007 Oct;12(10):1237–46.

    Article  Google Scholar 

  16. Gombos DS, Chevez-Barrios AP. Current treatment and management of retinoblastoma. Curr Oncol Rep. 2007 Nov;9(6):453–8.

    Article  Google Scholar 

  17. Chévez-Barrios P, Eagle R, Marback E. Histopathologic features and prognostic factors. Clin Ophthalmic Oncol Retinoblastoma [serial online]. 2015:167.

    Google Scholar 

  18. Chévez-Barrios P, Eagle RC, Marbach. Histological features and risks for metastatic spread of retinoblastoma. In: Singh AD, Damato B, Pe’er J, Murphree AL, Perry JD, editors. Clinical Ocular Oncology. 1st ed. London: Elsevier/Saunders; 2007. Section 6–77.

    Google Scholar 

  19. Chong EM, Coffee RE, Chintagumpala M, Hurwitz RL, Hurwitz MY, Chévez-Barrios P. Extensively necrotic retinoblastoma is associated with high-risk prognostic factors. Arch Pathol Lab Med. 2006 Nov;130(11):1669–72.

    Article  Google Scholar 

  20. Shields CL, Shields JA, Baez K, et al. Optic nerve invasion of retinoblastoma: metastatic potential and clinical risk factors. Cancer. 1994;73:692–8.

    Article  CAS  Google Scholar 

  21. Magramm I, Abramson DH, Ellsworth RM. Optic nerve involvement in retinoblastoma. Ophthalmology. 1989;96:217–22.

    Article  CAS  Google Scholar 

  22. Chantada GL, de Davila MT, Fandino A, et al. Retinoblastoma with low risk for extraocular relapse. Ophthalmic Genet. 1999;20:133–40.

    Article  CAS  Google Scholar 

  23. Retinoblastoma. In: Amin MB, Edge S, Greene F, Byrd DR. Brookland RK, Washingont ML. Gershenwald JE, Compton CC, Hess KR, Sullivan DC, Jessup JM, Brierley JD, Gaspar LE, Schilsky RL, Balch CM, Winchester DP, Asare EA, Madera M, Gress DM, Meyer LR, editors. AJCC Cancer Staging Manual. 8th ed. New York: Springer; 2017. Chapter 68. p. 819–831.

    Google Scholar 

  24. Amram AL, Rico G, Kim JW, Chintagumpala M, Herzog CE, Gombos DS, Chévez-Barrios P. Vitreous seeds in retinoblastoma. Clinicopathologic classification and correlation. Ophthalmology. 2017;124:1540–7.

    Article  Google Scholar 

  25. Singh AD, Topham A. Incidence of uveal melanoma in the United States: 1973–1997. Ophthalmology. 2003;11(5):956–61.

    Article  Google Scholar 

  26. Singh AD, Bergman L, Seregard S. Uveal melanoma: epidemiologic aspects. Ophthalmol Clin N Am. 2005;18(1):75–84.

    Article  Google Scholar 

  27. Kujala E, Damato B, Coupland SE, Desjardins L, Bechrakis NE, Grange JD, Kivelä T. Staging of ciliary body and choroidal melanomas based on anatomic extent. J Clin Oncol. 2013;31(22):2825–31.

    Article  Google Scholar 

  28. Haugh AM, Njauw CN, Bubley JA, Verzì AE, Zhang B, Kudalkar E, VandenBoom T, Walton K, Swick BL, Kumar R, Rana HQ, Cochrane S, McCormick SR, Shea CR, Tsao H, Gerami P. Genotypic and phenotypic features of BAP1 cancer syndrome: a report of 8 new families and review of cases in the literature. JAMA Dermatol. 2017;153(10):609–18.

    Article  Google Scholar 

  29. Kaliki S, Shields CL, Shields JA. Uveal melanoma: estimating prognosis. Indian J Ophthalmol. 2015;63(2):93–102.

    Article  Google Scholar 

  30. McLean IW, Foster WD, Zimmerman LE, Gamel JW. Modifications of Callender’s classification of uveal melanoma at the Armed Forces Institute of Pathology. Am J Ophthalmol. 1983;96(4):502–9.

    Article  CAS  Google Scholar 

  31. Zhao M, Mu Y, Dang Y, Zhu Y. Secondary glaucoma as initial manifestation of ring melanoma: a case report and review of literature. Int J Clin Exp Pathol. 2014;7(11):8163–9.

    PubMed  PubMed Central  Google Scholar 

  32. Burris CK, Rodriguez ME, Raven ML. Melanomalytic Glaucoma. Ophthalmology. 2015;122(12):2422.

    Article  Google Scholar 

  33. Rummelt V, Folberg R, Woolson RF, Hwang T, Pe'er J. Relation between the microcirculation architecture and the aggressive behavior of ciliary body melanomas. Ophthalmology. 1995;102(5):844–51.

    Article  CAS  Google Scholar 

  34. Folberg R, Rummelt V, Parys-Van Ginderdeuren R, Hwang T, Woolson RF, Pe'er J, Gruman LM. The prognostic value of tumor blood vessel morphology in primary uveal melanoma. Ophthalmology. 1993;100(9):1389–98.

    Article  CAS  Google Scholar 

  35. Tschentscher F, Hüsing J, Hölter T, Kruse E, Dresen IG, Jöckel KH, Anastassiou G, Schilling H, Bornfeld N, Horsthemke B, Lohmann DR, Zeschnigk M. Tumor classification based on gene expression profiling shows that uveal melanomas with and without monosomy 3 represent two distinct entities. Cancer Res. 2003;63(10):2578–84.

    PubMed  CAS  Google Scholar 

  36. Onken MD, Worley LA, Ehlers JP, Harbour JW. Gene expression profiling in uveal melanoma reveals two molecular classes and predicts metastatic death. Cancer Res. 2004;64(20):7205–9.

    Article  CAS  Google Scholar 

  37. Mäkitie T, Summanen P, Tarkkanen A, Kivelä T. Tumor-infiltrating macrophages (CD68(+) cells) and prognosis in malignant uveal melanoma. Invest Ophthalmol Vis Sci. 2001;42(7):1414–21.

    PubMed  Google Scholar 

  38. Field MG, Decatur CL, Kurtenbach S, Gezgin G, van der Velden PA, Jager MJ, Kozak KN, Harbour JW. PRAME as an independent biomarker for metastasis in uveal melanoma. Clin Cancer Res. 2016;22(5):1234–42.

    Article  CAS  Google Scholar 

  39. The Collaborative Ocular Melanoma Study (COMS) randomized trial of preenucleation radiation of large choroidal melanoma I: characteristics of patients enrolled and not enrolled. COMS report no. 9. Am J Ophthalmol. 1998;125(6):767.

    Google Scholar 

  40. Li W, Gragoudas ES, Egan KM. Metastatic melanoma death rates by anatomic site after proton beam irradiation for uveal melanoma. Arch Ophthalmol. 2000;118(8):1066–70.

    Article  CAS  Google Scholar 

  41. Krema H, Simpson ER, Pavlin CJ, Payne D, Vasquez LM, McGowan H. Management of ciliary body melanoma with iodine-125 plaque brachytherapy. Can J Ophthalmol. 2009;44(4):395–400.

    Article  Google Scholar 

  42. International Validation of the American Joint Committee on Cancer’s 7th Edition Classification of Uveal Melanoma. JAMA Ophthalmol. 2015;133(4):376–83.

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Patricia Chévez-Barrios .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Chévez-Barrios, P., Cernichiaro Espinosa, L.A. (2020). Selective Pathology of Intraocular Tumors. In: Moran, C.A., Kalhor, N., Weissferdt, A. (eds) Oncological Surgical Pathology . Springer, Cham. https://doi.org/10.1007/978-3-319-96681-6_4

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-96681-6_4

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-96680-9

  • Online ISBN: 978-3-319-96681-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics