eMedicine Specialties > Ophthalmology > Choroid
Melanoma, Choroidal: Differential Diagnoses & Workup
Updated: Jun 24, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Differential Diagnoses
Other Problems to Be Considered
Benign and malignant tumors, cysts, and other abnormal masses in the choroid, retina, and pigment epithelium must be distinguished from choroidal melanomas.
Melanocytic nevus
Melanocytoma
Metastatic tumors
Medulloepithelioma (diktyoma)
Choroidal osteoma
Adenoma
Adenocarcinoma
Combined hamartoma of the retina and pigment epithelium
Congenital hypertrophy and reactive hyperplasia of the retinal pigment epithelium
Retinal cavernous hemangioma
Presumed acquired retinal hemangioma
Lymphoid tumor
Hemangiopericytoma
Leiomyoma
Neurofibroma
Glioneuroma
Astrocytoma
Rhabdomyosarcoma
Posterior uveitis
Sarcoid nodules
Tubercular granuloma
Workup
Laboratory Studies
- The most common site of choroidal melanoma metastasis is the liver.
- Liver enzyme levels are indicated in any patient with uveal melanoma. The most sensitive tests of hepatic function are serum levels of alkaline phosphatase, glutamic-oxaloacetic transaminase, lactic dehydrogenase, and gamma-glutamyl transpeptidase.
- These test results are negative at the time of diagnosis in most patients with choroidal melanoma. If any of these laboratory test results are abnormal, order ultrasonographic and CT studies of the liver. Unfortunately, both imaging modalities have low sensitivity for metastasis smaller than 10-20 mm in diameter.
Imaging Studies
- A-scan ultrasound of the eye is a useful tool in tumors more than 2-3 mm in thickness. Choroidal melanoma characteristically shows an initial prominent spike, followed by low-to-medium internal reflectivity with diminishing amplitude and a significant echo. Vascular pulsations can be seen as fine oscillations of the internal spiking pattern within the tumor. Standardized ultrasonography has a diagnostic accuracy of more than 95%. Performing sequential A-scans, with accurate dimension measurements, in cases of diagnostic uncertainty is important.
- B-scan ultrasound of the eye is a routine test used in the evaluation of any posterior segment mass. It is especially needed in patients with media opacity. For choroidal melanomas, B-scan ultrasound is used to help establish the diagnosis, to evaluate possible extraocular extension, to estimate tumor size for periodic observation, and to plan therapeutic intervention.
- Intraocular melanomas have several distinctive features, to include the following:
- Low-to-medium reflectivity
- Excavation of underlying uveal tissue
- Shadowing of subjacent soft tissues
- Internal vascularity
- An acoustic quiet zone at the base of the tumor called acoustic hollowing
- Ultrasound biomicroscopy (UBM) uses high-frequency waves, with excellent resolution for anterior ocular abnormalities. It can differentiate very anterior choroidal melanomas from those of ciliary body origin and can help define the tumor's anterior border. It is also helpful in assessing angle-closure glaucoma.
- Fluorescein angiography and indocyanine green angiography do not show pathognomonic signs of choroidal melanoma but can help point to its diagnosis.
- Small choroidal melanomas may show fluorescein angiographic changes similar to some choroidal nevi, with such changes ranging from normal angiography to hypofluorescence secondary to blockage of background fluorescence.
- Larger melanomas may show a patchy pattern of early hypofluorescence and hyperfluorescence followed by late intense staining.
- Some choroidal melanomas demonstrate intrinsic vascularization, visible throughout the angiogram. The angiographic sign called the "double circulation pattern” refers to simultaneous fluorescence of retinal and choroidal circulation within the tumor. When it occurs, it is fairly distinctive of choroidal melanomas.
- CT scan of the globe and orbit is more expensive than ultrasound and is not as sensitive. It is useful to see extraocular extension and may help differentiate between choroidal or retinal detachment and a solid tumor. CT scan requires intravenous injection of contrast material. Choroidal melanoma shows enhancement with contrast, whereas exudation does not. CT scan also is sensitive in detecting calcium, a feature of some tumors that are different than uveal melanomas (characteristically choroidal osteoma).
- MRI of the globe and orbit is more expensive than CT scan. Use of surface coil imaging and gadolinium as a contrast material greatly improves its resolution, but it still remains less sensitive than ultrasound. Pigmented melanomas are seen as a high-density image in T1 and as a low-density image in T2. MRI also can be used to determine extrascleral extension of the melanoma and distinguish surrounding fluid from the tumor.
- Obtain a chest x-ray in patients with choroidal melanomas to rule out possible lung metastases.
Procedures
- Fine needle biopsy and incisional biopsy are not usually required but may be helpful in difficult diagnostic cases, particularly to distinguish amelanotic melanomas from metastatic tumors, and if results from other ancillary tests are equivocal.
- In experienced hands, both biopsy techniques have an accuracy of more than 95% in tumors larger than 3 mm.
- Incisional biopsy is more invasive and may have more associated complications, but it has less false-negative and false-positive results. The most common complication for tumor biopsy is intralesional or perilesional hemorrhage.
- Risk of spread of cancerous cells in the case of fine needle biopsy is small for choroidal melanoma (unlike retinoblastoma). Follow biopsy by prompt treatment to avoid extrascleral extension.
- Genetic analysis and karyotyping of biopsy specimens have gained increasing attention. Chromosome 3 monosomy in the choroidal tumor has been shown to be associated with a significantly greater risk of developing metastases.1 However, since no effective treatment is available for metastatic disease, the clinical impact of obtaining routine biopsy on choroidal melanoma is unclear at this point. Many experts have argued against this practice.
Histologic Findings
Histologic evaluation of the tumor after enucleation can confirm the diagnosis and evaluate prognosis. Three distinct cell types are recognized to occur in uveal melanomas, as follows: spindle A, spindle B, and epithelioid.
Spindle A cells have elongated nuclei and uncommonly have mitotic figures. Spindle B cells have a prominent nucleolus. They are found more commonly and also have an elongated profile but are slightly larger than spindle A cells. Epithelioid melanoma cells are highly anaplastic, poorly cohesive, and have considerable morphological variation. They tend to resemble epithelial cells and contain frequent mitotic figures.
The most commonly used histologic classification of uveal melanomas is the modified Callender classification. It divides uveal melanocytic tumors in several groups, as follows: spindle cell nevi, spindle cell melanomas, necrotic melanomas, epithelioid cell melanomas, and mixed cell melanomas. The latter two carry the poorest survival prognosis.
Evaluation of vascular supply of the tumor, age at presentation, presence of extrascleral extension, tumor size, tumor cell types, mitotic rate, nucleolar area, and quantification of nucleolar organizer regions have been used for prognostic purposes.
More on Melanoma, Choroidal |
| Overview: Melanoma, Choroidal |
Differential Diagnoses & Workup: Melanoma, Choroidal |
| Treatment & Medication: Melanoma, Choroidal |
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References
Prescher G, Bornfeld N, Hirche H, et al. Prognostic implications of monosomy 3 in uveal melanoma. Lancet. May 4 1996;347(9010):1222-5. [Medline].
Detorakis ET, Engstrom RE Jr, Wallace R, et al. Iris and anterior chamber angle neovascularization after iodine 125 brachytherapy for uveal melanoma. Ophthalmology. Mar 2005;112(3):505-10. [Medline].
Accuracy of diagnosis of choroidal melanomas in the Collaborative Ocular Melanoma Study. COMS report no. 1. Arch Ophthalmol. Sep 1990;108(9):1268-73. [Medline].
Augsburger JJ, Correa ZM, Freire J, et al. Long-term survival in choroidal and ciliary body melanoma after enucleation versus plaque radiation therapy. Ophthalmology. Sep 1998;105(9):1670-8. [Medline].
Augsburger JJ, Gamel JW, Sardi VF, et al. Enucleation vs cobalt plaque radiotherapy for malignant melanomas of the choroid and ciliary body. Arch Ophthalmol. May 1986;104(5):655-61. [Medline].
Augsburger JJ, Peyster RG, Markoe AM, et al. Computed tomography of posterior uveal melanomas. Arch Ophthalmol. Nov 1987;105(11):1512-6. [Medline].
Cham MC, Pavlin CJ. Ultrasound detection of posterior scleral bowing in young patients with choroidal melanoma. Can J Ophthalmol. Aug 2000;35(5):263-6. [Medline].
Chang M, Shields JA, Wachtel DL. Adenoma of the pigment epithelium of the ciliary body simulating a malignant melanoma. Am J Ophthalmol. Jul 1979;88(1):40-4. [Medline].
Char DH, Kroll S, Quivey JM, et al. Long term visual outcome of radiated uveal melanomas in eyes eligible for randomisation to enucleation versus brachytherapy. Br J Ophthalmol. Feb 1996;80(2):117-24. [Medline].
Char DH, Kroll SM, Miller T, et al. Irradiated uveal melanomas: cytopathologic correlation with prognosis. Am J Ophthalmol. Oct 1996;122(4):509-13. [Medline].
Collaborative Ocular Melanoma Study. The Collaborative Ocular Melanoma Study (COMS) randomized trial of pre- enucleation radiation of large choroidal melanoma III: local complications and observations following enucleation COMS report no. 11. Am J Ophthalmol. Sep 1998;126(3):362-72. [Medline].
Collaborative Ocular Melanoma Study. The Collaborative Ocular Melanoma Study (COMS) randomized trial of pre-enucleation radiation of large choroidal melanoma II: initial mortality findings. COMS report no. 10. Am J Ophthalmol. Jun 1998;125(6):779-96. [Medline].
Collaborative Ocular Melanoma Study Group. Factors predictive of growth and treatment of small choroidal melanoma: COMS Report No. 5. The Collaborative Ocular Melanoma Study Group. Arch Ophthalmol. Dec 1997;115(12):1537-44. [Medline].
Collaborative Ocular Melanoma Study Group. Mortality in patients with small choroidal melanoma. COMS report no. 4. The Collaborative Ocular Melanoma Study Group. Arch Ophthalmol. Jul 1997;115(7):886-93. [Medline].
Collaborative Ocular Melanoma Study Group. Ten-year follow-up of fellow eyes of patients enrolled in Collaborative Ocular Melanoma Study randomized trials: COMS report no. 22. Ophthalmology. May 2004;111(5):966-76. [Medline].
Collaborative Ocular Melanoma Study Group report no 4. Mortality in patients with small choroidal melanoma. The Collaborative Ocular Melanoma Study Group. Arch Ophthalmol. Jul 1997;115(7):886-93. [Medline].
Collaborative Ocular Melanoma Study report no 10. The Collaborative Ocular Melanoma Study (COMS) randomized trial of pre- enucleation radiation of large choroidal melanoma II: initial mortality findings. COMS report no. 10. Am J Ophthalmol. Jun 1998;125(6):779-96. [Medline].
Collaborative Ocular Melanoma Study report no 6. Histopathologic characteristics of uveal melanomas in eyes enucleated from the Collaborative Ocular Melanoma Study. Am J Ophthalmol. Jun 1998;125(6):745-66. [Medline].
Corrêa ZM, Augsburger JJ, Freire J, et al. Early-onset scleral necrosis after iodine I 125 plaque radiotherapy for ciliochoroidal melanoma. Arch Ophthalmol. Feb 1999;117(2):259-61. [Medline].
Daubert GL, Puklin. Intraoperative use of ultrasound to document proper plaque placement in treating choroidal melanoma. Ultrasonography in Ophthalmology. 1990;12:351-5.
Daubert GL, Puklin JE. Echographic patterns simulating extrascleral extension of malignant melanoma following plaque removal. Ultrasonography in Ophthalmology. 1990;12:345-9.
De Potter P, Shields CL, Shields JA, et al. Plaque radiotherapy for juxtapapillary choroidal melanoma. Visual acuity and survival outcome. Arch Ophthalmol. Nov 1996;114(11):1357-65. [Medline].
Dithmar S, Diaz CE, Grossniklaus HE. Intraocular melanoma spread to regional lymph nodes: report of two cases. Retina. 2000;20(1):76-9. [Medline].
Dithmar S, Völcker HE, Grossniklaus HE. Multifocal intraocular malignant melanoma: report of two cases and review of the literature. Ophthalmology. Jul 1999;106(7):1345-8. [Medline].
Donoso LA, Shields JA, Augsburger JJ, et al. Antigenic and cellular heterogeneity of primary uveal malignant melanomas. Arch Ophthalmol. Jan 1986;104(1):106-10. [Medline].
Egan KM, Ryan LM, Gragoudas ES. Survival implications of enucleation after definitive radiotherapy for choroidal melanoma: an example of regression on time-dependent covariates. Arch Ophthalmol. Mar 1998;116(3):366-70. [Medline].
Finger PT. Radiation therapy for choroidal melanoma. Surv Ophthalmol. Nov-Dec 1997;42(3):215-32. [Medline].
Finger PT, Berson A, Szechter A. Palladium-103 plaque radiotherapy for choroidal melanoma: results of a 7-year study. Ophthalmology. Mar 1999;106(3):606-13. [Medline].
Finger PT, Lipka AC, Lipkowitz JL, et al. Failure of transpupillary thermotherapy (TTT) for choroidal melanoma: two cases with histopathological correlation. Br J Ophthalmol. Sep 2000;84(9):1075-6. [Medline].
Finger PT, Romero JM, Rosen RB, et al. Three-dimensional ultrasonography of choroidal melanoma: localization of radioactive eye plaques. Arch Ophthalmol. Mar 1998;116(3):305-12. [Medline].
Folberg R, Mehaffey M, Gardner LM, et al. The microcirculation of choroidal and ciliary body melanomas. Eye. 1997;11 (Pt 2):227-38. [Medline].
Godfrey DG, Waldron RG, Capone A Jr. Transpupillary thermotherapy for small choroidal melanoma. Am J Ophthalmol. Jul 1999;128(1):88-93. [Medline].
Gragoudas ES, Lane AM, Regan S, et al. A randomized controlled trial of varying radiation doses in the treatment of choroidal melanoma. Arch Ophthalmol. Jun 2000;118(6):773-8. [Medline].
Grossniklaus HE, Albert DM, Green WR, et al. Clear cell differentiation in choroidal melanoma. COMS report no. 8. Collaborative Ocular Melanoma Study Group. Arch Ophthalmol. Jul 1997;115(7):894-8. [Medline].
Grossniklaus HE, Wilson MW, Barron BC, et al. Anterior vs posterior intraocular melanoma. Metastatic differences in a murine model. Arch Ophthalmol. Sep 1996;114(9):1116-20. [Medline].
Gunduz K, Shields CL, Shields JA, et al. Plaque radiotherapy for management of ciliary body and choroidal melanoma with extraocular extension. Am J Ophthalmol. Jul 2000;130(1):97-102. [Medline].
Gunduz K, Shields CL, Shields JA, et al. Plaque radiotherapy of uveal melanoma with predominant ciliary body involvement. Arch Ophthalmol. Feb 1999;117(2):170-7. [Medline].
Gunduz K, Shields CL, Shields JA, et al. Radiation complications and tumor control after plaque radiotherapy of choroidal melanoma with macular involvement. Am J Ophthalmol. May 1999;127(5):579-89. [Medline].
Gupta M, Rennie IG. Orbital metastasis from a choroidal melanoma. Eye. Feb 2005;19(2):227-9. [Medline].
Hawkins BS. The Collaborative Ocular Melanoma Study (COMS) randomized trial of pre-enucleation radiation of large choroidal melanoma: IV. Ten-year mortality findings and prognostic factors. COMS report number 24. Am J Ophthalmol. Dec 2004;138(6):936-51. [Medline].
Jakobiec FA, Coleman DJ, Chattock A, et al. Ultrasonically guided needle biopsy and cytologic diagnosis of solid intraocular tumors. Ophthalmology. Sep 1979;86(9):1662-81. [Medline].
Kiratli H, Bilgiç S. Sequential development of bilateral primary choroidal melanoma. Acta Ophthalmol Scand. Aug 2000;78(4):474-6. [Medline].
Kroll S, Char DH, Quivey J, et al. A comparison of cause-specific melanoma mortality and all-cause mortality in survival analyses after radiation treatment for uveal melanoma. Ophthalmology. Nov 1998;105(11):2035-45. [Medline].
Maberly DA, Pavlin CJ, McGowan HD, et al. Ultrasound biomicroscopic imaging of the anterior aspect of peripheral choroidal melanomas. Am J Ophthalmol. Apr 1997;123(4):506-14. [Medline].
Mafee MF, Puklin J, Barany M, et al. MRI and in vivo proton spectroscopy of the lesions of the globe. Semin Ultrasound CT MR. Dec 1988;9(6):428-42. [Medline].
Makitie T, Summanen P, Tarkkanen A, et al. Microvascular density in predicting survival of patients with choroidal and ciliary body melanoma. Invest Ophthalmol Vis Sci. Oct 1999;40(11):2471-80. [Medline].
Makitie T, Summanen P, Tarkkanen A, et al. Microvascular density in predicting survival of patients with choroidal and ciliary body melanoma. Invest Ophthalmol Vis Sci. Oct 1999;40(11):2471-80. [Medline].
Marcus DM, Minkovitz JB, Wardwell SD, et al. The value of nucleolar organizer regions in uveal melanoma. The Collaborative Ocular Melanoma Study Group. Am J Ophthalmol. Nov 15 1990;110(5):527-34. [Medline].
McLean IW, Ainbinder DJ, Gamel JW, et al. Choroidal-ciliary body melanoma. A multivariate survival analysis of tumor location. Ophthalmology. Jul 1995;102(7):1060-4. [Medline].
McLean IW, Zimmerman LE, Evans RM. Reappraisal of Callender's spindle a type of malignant melanoma of choroid and ciliary body. Am J Ophthalmol. Oct 1978;86(4):557-64. [Medline].
McLean MJ, Foster WD, Zimmerman LE. Prognostic factors in small malignant melanomas of choroid and ciliary body. Arch Ophthalmol. Jan 1977;95(1):48-58. [Medline].
Mehaffey MG, Gardner LM, Folberg R. Distribution of prognostically important vascular patterns across multiple levels in ciliary body and choroidal melanomas. Am J Ophthalmol. Sep 1998;126(3):373-8. [Medline].
Meredith TA. Choroidal melanoma: diagnosis and management. Am J Ophthalmol. Jun 1998;125(6):865-7. [Medline].
Meyer A, Levy C, Blondel J, et al. [Optic neuropathy after proton-beam therapy for malignant choroidal melanoma]. J Fr Ophtalmol. Jun 2000;23(6):543-53. [Medline].
Midena E, de Belvis V, Dei Tos AP, et al. Isolated brain metastasis of malignant choroidal melanoma 27 years after enucleation. Arch Ophthalmol. Nov 1999;117(11):1553-6. [Medline].
Missotten L, Dirven W, Van der Schueren A, et al. Results of treatment of choroidal malignant melanoma with high-dose- rate strontium-90 brachytherapy. A retrospective study of 46 patients treated between 1983 and 1995. Graefes Arch Clin Exp Ophthalmol. Mar 1998;236(3):164-73. [Medline].
Mueller AJ, Folberg R, Freeman WR, et al. Evaluation of the human choroidal melanoma rabbit model for studying microcirculation patterns with confocal ICG and histology. Exp Eye Res. Jun 1999;68(6):671-8. [Medline].
Oosterhuis JA, Journee-de Korver HG, Keunen JE. Transpupillary thermotherapy: results in 50 patients with choroidal melanoma. Arch Ophthalmol. Feb 1998;116(2):157-62. [Medline].
Rummelt V, Folberg R, Rummelt C, et al. Microcirculation architecture of melanocytic nevi and malignant melanomas of the ciliary body and choroid. A comparative histopathologic and ultrastructural study. Ophthalmology. Apr 1994;101(4):718-27. [Medline].
Rummelt V, Folberg R, Woolson RF, et al. Relation between the microcirculation architecture and the aggressive behavior of ciliary body melanomas. Ophthalmology. May 1995;102(5):844-51. [Medline].
Rummelt V, Mehaffey MG, Campbell RJ, et al. Microcirculation architecture of metastases from primary ciliary body and choroidal melanomas. Am J Ophthalmol. Aug 1998;126(2):303-5. [Medline].
Sabetti L, Toscano A, Specchia G, et al. Neoplasia-like echographic evidence in retinopathies of several aetiologies: a case report. Ophthalmologica. 1998;212 Suppl 1:93-4. [Medline].
Scott IU, Murray TG, Hughes JR. Evaluation of imaging techniques for detection of extraocular extension of choroidal melanoma. Arch Ophthalmol. Jul 1998;116(7):897-9. [Medline].
Scotto J, Fraumeni JF Jr, Lee JA. Melanomas of the eye and other noncutaneous sites: epidemiologic aspects. J Natl Cancer Inst. Mar 1976;56(3):489-91. [Medline].
Shields CL, Ganguly A, Materin MA, et al. Chromosome 3 analysis of uveal melanoma using fine-needle aspiration biopsy at the time of plaque radiotherapy in 140 consecutive cases: the Deborah Iverson, MD, Lectureship. Arch Ophthalmol. Aug 2007;125(8):1017-24. [Medline].
Shields CL, Shields JA. Transpupillary thermotherapy for choroidal melanoma. Curr Opin Ophthalmol. Jun 1999;10(3):197-203. [Medline].
Shields CL, Shields JA, Cater J, Augsburger JJ. Transpupillary thermotherapy for choroidal melanoma: tumor control and visual results in 100 consecutive cases. Ophthalmology. Apr 1998;105(4):581-90. [Medline].
Shields CL, Shields JA, De Potter P, et al. Diffuse choroidal melanoma. Clinical features predictive of metastasis. Arch Ophthalmol. Aug 1996;114(8):956-63. [Medline].
Shields CL, Shields JA, Shields MB, et al. Prevalence and mechanisms of secondary intraocular pressure elevation in eyes with intraocular tumors. Ophthalmology. Jul 1987;94(7):839-46. [Medline].
Shields JA, Augsburger JJ. Cataract surgery and intraocular lenses in patients with unsuspected malignant melanoma of the ciliary body and choroid. Ophthalmology. Jun 1985;92(6):823-6. [Medline].
Shields JA, Shields CL. Current management of posterior uveal melanoma. Mayo Clin Proc. Dec 1993;68(12):1196-200. [Medline].
Shields JA, Shields CL. Massive orbital extension of posterior uveal melanomas. Ophthal Plast Reconstr Surg. 1991;7(4):238-51. [Medline].
Shields JA, Shields CL, De Potter P, et al. Diagnosis and treatment of uveal melanoma. Semin Oncol. Dec 1996;23(6):763-7. [Medline].
Shields JA, Shields CL, Gunduz K, et al. Adenoma of the ciliary body pigment epithelium: the 1998 Albert Ruedemann, Sr, memorial lecture, Part 1. Arch Ophthalmol. May 1999;117(5):592-7. [Medline].
The Collaborative Ocular Melanoma Study (COMS) randomized trial of pre-enucleation radiation of large choroidal melanoma I: characteristics of patients enrolled and not enrolled. COMS report no. 9. Am J Ophthalmol. Jun 1998;125(6):767-78. [Medline].
Umlas J, Diener-West M, Robinson NL, et al. Comparison of transillumination and histologic slide measurements of choroidal melanoma. Arch Ophthalmol. Apr 1997;115(4):474-7. [Medline].
Yanoff M. Mechanisms of glaucoma in eyes with uveal malignant melanomas. Int Ophthalmol Clin. Spring 1972;12(1):51-62. [Medline].
Further Reading
Keywords
choroidal melanoma, malignant choroidal melanoma, intraocular tumor, intraocular melanoma, malignant tumor, malignant melanoma, uveal melanoma, primary malignant melanoma, anterior choroidal melanoma, posterior choroidal melanoma, anterior uveal melanoma, posterior uveal melanoma, uvea, iris, choroid, ciliary body, uveal structure
Differential Diagnoses & Workup: Melanoma, Choroidal