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Intraocular (Uveal) Melanoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Classification and Stage Information for Intraocular (Uveal) Melanoma

Table 2. Regional Lymph Nodes (N)a

a Reprinted with permission from AJCC: Malignant melanoma of the uvea. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 547-59.
NXRegional lymph nodes cannot be assessed.
N0No regional lymph node metastasis.
N1Regional lymph node metastasis.

Table 3. Distant Metastasis (M)a

a Reprinted with permission from AJCC: Malignant melanoma of the uvea. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 547-59.
M0No distant metastasis.
M1Distant metastasis.
M1aLargest diameter of the largest metastasis ≤3 cm.
M1bLargest diameter of the largest metastasis 3.1–8.0 cm.
M1cLargest diameter of the largest metastasis ≥8 cm.

Table 4. Anatomic Stage/Prognostic Groupsa

StageTNM
a Reprinted with permission from AJCC: Malignant melanoma of the uvea. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 547-59.
IT1aN0M0
IIAT1b–dN0M0
T2aN0M0
IIBT2bN0M0
T3aN0M0
IIIAT2c–dN0M0
T3b–cN0M0
T4aN0M0
IIIBT3dN0M0
T4b–cN0M0
IIICT4d–eN0M0
IVAny TN1M0
Any TAny NM1a–c

There are a variety of other factors that are not part of the AJCC staging system but that may help in refining estimates of prognosis.[7]

Prognostic features

There are a number of key prognostic features that are important to collect in malignant melanoma of the uvea, even though they are not included in staging algorithms. These include the following:[7]

Molecular features

  1. Chromosomal alterations.
    1. Chromosome 3 status (loss or no loss; complete or partial).
    2. Chromosome 6p status (gain or no gain).
    3. Chromosome 8q status (gain or no gain).

      Indicate:

      • Technique used for assessing chromosome status may include the following:
        • Karyotyping.
        • Fluorescence in situ hybridization.
        • Comparative genomic hybridization.
        • Loss of heterozygosity using DNA polymorphism analysis (e.g., single nucleotide polymorphism, microsatellite).
        • Other.
      • How specimen was obtained may include the following:
        • Enucleation.
        • Local resection.
        • Biopsy.
        • Fine-needle aspiration biopsy.
      • For needle biopsies, whether cytopathologic evaluation was performed to confirm the presence of tumor cells.
  2. Gene-expression profile: class 1 or class 2.

    Indicate:

    • Technique used for gene-expression profiling (e.g., microarray, pathologic complete response).
    • How specimen was obtained (e.g., enucleation, local resection, biopsy, fine-needle aspiration biopsy).
    • For needle biopsies, whether cytopathologic evaluation was performed to confirm the presence of tumor cells.

Clinical and histopathologic features

  1. Clinical.
    1. Positron-emission tomography/computed tomography.
      • 18-Fluorine-labelled 2-deoxy-2-fluoro-D-glucose standardized uptake values (higher values in primary tumor may be associated with shorter survival).
    2. Confocal indocyanine green angiography.
      • Identification of complex monocirculatory patterns (i.e., loops, networks, arcs with branching, parallel with cross-linking or a combination of these patterns may be associated with shorter survival).
  2. Histopathologic.
    1. Mitotic count.
      • Number of mitotic figures per 40 high-power fields (typical field area 0.15–0.19 mm2, higher counts are associated with shorter survival).
    2. Mean diameter of the ten largest nucleoli.
      • Mean of the longest nucleoli (MLN) is measured along a central 5-mm long strip, e.g., after silver staining (larger values are associated with shorter survival).
    3. Presence of extravascular matrix patterns.
      • Loops.
        • Absent.
        • Present (shorter survival).
      • Loops forming networks.
        • Absent.
        • Present (shorter survival).
      • Other complex patterns (arcs with branching, parallel with cross-linking; absent or present).

        The patterns are assessed with light microscopy under a dark green filter after staining with periodic-acid Schiff without counterstain.

    4. Microvascular density.
      • Number of immunopositive elements labeled with markers for vascular endothelial cells (e.g., CD34 epitope, factor VIII-related antigen) in areas of densest vascularization (typical field area 0.31 mm2, higher counts are associated with shorter survival).
    5. Insulin-like growth factor 1 receptor (IGF1-R).
      • Percentage of immunopositive tumor cells (high expression is associated with shorter survival).
    6. Tumor-infiltrating lymphocytes.
      • Few (longest survival).
      • Moderate numbers.
      • Many (shortest survival).
    7. Tumor-infiltrating macrophages.
      • Few (longest survival).
      • Moderate numbers.
      • Many (shortest survival).

        The number can be compared with standard photographs.[13]

    8. HLA Class I expression.
      • Percentage of immunopositive tumor cells (low expression is associated with longer survival).

References:

  1. Factors predictive of growth and treatment of small choroidal melanoma: COMS Report No. 5. The Collaborative Ocular Melanoma Study Group. Arch Ophthalmol 115 (12): 1537-44, 1997.
  2. Diener-West M, Earle JD, Fine SL, et al.: The COMS randomized trial of iodine 125 brachytherapy for choroidal melanoma, II: characteristics of patients enrolled and not enrolled. COMS Report No. 17. Arch Ophthalmol 119 (7): 951-65, 2001.
  3. Shields CL, Shields JA, De Potter P, et al.: Diffuse choroidal melanoma. Clinical features predictive of metastasis. Arch Ophthalmol 114 (8): 956-63, 1996.
  4. Scott IU, Murray TG, Hughes JR: Evaluation of imaging techniques for detection of extraocular extension of choroidal melanoma. Arch Ophthalmol 116 (7): 897-9, 1998.
  5. Romero JM, Finger PT, Iezzi R, et al.: Three-dimensional ultrasonography of choroidal melanoma: extrascleral extension. Am J Ophthalmol 126 (6): 842-4, 1998.
  6. Echography (ultrasound) procedures for the Collaborative Ocular Melanoma Study (COMS), Report no. 12, Part I. J Ophthalmic Nurs Technol 18 (4): 143-9, 1999 Jul-Aug.
  7. Malignant melanoma of the uvea. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 547-59.
  8. Dithmar S, Diaz CE, Grossniklaus HE: Intraocular melanoma spread to regional lymph nodes: report of two cases. Retina 20 (1): 76-9, 2000.
  9. Diener-West M, Reynolds SM, Agugliaro DJ, et al.: Development of metastatic disease after enrollment in the COMS trials for treatment of choroidal melanoma: Collaborative Ocular Melanoma Study Group Report No. 26. Arch Ophthalmol 123 (12): 1639-43, 2005.
  10. Shields CL, Santos MC, Shields JA, et al.: Extraocular extension of unrecognized choroidal melanoma simulating a primary optic nerve tumor: report of two cases. Ophthalmology 106 (7): 1349-52, 1999.
  11. Singh AD, Shields JA, Shields CL, et al.: Choroidal melanoma metastatic to the contralateral choroid. Am J Ophthalmol 132 (6): 941-3, 2001.
  12. Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010.
  13. Mäkitie T, Summanen P, Tarkkanen A, et al.: Tumor-infiltrating macrophages (CD68(+) cells) and prognosis in malignant uveal melanoma. Invest Ophthalmol Vis Sci 42 (7): 1414-21, 2001.
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WebMD Public Information from the National Cancer Institute

Last Updated: February 25, 2014
This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.
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