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Classification and Stage Information for Intraocular (Uveal) Melanoma

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    Systemic metastases are generally hematogenous in origin, and the first site identified is usually the liver.[9] Lung, bone, and subcutaneous sites are also common.[9] In the COMS trials, the liver was the only site of detectable metastasis in 46% of patients with metastases reported during follow-up or at the time of death; 43% had metastases diagnosed in the liver and other sites.[9] In patients with a history of ocular melanoma who present with hepatic metastases of unknown origin, metastatic melanoma should be considered in the differential diagnosis.

    It is particularly unusual for choroidal melanomas of any size to invade the optic nerve or its meninges.[10] Metastasis of choroidal melanoma to the contralateral choroid is also rare.[9,11]

    Staging

    Definitions of TNM

    An American Joint Committee on Cancer staging system has been developed for melanoma of the uveal tract.[7]

    Table 1. Primary Tumor (T)a,b

    All Uveal Melanomas
    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.
    b These definitions apply to both clinical and pathologic staging.[12] Note: In clinical practice, the largest tumor basal diameter may be estimated in optic disc diameters (dd, average: 1 dd = 1.5 mm). Tumor thickness may be estimated in diopters (average: 2.5 diopters = 1 mm). However, techniques such as ultrasonography and fundus photography are used to provide more accurate measurements. Ciliary body involvement can be evaluated by the slit-lamp, ophthalmoscopy, gonioscopy, and transillumination. However, high-frequency ultrasonography (ultrasound biomicroscopy) is used for more accurate assessment. Extension through the sclera is evaluated visually before and during surgery, and with ultrasonography, computed tomography, or magnetic resonance imaging.Note: When histopathologic measurements are recorded after fixation, tumor diameter and thickness may be underestimated because of tissue shrinkage.
    c Iris melanomas originate from, and are predominantly located in, this region of the uvea. If less than half of the tumor volume is located within the iris, the tumor may have originated in the ciliary body and consideration should be given to classifying it accordingly.
    d Primary ciliary body and choroidal melanomas are classified according to four tumor-size categories.
    TXPrimary tumor cannot be assessed.
    T0No evidence of primary tumor.
    Irisc
    T1Tumor limited to the iris.
    T1aTumor limited to the iris not more than 3 clock hours in size.
    T1bTumor limited to the iris more than 3 clock hours in size.
    T1cTumor limited to the iris with secondary glaucoma.
    T2Tumor confluent with or extending into the ciliary body, choroid, or both.
    T2aTumor confluent with or extending into the ciliary body, choroid, or both, with secondary glaucoma.
    T3Tumor confluent with or extending into the ciliary body, choroid, or both, with scleral extension.
    T3aTumor confluent with or extending into the ciliary body, choroid, or both, with scleral extension and secondary glaucoma.
    T4Tumor with extrascleral extension.
    T4aTumor with extrascleral extension ≤5 mm in diameter.
    T4bTumor with extrascleral extension >5 mm in diameter.
    Ciliary Body and Choroidd
    T1Tumor size category 1.
    T1aTumor size category 1 without ciliary body involvement and extraocular extension.
    T1bTumor size category 1 with ciliary body involvement.
    T1cTumor size category 1 without ciliary body involvement but with extraocular extension ≤5 mm in diameter.
    T1dTumor size category 1 with ciliary body involvement and extraocular extension ≤5 mm in diameter.
    T2Tumor size category 2.
    T2aTumor size category 2 without ciliary body involvement and extraocular extension.
    T2bTumor size category 2 with ciliary body involvement.
    T2cTumor size category 2 without ciliary body involvement but with extraocular extension ≤5 mm in diameter.
    T2dTumor size category 2 with ciliary body involvement and extraocular extension ≤5 mm in diameter.
    T3Tumor size category 3.
    T3aTumor size category 3 without ciliary body involvement and extraocular extension.
    T3bTumor size category 3 with ciliary body involvement.
    T3cTumor size category 3 without ciliary body involvement but with extraocular extension ≤5 mm in diameter.
    T3dTumor size category 3 with ciliary body involvement and extraocular extension ≤5 mm in diameter.
    T4Tumor size category 4.
    T4aTumor size category 4 without ciliary body involvement and extraocular extension.
    T4bTumor size category 4 with ciliary body involvement.
    T4cTumor size category 4 without ciliary body involvement but with extraocular extension ≤5 mm in diameter.
    T4dTumor size category 4 with ciliary body involvement and extraocular extension ≤5 mm in diameter.
    T4eAny tumor size category with extraocular extension >5 mm in diameter.
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