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Eye Cancer: Cancers of the Orbit

Orbital lymphoma: This is the most common type of cancer of the orbit in adults, and it is usually a form of B-cell non-Hodgkin’s lymphoma. It may show up as a nodule in the eyelid or around the eye, or it may cause the eye to be pushed out. This type of eye cancer usually does not cause pain.  

The first step in diagnosis of orbital lymphoma may be a CT scan of the orbit followed by a surgical biopsy. Making the correct diagnosis of the biopsy is very important.  Our expert pathologists use the most-advanced methods to classify orbital lymphoma.

Types of B-cell lymphoma that occur in the orbit include MALT, follicular lymphoma, large B-cell lymphoma and mantle cell lymphoma. Less- common types include natural killer T-cell lymphoma, mycosis fungoidis and other rare types of lymphoma such as Hodgkin’s lymphoma.

Treatment for orbital lymphoma may include radiation therapy, monoclonal antibody therapy, chemotherapy or a combination of these, depending on type of lymphoma the stage of the tumor. Our doctors use the latest techniques to stage this type of eye cancer including positron emission tomography (PET) scans, computed axial tomography (CT or CAT) scans, magnetic resonance imaging (MRI) scans and bone marrow biopsy.

Orbital sarcoma:
Sarcomas arise from muscles or fatty tissue. The most common sarcoma in the orbit is rhabdomyosarcoma, which is found most frequently in children.

Our orbital surgeons work closely with our sarcoma experts to diagnose and treat orbital sarcomas. Every effort is made to save the eye and its function while treating this type of eye cancer.

The majority of rhabdomyosarcoma patients survive with preservation of the eye and orbit. A combination of chemotherapy and proton therapy can be used in children with the most common type of orbital rhabdomyosarcoma. This combination treatment can prevent the need for removal of the eye and orbital content in the majority of patients.

Orbital and optic nerve meningiomas: These benign tumors can be associated with the optic nerve or arise from meninges in the base of the skull and extend into the orbit. The symptoms may be gradual loss of vision or visual field (side vision).

An imaging study such as an MRI of the orbit can help with diagnosis. Surgical biopsies usually are not necessary and are avoided if possible to minimize risk of visual loss. Treatment of optic nerve meningioma usually includes radiation therapy, particularly proton therapy.  

Metastatic orbital tumors: Almost all types of cancer can metastasize (spread) to the orbit (space behind and around the eye). The most common are breast cancer, prostate, melanoma, lung and carcinoid tumors. Metastatic tumors of the orbit are treated with chemotherapy and/or radiation.

If you have been diagnosed with eye cancer, we’re here to help. Call 1-877-632-6789 to make an appointment or request an appointment online.

Why Choose MD Anderson?

  • Latest eye cancer treatments, including specialized surgical techniques to remove cancer but save the eye, proton therapy, targeted therapies, advanced reconstructive surgery for eyelid and orbital cancer
  • Advanced diagnostic techniques including sentinel lymph node biopsy, ultrasound biomicroscopy, confocal biomicroscopy and optical coherence tomography (OCT)
  • Skilled, highly specialized eye surgeons and reconstructive surgeons who have dedicated many years of their career to exclusively treat cancer
  • We treat more eye cancers than most centers
  • Team approach to treating eye cancers
  • Clinical trials of leading-edge treatments for eye cancer

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Orbital Lymphoma Staging

(source: American Joint Committee on Cancer)

If you are diagnosed with eye cancer, your doctor will determine the stage of the disease. Staging is a way of classifying how much disease is in the body and where it has spread when it is diagnosed. This information helps your doctor plan the best type of treatment for you. Once the staging classification is determined, it stays the same even if treatment is successful or the cancer spreads.

The most common staging system used for ocular cancers was developed by the American Joint Committee on Cancer (AJCC). The TNM system is based on three key pieces of information:

  • T describes the size of the primary tumor and/or whether it has invaded nearby structures
  • N describes whether the cancer has spread to nearby (regional) lymph nodes
  • M indicates whether the cancer has metastasized (spread) to other parts of the body (The most common site of eye melanoma spread is the liver)

Numbers or letters appear after T, N and M to provide more details about each of these factors:

  • The numbers 0 through 4 indicate increasing severity
  • The letter X means "cannot be assessed" because the information is not available.

Orbital lymphoma is staged as follows:

T categories:

  • TX: Lymphoma extent not specified
  • T0: No evidence of lymphoma
  • T1: Lymphoma involving the conjunctiva alone without orbital involvement
  • T1a: Bulbar conjunctive only
  • T1b: Palpebral conjunctiva ± fornix ± caruncle
  • T1c: Extensive conjunctival involvement
  • T2: Lymphoma with orbital involvement ± any conjunctival involvement
  • T2a: Anterior orbital involvement (± conjunctival involvement)
  • T2b: Anterior orbital involvement (± conjunctival + lacrimal involvement)
  • T2c: Posterior orbital involvement (± conjunctival involvement ± anterior involvement ± any extraocular muscle involvement)
  • T2d: Nasolacrimal drainage system involvement (± conjunctival involvement but not including nasopharynx)
  • T3: Lymphoma with pre-septal eyelid involvement (defined above) ± orbital involvement ± conjunctival involvement
  • T4: Orbital adnexal lymphoma extending beyond orbit to adjacent structures such as bone and brain
  • T4a: Involvement of nasopharynx
  • T4b: Osseous involvement (including periosteum)
  • T4c: Involvement of maxillofacial, ethmoidal and/or frontal sinuses
  • T4d: Intracranial spread

N categories:

  • NX: Regional lymph nodes cannot be assessed
  • N0: No evidence of lymph node involvement
  • N1: Involvement of ipsilateral regional lymph nodes
  • N2: Involvement of contralateral or bilateral regional lymph nodes
  • N3: Involvement peripheral lymph nodes not draining ocular adnesal region
  • N4: Involvement of central lymph nodes

M categories:

  • M0: No evidence of involvement of other extranodal sites
  • M1a: Noncontiguous involvement of tissues or organs external to the ocular adnexa
  • M1b: Lymphomatous involvement of the bone marrow
  • M1c: Both M1a and M1b involvement

Orbital Sarcoma Staging

T categories:

  • TX: Primary tumor cannot be assessed
  • T0: No evidence of primary tumor
  • T1: Tumor ≤ 15 mm in greatest dimension
  • T2: Tumor >15 mm in greatest dimension without invasion of globe or bony wall
  • T3: Tumor of any size with invasion of orbital tissues and/or bony walls
  • T4: Tumor invasion of globe or periorbital structures, such as eyelids, temporal fossa, nasal cavity, and paranasal sinuses, and/or central nervous system

N categories:

  • NX: Regional lymph nodes cannot be assessed
  • N0: No regional lymph node metastasis
  • N1: Regional lymph node metastasis

M categories:

  • M0: No distant metastasis
  • M1: Distant metastasis

© 2014 The University of Texas MD Anderson Cancer Center