Orbital Tumors

Deciding on a Treatment Plan

Before starting treatment, your doctor will complete a preoperative evaluation.

Preoperative Evaluation

  • A full history and physical examination
  • An evaluation by the members of a head and neck cancer team and an ophthalmologist
  • A biopsy to confirm a diagnosis of cancer
  • Evaluation of the lungs to check for spread
  • Imaging of the primary tumor and the neck
  • Maybe a PET-CT for advanced cancers
  • Preoperative medical clearance and optimization of medical conditions

Then your doctor will recommend a course of treatment for you, depending on a number of factors. Like for all cancers in the head and neck, there are three general therapeutic options to consider:

However, unlike most other sites of head and neck cancer, there are no National Comprehensive Cancer Network® (NCCN®) Clinical Practice Guidelines for the treatment of various orbital cancers. The reason it is difficult to get a consensus on treatment is because:

  • These cancers are very rare, and it is difficult for one study (based in one institution) to gather enough patients to compare different treatments.
  • Those studies that do collect patients over time include many different types of orbital cancers and many different treatment regimens that may change over time.
  • Many orbital tumors don’t have a clear staging system.

However, data is accumulating, and doctors are continually learning about what the best treatment options are. You should have an in-depth discussion about different treatment options with your doctor, who should ideally specialize in cancers of the head and neck or orbital region.

Some general treatment information is as follows. Keep in mind that the treatment recommendation you receive may vary depending on the type of cancer you have.

  • Orbital lymphoma: Surgery can be helpful in making the diagnosis of lymphoma, but it does not play a major role in treating it. Studies have found success using radiotherapy alone. Of course, successful treatment using just a single mode of therapy depends on the type of lymphoma and the stage. Additional treatment might be required in some cases.
  • Metastatic orbital tumors: When tumors from other parts of the body have spread to the orbit (a rare occurrence), there are currently no effective long-term treatment options. However, modern treatments are continually improving in terms of making patients comfortable. Minimizing pain and other symptoms as long as possible are the main goals in such cases.
  • Rhabdomyosarcoma: This is the most common orbital tumor in children. Most patients with rhabdomyosarcoma of the orbit are treated within trials at major cancer hospitals using a combination of chemotherapy and radiation. Surgical removal may be indicated if there is tumor remaining after the initial treatment.
  • Lacrimal apparatus cancer: This includes cancers of the lacrimal gland, lacrimal duct and lacrimal sac. These can be different types of cancer, including adenoid cystic carcinoma, adenocarcinoma or squamous cell carcinoma. Whether all of the lacrimal structures should be grouped and treated together is questionable. Existing treatment studies are based on small groups of patients with a number of different types of tumors. Most of the studies are for adenoid cystic carcinoma. You should speak to your doctor in detail about different treatment options for these types of cancers. Some basic information for you to know:
    • Surgery: This is the generally accepted first line treatment for lacrimal gland and lacrimal apparatus cancers whenever possible. The cancer should be completely removed, and this may or may not involve removal of your eye (orbital exenteration).
    • Radiation: Some treatment centers might use external beam radiation as an initial treatment, but in most cases radiation will be used after surgical removal. Other radiation treatment methods such as neutron beam, gamma knife radiosurgery and brachytherapy have also been described.
    • Chemotherapy: Because the prognosis for lacrimal cancer has room for improvement, some treatment centers might consider chemotherapy. If this is considered, it should typically be done in addition to radiation and surgery in select cases.

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