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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.4, Smitt, MC, Donaldson SS. Radiotherapy is successful treatment for orbital lymphoma. International Journal of Radiation Oncology*Biology*Physics. 1993;26(1):59-66.5 Bolek TW, Moyses HM, Marcus Jr RB, Gorden III L, Maiese RL, Almasri NM, Mendenhall NP. Radiotherapy in the management of orbital lymphoma. International Journal of Radiation Oncology*Biology*Physics. Volume 44, Issue 1, 1 April 1999;31-36. 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.6 Goldberg RA, Rootman J. Clinical characteristics of metastatic orbital tumors. Ophthalmology. 1990 May;97(5):620-4.
  • 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).7 Skinner HD, Garden AS, Rosenthal DI, Ang KK, Morrison WH, Esmaeli B, Pinnix CC, Frank SJ. Outcomes of Malignant Tumors of the Lacrimal Apparatus: The University of Texas MD Anderson Cancer Center Experience. Cancer. 2011;117(12):2801-2810.
    • Radiation: Some treatment centers might use external beam radiation as an initial treatment, but in most cases radiation will be used after surgical removal.8 Wright J, Rose G, Garner A. Primary malignant neoplasms of the lacrimal gland. Br J Ophthalmology. 1992;76:401-407. Other radiation treatment methods such as neutron beam, gamma knife radiosurgery and brachytherapy have also been described.9, Kim M, Park K, Kim JH, Kim Y, Lee J. Gamma knife radiosurgery for orbital tumors. Clin Neurol Neurosurg. 2008;110:1003-1007.10, Douglas JG, Laramore GE, Austin-Seymour M, et al. Treatment of locally advanced adenoid cystic carcinoma of the head and neck with neutron radiotherapy. Int J Radiat Oncol Biol Phys. 2000;46:551-557.11 Finger PT. Radiation Therapy for Orbital Tumors: Concepts, Current Use, and Ophthalmic Radiation Side Effects, Survey of Ophthalmology. Volume 54, Issue 5, September–October 2009;545-568.
    • Chemotherapy: Because the prognosis for lacrimal cancer has room for improvement, some treatment centers might consider chemotherapy.12, Meel R, Pushker N, Bakhshi S. Adjuvant chemotherapy in lacrimal gland adenoid cystic carcinoma. Pediatr Blood Cancer. 2009;53:1163-1164.13 Meldrum ML, Tse DT, Benedetto P. Neoadjuvant intracarotid chemotherapy for treatment of advanced adenocystic carcinoma of the lacrimal gland. Arch Ophthalmol. 1998;116:315-321. If this is considered, it should typically be done in addition to radiation and surgery in select cases.
References

1 Riedel KG, Markl A, Hasenfratz G, Kampik A, Stefani FH, Lund OE. Epithelial tumors of the lacrimal gland: clinico-pathologic correlation and management. Neurosurg Rev. 1990;13(4):289-98.

2 Tijl JW, Koornneef L. Fine needle aspiration biopsy in orbital tumours. Br J Ophthalmol.1991 August;75(8):491-492.

3 Spiro RH, Thaler HT, Hicks WF, Kher UA, Huvos AH, Strong EW. The importance of clinical staging of minor salivary gland carcinoma. Am J Surg. 1991 Oct;162(4):330-6.

4 Smitt, MC, Donaldson SS. Radiotherapy is successful treatment for orbital lymphoma. International Journal of Radiation Oncology*Biology*Physics. 1993;26(1):59-66.

5 Bolek TW, Moyses HM, Marcus Jr RB, Gorden III L, Maiese RL, Almasri NM, Mendenhall NP. Radiotherapy in the management of orbital lymphoma. International Journal of Radiation Oncology*Biology*Physics. Volume 44, Issue 1, 1 April 1999;31-36.

6 Goldberg RA, Rootman J. Clinical characteristics of metastatic orbital tumors. Ophthalmology. 1990 May;97(5):620-4.

7 Skinner HD, Garden AS, Rosenthal DI, Ang KK, Morrison WH, Esmaeli B, Pinnix CC, Frank SJ. Outcomes of Malignant Tumors of the Lacrimal Apparatus: The University of Texas MD Anderson Cancer Center Experience. Cancer. 2011;117(12):2801-2810.

8 Wright J, Rose G, Garner A. Primary malignant neoplasms of the lacrimal gland. Br J Ophthalmology. 1992;76:401-407.

9 Kim M, Park K, Kim JH, Kim Y, Lee J. Gamma knife radiosurgery for orbital tumors. Clin Neurol Neurosurg. 2008;110:1003-1007.

10 Douglas JG, Laramore GE, Austin-Seymour M, et al. Treatment of locally advanced adenoid cystic carcinoma of the head and neck with neutron radiotherapy. Int J Radiat Oncol Biol Phys. 2000;46:551-557.

11 Finger PT. Radiation Therapy for Orbital Tumors: Concepts, Current Use, and Ophthalmic Radiation Side Effects, Survey of Ophthalmology. Volume 54, Issue 5, September–October 2009;545-568.

12 Meel R, Pushker N, Bakhshi S. Adjuvant chemotherapy in lacrimal gland adenoid cystic carcinoma. Pediatr Blood Cancer. 2009;53:1163-1164.

13 Meldrum ML, Tse DT, Benedetto P. Neoadjuvant intracarotid chemotherapy for treatment of advanced adenocystic carcinoma of the lacrimal gland. Arch Ophthalmol. 1998;116:315-321.

14 Oberlin O, Rey A, Anderson J, Carli M, Raney RB, Treuner J, Stevens, MC. Treatment of orbital rhabdomyosarcoma: survival and late effects of treatment—results of an international workshop. Journal of clinical oncology. 2001;19(1),197-204.