Malignant eye tumors

Nathalie Cassoux
07/24/2017
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Malignant eye tumors may involve several structures of the eye: the eyelids, the conjunctiva, the eye socket and the eye itself. Most of these tumors are rare but serious, and require early treatment at a specialized center.
Nathalie Cassoux
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Conjunctival, palpebral and orbital tumors

Eye tumors require a multidisciplinary approach to optimize the chances of recovery.

  • Tumors of the eyelids include basal cell carcinomas, which are by far the most frequent, and which, in most cases, have an excellent prognosis. These tumors are malignant locally, but do not produce metastases. Initial surgery is the standard treatment. If performed early on, the patient can recover with minimal surgical after-effects. In more advanced forms, broader surgery with immediate reconstruction of the eyelid is recommended. The histological results are discussed in a multidisciplinary consultation meeting. Forms that are very advanced locally can also benefit from radiotherapy or in some cases targeted therapy.
  • Other malignant tumors of the eyelid are rarer and more aggressive, and can produce metastases, such as epidermoid carcinoma, sebaceous carcinoma or Merkel cell carcinoma. These tumors are usually treated with a combination of surgery and radiotherapy. Here again, they benefit from a multidisciplinary approach and recent biological advances.
  • Malignant tumors of the conjunctiva include lymphoma, severe dysplasia and conjunctival carcinoma, pre-cancerous melanosis and conjunctival melanoma. These lesions are rare but potentially serious, and require early, appropriate treatment. Conjunctival carcinomas are epithelial tumors whose prognosis depends on the initial size, prior surgeries, the number of local relapses and the histological aggressiveness. When the tumor is treated using a combination of surgery and disc or proton radiotherapy, it can be very well controlled locally with few after-effects for the eye, and lymph node relapse and metastases can be prevented. These tumors may also benefit from chemotherapy or immunotherapy (Interferon alfa-2b), with collyrium in addition.
  • Conjunctival melanomas are aggressive malignant tumors. They are similar to skin melanomas, but a lot rarer. These tumors occur most frequently subsequent to a pre-cancerous state known as Reese melanosis or after degeneration of a former nevus or even de novo. The tumor is generally pigmented and grows rapidly on a subject with fair skin at the age of around 50. Treatment requires a specialized center and involves surgical removal followed by additional radiotherapy, the terms of which will be discussed in a multidisciplinary consultation meeting. The search for mutations (BRAD, RAS, KIT) means that, if there are metastases, targeted therapy or appropriate immunotherapy is administered. Research in conjunctival melanoma is being conducted on mutations found in tumors.
  • Conjunctival lymphoma usually has a good prognosis when associated with the mucous membranes (MALT lymphoma). There are rarer, more aggressive forms. The diagnosis is performed by simple biopsy, and treatment is then discussed in a multidisciplinary consultation meeting with hematologists and radiotherapists.
  • Tumors of the eye socket are rare and a lot more polymorphous. All types of tumor may be found in the eye socket. The diagnosis is often based on a biopsy or removal analyzed by a seasoned anatomopathologist.
  • Malignant eye tumors, strictly speaking, include primitive tumors (retinoblastoma in children, choroidal melanoma, primitive lymphoma of the eye socket in adults) and choroidal metastases from a cancer located elsewhere, such as the breast, lung or colon. The diagnosis is established by an ophthalmologist specializing in eye tumors based on clinical and echographical evidence. Biopsy of the eye is always a delicate endeavor, but is proposed in certain cases when the diagnosis is difficult.

Treatment of eye tumors at Institut Curie

All of these tumors, with the exception of basal cell carcinoma, are rare tumors that require thorough treatment to prevent local relapse, which could lead to disfiguring surgery or metastases. Treatment at Institut Curie involves surgery performed by surgeons specializing in these tumors, one-stage reconstructions with the assistance of a plastic surgeon, a histological analysis performed by trained pathologists, and additional treatments as discussed in a multidisciplinary context.

Institut Curie has all therapeutic methods available, including the most innovative. Treatment can be adapted to suit each patient and each type of tumor (tailored treatment). Research involving study of the biology of these tumors helps provide innovative treatments for the most serious forms.

Research into eye tumors

Researchers are currently working on an in-depth study of the biology of conjunctival melanoma in collaboration with a team from Manchester. This helps to better understand the profile of the mutations of this tumor, which, even if similar to skin melanoma, presents differences that may explain why immunotherapy used in skin melanoma does not respond as well.

Physicians specializing in eye tumors

  • Specialized ophthalmologists

Nathalie Cassoux

Christine Levy

Livia Lumbroso

Laurence Desjardins

  • Oncological radiotherapists

Rémi Dendale

Valentin Calugaru

  • Medical oncologists

Manuel Rodrigues

Sophie Piperno-Neumann

  • Referring pathologists

Sophie Gardrat

Anne Salomon

  • Plastic surgeons

Benoît Couturaud