The main ocular tumors include: choroidal melanoma and metastasis in adults and retinoblastoma in children. Main conjunctive neoplasms typical of adults include squamous carcinoma and conjunctiva melanoma. Conjuctive tumors affect adults and include conjunctive carcinoma and melanoma. The commonest orbital tumors are: angioma (benign, in children and adults), rhabdomyosarcoma (malignant, in children), lymphoma and metastasis (malignant, in adults)
The clinical approach to a patient presumably affected by a tumor (neoplasm) in the eye or the orbital area requires a meticulous and careful preliminary ophthalmological assessment, including an analysis of the patient and his or her family’s clinical histories to identify elements useful for diagnosis and prognosis. A systematic examination continues with a full ophthalmological evaluation (visual acuity, biomicroscopic exam of the anterior and posterior segment, ophthalmoscopy, intraocular tension), and all the necessary collateral examinations for the case in question (field of vision exam, exophthalometry, study of the functionality of the retina and of the optical path), which are extended to all those second-level surveys considered useful in the specific case, such as: ocular ultrasound, optical consistency tomography (OCT) examination of the visual field, study of retinal function and optical pathway.
Any lesions encountered – both on the anterior and posterior segment of the eye – must always be photographically documented: this test is fundamental for objectively assessing over time the natural history or the effect of therapy on the lesions encountered. Other important investigations in ocular oncology include retinal and choroid angiography, angiography using Optical Consistency Tomography (angio-OCT), retinal autofluorescence and confocal ocular surface microscopy. Specific neuroradiological (TC and MRI) surveys may also be useful in selected cases. If the above mentioned methodologies are not directed, mini-invasive diagnostic methods such as diagnostic cytology, or invasive methods such as incisional or excisional surgical biopsies (partial or total export of the lesion) will be used.
Treatment in ocular and orbital oncology is based on teamwork (oncological team), which the ocular oncologist is in charge of coordinating. Currently, the approach to ocular tumour is as conservative as possible, meaning that it attempts to destroy the neoplastic lesion while preserving the eye and the visual function. When the conservation of the eye affected by the (malignant) neoplasm endangers the life of the patient, the eye must be enucleated. Radiotherapy is the main and most effective method of treating malignant and benign cancers of the eye. Radiotherapy is mainly applied by contact (chips loaded with radioactive isotopes), or by external sources (linear accelerator or heavy particles). Other treatments carried out are: transpupillary thermotherapy, which is indicated, in combination with chemotherapy, in the treatment of retinoblastoma, as well as cryotherapy, surgery, systemic and topical chemotherapy, photodynamic therapy, super-selective chemotherapy with incannulation of the ophthalmic artery, intraocular injection of antineoplastic drugs and immunotherapy.
Choroidal melanoma is the most frequent malignant primitive intraocular tumour in adults, with an incidence of 6 cases per million people a year.Conservative radiotherapy (brachytherapy or accelerated protons) can now successfully treat over 95% of cases. For lesions that are too large, the enucleation of the eye is still necessary. In spite of favourable results in the local treatment of neoplasia, choroidal melanoma remains a potentially fatal disease, with a risk of metastasis after five years of about 50%.
Choroidal metastases are the most frequent malignant intraocular neoplasia, although they are relatively rarely diagnosed since they generally affect patients in advanced stages of a systemic disease, and who are often not subjected to ophthalmological visits. They are generally diagnosed through ophthalmoscopy, although diagnosis should be supported by ecography and, if needed, angiography. The ophthalmoscopic aspect is that of single or, more often, multiple and/or bilateral lesions, hypopygmented and subretinal, and located mainly in the posterior segment, near the optic nerve and the macula. Choroidal metastases require multidisciplinary treatment based on systemic chemotherapy and, if needed, radiotherapy (standard or brachytherapy) or other focal treatment (photodynamic therapy).
Retinoblastoma is the most frequent malignant intraocular tumour in children. It can affect either one or both eyes, and may or may not be hereditary. Currently, retinoblastoma has a very high survival rate (>95%). The most common initial sign is leukocoria (white reflection in the pupil), which unfortunately only becomes visible rather late in the disease. Standard treatment is chemoreduction (systemic chemotherapy aiming to decrease the size of the ocular lesions) associated with focal consolidation treatment (thermotherapy, cryotherapy, brachytherapy). The enucleation of the eye remains a treatment option in cases of locally advanced neoplasia.
Choroidalhemangioma is the most frequent primitive vascular tumor of the choroid. This type of tumor generally remains undiagnosed until it becomes symptomatic. The appearance of symptoms such as lower visual acuity and/or metamorphopsia generally occurs in the third or fourth decade of life. Choroidalhaemangioma is a benign tumor, and thus the purpose of treatment is not the destruction of the tumour but the conservation of the best possible visual acuity for the longest possible time. If the patient has no symptom and visual acuity does not diminish, treatment is not necessary and the patient must be monitored over time. If a lifting of the neuroepithelium leads to decreased visual acuity, then treatment will have to be taken into consideration. The most commonly used treatment today is photodynamic therapy.
Corneal-conjunctival squamous neoplasia
Squamous corneoconjuctivalneoplasia includes clinical profiles ranging from simple epithelia dysplasia to frankly invasive squamous carcinoma. Standard treatment is a surgical excision associated with cryotherapy of the surgical bed and reconstruction of the conjunctival plane. The use of topical antimetabolites (eye drop chemotherapy) has recently been introduced and is becoming increasingly frequent in the integrated treatment of this pathology, both in association with surgery and on its own.
Conjunctival melanoma is a very rare neoplasia of the conjunctiva (less than 2% of all malignant ocular tumors). The therapeutic approach is driven by the size and location of the melanoma. In small melanomas located in favorable sites (bulbar and perilimbal conjunctiva), surgical resection is sufficient; in cases located in unfavorable locations (conjunctive eyelid, fornix, caruncle and lid margin) or in large areas, surgical excision may not be sufficient even if associated with topical chemotherapy or radiotherapy. In non-respondent cases, very extensive and infiltrating the orbit, the exenteratio orbitae, radical surgical technique in which it removes all orbital content, is still used. The use of topical antimetabolites is now increasingly used, mainly in the treatment of pre-malignant lesions (primary acquired melanosis).