Orbit tumors
Orbit tumors, also known as eye socket tumors, encompass a range of growths found in the orbit, the cavity that houses the eyeball and surrounding structures. These tumors can be classified as primary, originating from the orbit itself, or metastatic, developing from tissue adjacent to or distant from the orbit. Common types of orbit tumors include rhabdomyosarcomas in children, which represent a significant portion of pediatric orbital tumors, and lymphoid tumors, cavernous hemangiomas, and various other benign and malignant lesions in adults. Symptoms often include proptosis (forward displacement of the eye), eye pain, visual disturbances, and orbital swelling.
Diagnosis typically involves a detailed patient history, physical examination, and imaging studies such as MRI or CT scans, with definitive identification achieved through tissue biopsy. Treatment varies by tumor type, with surgical removal being a preferred method for many lesions, while others may necessitate radiotherapy or chemotherapy. The prognosis largely depends on the tumor's nature, with most primary tumors being benign and having favorable outcomes, whereas metastatic tumors often indicate a poorer prognosis. Understanding the nature of orbit tumors is essential for effective management and treatment strategies.
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Orbit tumors
ALSO KNOWN AS: Eye socket tumors, rhabdomyosarcomas, dermoid cysts, capillary hemangiomas, lymphoid tumors, cavernous hemangiomas, neurofibromas, schwannomas, optic gliomas, skin cancers of the eyelids, osteomas
RELATED CONDITIONS: Arteriovenous malformations, gene mutations, trauma, systemic diseases, congenital anatomic defects, chronic inflammation or infection, metastasis from adjacent or distant primary tumors
DEFINITION: Orbit tumors are tumors found in the orbit, also known as the eye socket, which encases the eyeball, optic nerve, extraocular muscles, blood vessels, and soft tissue. Orbit tumors can be primary, in which the tumor originates from the orbit, or metastatic, in which the tumor develops from adjacent or distant tissue and invades the orbit. Most orbit tumors are but, because of their space-occupying nature, are readily conspicuous.
Risk factors: The presence of an invasive tumor in adjacent tissue may increase the risk of developing an orbit tumor. There are no other clearly identifiable risk factors for developing orbit tumors.
Etiology and the disease process: The etiology of orbit tumors varies and encompasses the etiologies of arteriovenous malformations, gene mutations, trauma, systemic diseases (such as amyloidosis or lymphoma), congenital anatomic defects, chronic inflammation or infection, and metastasis from adjacent or distant primary tumors.
Incidence: Orbit tumors can develop in children and adults, with different incidence rates depending on tumor type. In children, rhabdomyosarcoma is the most common orbit tumor. It accounts for 40 percent of all pediatric orbital tumors in the United States. Capillary hemangioma, which occurs in 2 percent of infants, is one of the most common benign pediatric tumors. The most common orbit tumors in adults are lymphoid tumors, cavernous hemangioma, and metastatic tumors. Other orbit tumors include dermoid cysts, neurofibromas, schwannomas, optic gliomas, skin cancer of the eyelid, and osteomas.
Symptoms: The most common symptom is proptosis (forward displacement of the eye). Eye pain, visual abnormalities such as double vision or even visual loss, orbital edema, and eye redness are other symptoms of orbit tumors.
Screening and diagnosis: The diagnosis of orbit tumors relies on a meticulous patient history, thorough physical exam, and (MRI) or computed (CT) scans. The diagnosis is confirmed by performing a tissue biopsy, either by fine-needle (FNAB) or by of the orbit (orbitotomy). Histological analysis will determine the type of orbit tumor and lay the groundwork for a treatment plan.
Treatment and therapy: Treatment depends on the tumor type. Surgical removal is usually the best option, especially with disfiguring, massive lesions. Some tumors require external beam radiotherapy or adjuvant chemotherapy. In pediatric patients, dermoid tumors are best treated by surgical excision, while capillary hemangiomas spontaneously regress, therefore not requiring any intervention. In adults, radiotherapy is the treatment of choice for lymphoid tumors, while surgery effectively treats cavernous hemangioma.
While surgery remained the best option, advances in other treatments have shown promise in treating orbit tumors. Intensity-modulated radiation therapy, or CyberKnife radiosurgery, and proton therapy allowed radiation treatments to more accurately target cancerous cells, leaving valuable, more healthy eye tissue. Enhanced immunotherapy was also increasingly used.
Prognosis, prevention, and outcomes: The prognosis and outcomes depend on the type of tumor. Most primary orbit tumors are benign and, therefore, have a good prognosis. Metastatic orbit tumors (with a primary source of cancer elsewhere in the body) usually signify a poor prognosis.
Bibliography
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Hassan, Waleed M., et al. "Orbital Tumors in USA: Difference in Survival Patterns." Cancer Epidemiology, vol. 38.5, 2014, pp. 515–22.
Ioakeim-Ioannidou, Myrsini, and Shannon M. MacDonald. "Evolution of Care of Orbital Tumors with Radiation Therapy." Journal of Neurological Surgery. Part B, Skull Base, vol. 81, no. 4, 2020, pp. 480-496, doi.org/10.1055/s-0040-1713894. Accessed 15 June 2024.
Laplant, Jacquelyn, and Kimberly Cockerham. "Primary Malignant Orbital Tumors." Journal of Neurological Surgery. Part B, Skull Base, vol. 82, no. 1, 2021, pp. 81-90, doi.org/10.1055/s-0040-1722635. Accessed 15 June 2024.
Pfortner, R., et al. "Orbital Tumors: Operative and Therapeutic Strategies." Facial Plastic Surgery, vol. 30.5, 2014, pp. 570–77.
Reyes-Soto, Gervith, et al. "Surgical Treatment of Orbital Tumors in a Single Center: Analysis and Results." Surgical Neurology International, vol. 15, 2024, doi.org/10.25259/SNI‗1016‗2023. Accessed 15 June 2024.