Jacqueline Carrasco
BASICS
DESCRIPTION
• Metastatic orbital tumors are tumors that have spread hematogenously to the soft tissue or bones in the orbit.
• The most common primary sites are breasts in women and lungs in men.
• Other metastases may originate from prostate gland, skin melanoma, gastrointestinal, and kidney cancers.
• Carcinoid tumors of ileal or appendiceal origin usually metastasize to orbit, whereas bronchial carcinoid tumors usually involve the uveal tract.
• Cutaneous melanoma can also metastasize to the orbit.
• Metastatic tumors in children are uncommon and come from adrenal neuroblastoma, Wilms tumors, and Ewing tumor.
EPIDEMIOLOGY
Incidence
Data not available.
Prevalence
Metastatic orbital tumors comprise about 7% of all orbital tumors.
RISK FACTORS
The risk factors for primary neoplasm are also the risk factors for orbital metastasis.
Genetics
• Genetics plays a strong role in many cancers and their ability to metastasize. We have not yet characterized the genes that specifically enable cancers to metastasize to the orbit.
• There is no specific gender predilection.
GENERAL PREVENTION
Prevention and treatment of primary neoplasm helps prevent metastatic orbital tumors.
PATHOPHYSIOLOGY
• Extraocular muscles are often seeded because of their abundant blood supply.
• The bone marrow of the sphenoid bone is also commonly involved because of the high volume of low-flow venous channels in the bone.
ETIOLOGY
See above.
COMMONLY ASSOCIATED CONDITIONS
Not applicable.
DIAGNOSIS
HISTORY
• Average age of presentation is seventh decade in adult cases.
• 25–35% of patients do not have a primary malignancy at the time of ocular presentation.
• Diplopia, pain, and visual loss are most common symptoms.
• Also, ptosis (droopy eyelid), proptosis (bulging eye), eyelid swelling, and palpable mass are common features.
PHYSICAL EXAM
• Clinical presentation can be divided into 3 subcategories:
– Infiltrative is characterized by motility restriction, resistance to retropulsion, ptosis, and enophthalmos.
– Mass is characterized by proptosis and often a palpable orbital mass.
– Inflammatory is characterized by pain, chemosis, erythema, and periorbital swelling.
• Decreased vision can occur from orbital congestion, with optic neuropathy and choroidal folds.
• Enophthalmos is another important diagnostic sign. It comes from tethering and posterior traction on the globe from scirrhous metastasis such as breast or gastrointestinal carcinoma.
• Cranial neuropathies from orbital apex involvement or cavernous sinus involvement are possible.
DIAGNOSTIC TESTS & INTERPRETATION
Lab
Initial lab tests
Patients suspected of having metastatic disease by history and examination should have biopsy before extensive lab testing is performed. However, if inflammatory processes are also in the differential, ACE, p-ANCA, c-ANCA, and PPD may be helpful.
Follow-up & special considerations
Once biopsy results show suspected metastatic disease, prompt referral to a medical oncologist is warranted and further laboratory testing is indicated based upon the individual cancer.
Imaging
Initial approach
CT scan or MRI with contrast can delineate extent of orbital involvement. These images may assist in surgical planning.
Follow-up & special considerations
Medical oncologists coordinate further imaging, including PET scanning and other modalities to determine the involved sites.
Diagnostic Procedures/Other
• Histopathology is necessary to make diagnosis.
– Incisional biopsy is done when the tumor is infiltrative and/or unable to be completely removed because it is adherent to adjacent vital structures.
– Excisional biopsy is preferred whenever possible.
– Fine-needle aspiration biopsy (FNAB) may also be performed. Scirrhous breast carcinoma and gastrointestinal cancer may be dry taps.
Pathological Findings
• Histopathology will be similar to primary neoplasm. Standard hematoxylin and eosin stains can help make the diagnosis.
• Immunohistochemistry, cytopathology, and special stains may aid in distinguishing final pathologic diagnosis, particularly in poorly differentiated specimens.
DIFFERENTIAL DIAGNOSIS
• Inflammatory orbital processes such as sarcoid, TB, and idiopathic orbital inflammation
• Primary orbital tumors, such as ocular adnexal lymphoma, meningioma, or vascular lesions
• Orbital cellulitis
• Thyroid eye disease
TREATMENT
MEDICATION
Medical treatment, that is, chemotherapy and hormone therapy, varies depending upon the type of primary neoplasm and its stage. External beam radiotherapy if often used as additional therapy.
ADDITIONAL TREATMENT
General Measures
• Treatment is aimed at preserving vision and addressing pain.
• External beam radiotherapy is often first line treatment for local control of orbital tumor.
Issues for Referral
Prompt referral to a medical oncologist is necessary.
COMPLEMENTARY & ALTERNATIVE THERAPIES
Discuss with primary medical oncologist.
SURGERY/OTHER PROCEDURES
Aggressive surgical treatment may rarely be indicated including wide excision or exenteration with radiation depending upon staging and prognosis.
IN-PATIENT CONSIDERATIONS
Initial Stabilization
Typically not indicated.
Admission Criteria
Patients are typically staged as outpatients but may be admitted to the hospital for medical or social indications.
IV Fluids
As needed for associated systemic problems.
Nursing
As needed for associated systemic problems.
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
• Close follow-up is indicated to stage patient from a systemic standpoint. Ophthalmic care should be coordinated in a step-wise logical fashion with life-saving treatments.
• Palliative radiotherapy, chemotherapy, and hormone therapy are indicated depending upon the cancer.
PROGNOSIS
• Overall prognosis is poor. It is about 1 year.
• The exception is orbital carcinoid metastases, whose 5-year survival is 72%.
• Survival is short-lived in most cases of metastatic adenocarcinoma.
COMPLICATIONS
Systemic manifestations of metastatic cancer.
ADDITIONAL READING
• Char DH, Miller T, Kroll S. Orbital Metastases: Diagnosis and course. Br J Ophthalmol. 1997;81(5): 386–390.
• Ferry AP, Font RL. Carcinoma metastatic to the eye and orbit. I. A clinicopathologic study of 227 cases. Arch Ophthalmol 1975;93(7):472–482.
• Goldberg RA, Rootman J. Clinical characteristics of Metastatic Orbital Tumors. Ophthalmology 1989;97: 620–624.
• Mehta JS, Abou-Rayyah Y, Rose GE. Orbital carcinoid metastases. Ophthalmology 2006;113(3): 466–472.
• Shields JA, Shields, CL, Scartozzi R. Survey of 1264 Patients with Orbital Tumors and Simulating Lesions. Ophthalmology 2004;11;997–1008.
• Shields JA, Shields CL. Orbital Metastatic Cancer. In: Shields JA, et al. Eyelid, Conjunctival, and Orbital Tumors: an Atlas and Textbook. Orbital Metastatic Cancer. Philadelphia: Lippincott Williams and Wilkins, 2007:728–743.
CODES
ICD9
198.4 Secondary malignant neoplasm of other parts of nervous system
CLINICAL PEARLS
• A quarter of all patients with orbital metastasis do not have a primary diagnoses at the time of presentation.
• An ophthalmologist may therefore be the first to diagnose a serious illness like metastatic cancer. A high index of suspicion and rapid diagnosis is crucial. Prompt referral for systemic work-up can then be pursued.
• Enophthalmos can be the tricky presenting sign of scirrhous breast carcinoma or gastrointestinal cancer. It is often confused with contralateral proptosis.
• Although survival is dismal, local treatment as well as systemic chemotherapy can greatly affect quality of life. Radiotherapy can improve vision.
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