Abstract
A wide variety of space occupying lesions may be encountered in the orbit. CT and MR imaging frequently help confirm the presence of a mass and define its extent. Characteristic imaging features may help distinguish among lesions that have overlapping clinical presentations. This review focuses on some of the common orbital masses. Common vascular lesions that are reviewed include: capillary (infantile) hemangioma, cavernous hemangioma (solitary encapsulated venous-lymphatic malformation), and lymphangioma (venous-lymphatic malformation). Benign tumors that are reviewed include: optic nerve sheath meningioma, schwannoma, and neurofibroma. Malignancies that are reviewed include: lymphoma, metastasis, rhabdomyosarcoma, and optic glioma. Key imaging features that guide radiological diagnosis are discussed and illustrated.
Keywords: Benign orbital masses, Orbital vascular lesions, Malignant orbital masses, Pediatric orbital masses
Introduction
A wide variety of processes can produce space-occupying lesions in and around the orbit. These include benign neoplasms, malignant neoplasms, vascular lesions, inflammatory disease, congenital lesions, and infection, among other causes. Imaging can be used to precisely localize a lesion, to help establish a diagnosis or generate a differential diagnosis that guides management, to follow a known lesion for progression, or some combination of these.
The first step in establishing a differential diagnosis is obtaining an accurate clinical history. CT is excellent for confirming the presence of a mass, is well tolerated, can be rapidly obtained at most medical centers, and gives clues to the specific diagnosis. Although MRI can be more difficult to perform, it can reveal additional key imaging features that allow one to more confidently distinguish between similar appearing lesions, and often arrive at a single most likely diagnosis.
Our goal is to review the most commonly encountered lesions to make the reader aware of their typical appearances and presentations, with emphasis on key imaging features that may distinguish among similar lesions.
This review focuses on the most common vascular masses, benign neoplasms, and malignant neoplasms. Due to space constraints, infection1–3 and inflammatory disease3–6 are not discussed here. In addition, a number of other vascular lesions and neoplasms are not discussed, including orbital venous varix,3,7,8 arteriovenous malformation,3,9 cavernous sinus thrombosis,3,10 carotid cavernous fistula,3,7,8 a variety of benign and malignant mesenchymal tumors,3,11,12 epithelial neoplasms of the lacrimal apparatus,3,7,13–15 and ocular tumors that secondarily involve the orbit.3,7 One may also encounter other masses that are not true neoplasms, such as dermolipomas,3,16,17 dermoid cysts,3,18–20 and epidermoid cysts.3,20 For discussion of these lesions, and others, we direct the reader to the referenced sources.
Vascular lesions
Capillary hemangioma (infantile hemangioma)
Clinicopathologic features
Although the term “hemangioma” is sometimes used to describe certain vascular malformations, it more appropriately describes a true neoplasm with vascular channels lined by proliferating endothelial cells.21 Capillary hemangiomas are the commonest tumors of infancy,7,22–24 typically appearing as a reddish macule in the first 6 months of life. A proliferative phase occurs up to 10 months, followed by a slow involution phase for up to 10 years.21,25,26 Though typically sporadic, they may occur as part of a genetic syndrome such as PHACES syndrome.25
Imaging findings
Contrast enhanced MRI is the preferred modality for imaging hemangioma, although CT can also be considered if it is not possible to sedate the patient for MRI. The diagnosis is typically well-established clinically, but imaging is indicated to assess the extent of the lesion and mass effect on adjacent structures. With deep lesions, the clinical diagnosis may be challenging. Capillary hemangioma typically shows: (1) lobular contour borders, (2) bright T2 signal with T2 dark septa between lobules, (3) fine internal flow voids, (4) intense, homogeneous enhancement, and (5) preservation of adjacent bone (Fig. 1). Capillary hemangioma can have an atypical appearance without all of these features. Nevertheless, if these features are not all present, then alternative diagnoses should be considered, including rhabdomyosarcoma. DWI may help distinguish these lesions in challenging cases, as rhabdomyosarcoma typically shows lower ADC and brighter DWI signal than hemangioma.27–29 CT may demonstrate bony orbital expansion or scalloping with rapidly growing lesions. True bone invasion and calcifications are rarely seen.
CT and MR may both demonstrate fat deposition and heterogeneous appearing enhancement, if imaging is performed during the involution phase.9,23–25
Differential diagnosis
Rhabdomyosarcoma, vascular malformation, infantile fibromatosis, and infantile fibrosarcoma.
Key features
Bright T2 signal, lobular borders, fine internal flow voids, extraconal location, and intense homogeneous enhancement.
‘Cavernous hemangioma’ (solitary, encapsulated venous-lymphatic malformation)
Clinicopathologic features
‘Cavernous hemangioma’ is a term that is widely used to describe a solitary, encapsulated venous-lymphatic malformation, the most common vascular lesion of the orbit.30 The typical clinical presentation is of mostly painless proptosis (mean 5–6 mm), pain, lid swelling, diplopia, lump, and recurrent obstructed vision. Middle-aged women are the most commonly affected group, and the average duration from symptom onset to presentation is 4 years.22 The most frequent locations are the retrobulbar muscle cone, especially the lateral aspect of the intraconal space.31 However, a small minority (less than 10%) of these lesions are extraconal.32 Cavernous hemangiomas rarely bleed33 due to firmer texture from the surrounding support of rich fibrous tissue.34 Orbital cavernous hemangiomas are typically solitary and unilateral, though multifocal and bilateral lesions have been reported.35–38
Imaging appearance
Cavernous hemangioma typically appears as a well-circumscribed intraconal mass.39–42 Although most lesions are ovoid or round, larger lesions have lobulated margins. Larger lesions will distort surrounding structures, as opposed to lymphoma which molds around structures. CT shows homogeneous soft tissue density, and may show small calcifications or phleboliths.39 MR shows isointense T1 signal, bright T2 signal, dark internal septations, and a dark circumferential rim that represents a fibrous pseudocapsule (Fig. 2).22,34,43,44
MR classically demonstrates nodular enhancement on early post-contrast images, and progressive accumulation of contrast on delayed post-contrast images.31 The same findings are seen with multiphase CT, though concerns related to the increased radiation exposure of multiphase CT make this technique relatively less desirable. Small lesions often show early, uniform enhancement. MR angiography and CT angiography are generally not able to identify the feeding vessels of a cavernous hemangioma, likely due to their small caliber.
Differential diagnosis
Venous varix, schwannoma, optic nerve sheath meningioma, and lymphoma.
Key imaging features
Intraconal location; very bright T2 signal with hypointense pseudocapsule; and an early nodular enhancement with progressive accumulation of contrast on later phase images.
Lymphangioma (venous lymphatic malformation)
Clinicopathologic features
‘Lymphangiomas’ are benign vascular malformations (venous lymphatic malformations), commonly affecting children and rarely involving the orbit. They are unencapsulated and composed of fibrous material including endothelial lacunae filled with blood or serious fluid.45 Lymphangiomas tend to enlarge suddenly due to intralesional bleeding, but may also enlarge with upper respiratory infection.46 Symptoms include: swelling, intraorbital hemorrhage, ocular proptosis, blepharoptosis and cellulitis. Ocular complications include: astigmatism, corneal exposure, hyperopia secondary to pressure on posterior globe, strabismus, glaucoma and compressive optic neuropathy.47–49
Imaging findings
Despite belonging to the same pathological category as ‘cavernous hemangioma’ (i.e. low flow vascular malformation without endothelial proliferation), lymphangioma has a different appearance and growth pattern. In keeping with their unencapsulated nature, lymphangiomas exhibit an infiltrative, trans-spatial growth pattern, often involving both the intraconal and extraconal compartments and pre- and post-septal compartments, violating fascial planes. Both microcysts and macrocysts may be identified.50 CT may demonstrate phleboliths in the venous component of a lesion, and may demonstrate bony abnormalities.46 Venous or solid components of the tumor are hyperdense compared to brain tissue on unenhanced CT scans.50–52
MR is accurate for delineating the anatomic location and vascular components, and fluid filled levels or menisci can be seen.46 The mass is usually isointense to slightly hyperintense relative to normal brain tissue on T1 weighted imaging and very hyperintense relative to the brain on T2 weighted imaging46, with internal septations (Fig. 3). T1 and T2 signal intensity vary depending on the presence and age of internal blood products. The venous component of a lesion will typically enhance, whereas the lymphatic component will only show fine enhancement of septations. No flow voids or enlarged feeder vessels are usually found, in keeping with the low flow nature of lymphangioma and differentiating it from high flow lesions, including high-flow vascular malformations and true neoplasms such as capillary hemangioma.50,53,54
Differential diagnosis
Cavernous hemangioma, capillary hemangioma, dermoid, and rhabdomyosarcoma.
Key imaging features
Trans-spatial, markedly T2 bright non-enhancing mass with internal septations, with or without layering blood products and solidly enhancing components.
Benign neoplasms
Optic nerve sheath meningioma
Clinicopathologic features
The typical clinical presentation of optic nerve sheath meningioma is painless, gradual vision loss and proptosis in a woman between ages 30 and 50. Optic nerve sheath meningioma is typically unilateral but can be bilateral, particularly in the setting of type II neurofibromatosis. Primary meningioma arises from capillary cells of the arachnoid around the intraorbital or intracanalicular portions of the optic nerve. Secondary meningioma arises intracranially from the sphenoid ridge, tuberculum sellae, or olfactory groove and invades the optic canal and orbit by extension between the dura and arachnoid of the optic nerve.55,56 Visual disturbance in the affected eye is common. While some patients only suffer transient visual loss lasting a few seconds, others experience visual loss in a particular field of gaze.57,58 Optic neuropathy, proptosis and strabismus occur later.
Imaging findings
The key imaging finding of optic nerve sheath meningioma is a homogeneously enhancing mass that surrounds the optic nerve. CT may show calcification. MRI typically shows homogeneous, intermediate T1 and T2 signals. The optic nerve may be in the center of the lesion, or may be eccentrically positioned. In some cases, the optic nerve will show abnormal T2 hyperintense signal, which is presumably related to chronic venous insufficiency.
Optic nerve sheath meningioma may produce an expansile mass (Fig. 4). Alternatively, it may only form a thin sheet around the optic nerve, termed “en plaque meningioma”, producing the classically described ‘tram track’ sign.59 In these cases, fat-suppressed post-contrast T1-weighted images in the coronal plane are essential in making the diagnosis (Fig. 5). Any part of the nerve may be involved, and radiologic diagnosis can be challenging when only the intracanalicular portion of the nerve is affected. Fat suppression is helpful in these cases, but can also be degraded by blooming artifact around a well-pneumatized sphenoid sinus. For this reason, coronal post-contrast images without fat suppression should also be considered.
Optic nerve sheath meningioma is distinguished from optic glioma by the fact that the optic glioma expands the optic nerve. Orbital lymphoma can sometimes surround the optic nerve and simulate optic nerve sheath meningioma by imaging (Fig. 6). In such cases, lymphoma’s tendency to mold around structures helps differentiate it from meningioma, which tends to distort structures. Lymphoma also typically shows less intense enhancement than meningioma. Meningioma frequently shows calcification on CT, whereas untreated lymphoma almost never calcifies. Perineuritic inflammatory disease can produce a similar appearance to en plaque meningioma, but is clinically distinguished by the presence of pain. Demyelinating optic neuritis is distinguished from en plaque meningioma by its typical homogeneous enhancement of the substance of the nerve, as opposed to the target-like enhancement of meningioma.
Differential diagnosis
Sarcoidosis, pseudotumors, lymphoma, and optic neuritis.
Key imaging features
Mass surrounding and constricting the optic nerve; calcification on CT; extension of tumor into the optic canal; “tram track” or “target” like enhancement.
Nerve sheath tumors
Clinicopathologic features
Nerve sheath tumors can be divided into schwannomas and neurofibromas, which are less common and typically associated with NF-1. Schwannomas constitute 1% of orbital tumors, typically occurring in young and middle-aged adults.30,60 They typically arise from intraorbital branches of V1,60 and produce symptoms related to local mass effect. Schwannomas are associated with NF-1 in 2–18% of patients.61 Neurofibromas are more strongly associated with NF-1, and also produce symptoms related to mass effect.
Imaging findings
Schwannoma appears as a smooth, ovoid, orbital retrobulbar mass, found in the intra- or extra-conal space with the long axis in the anterior–posterior direction (Fig. 7).39,62 CT shows isodensity or hypodensity compared to gray matter. MR shows isointense T1 signal, and T2 hyperintensity. Although some schwannomas may be cystic and non-enhancing,63 most schwannomas enhance with contrast. Although schwannoma enhancement is typically more homogeneous than cavernous hemangioma, in some cases schwannoma can show the same type of early nodular enhancement with progressive fill-in that is commonly seen with cavernous hemangioma. The MR and CT appearance of schwannoma can overlap with optic nerve sheath meningioma. In challenging cases, one should look carefully for extension into the superior orbital fissure, which would favor schwannoma, or for extension into the optic canal, which would favor meningioma. Neurofibromas often have a similar appearance to schwannomas, though plexiform neurofibromas typically show a more infiltrative growth pattern (Fig. 8), and are frequently associated with other stigmata of NF-1.
Differential diagnosis
Cavernous hemangioma, meningioma, and lymphoma.
Key imaging features
Smooth, homogeneous spherical mass. Extension into superior orbital fissure. Enhancement is typically more heterogeneous than lymphoma or meningioma, but less nodular than cavernous hemangioma.
Malignant neoplasms
Orbital lymphoid tumors (including malignant lymphoma)
Clinicopathologic features
Reactive lymphoid lesions overlap histologically with malignant lymphoma, and are sometimes grouped as ‘orbital lymphoid lesions’.64 The typical presentation is low-grade proptosis with minimal pain.1 Lymphoid masses comprise 10–15% of all orbital tumors65 and up to 55% of malignant orbital masses.66 Malignant lymphoma of the orbit is typically B cell lymphoma of a non-Hodgkin type, arising from mucosa-associated lymphoid tissue of ocular adnexa (MALTOMA).67 Systemic lymphoma may involve the orbit in 1.5–5% of patients.68,69 Up to 75% of patients with orbital lymphoma may later develop systemic disease.70 Suspicion of malignant lymphoma is increased in the presence of systemic disease, lacrimal duct or gland disease, and bilateral disease.71
Image appearance
Lymphoid tumors typically appear as homogeneous, lobulated masses on CT and MRI. They mold around normal structures without deforming them, and typically do not erode adjacent bone. They may be well defined, or infiltrative in appearance. CT shows density similar to skeletal muscle. MRI demonstrates homogenous, intermediate T1 and T2 signals, and homogeneous contrast enhancement.64,72,73
Lymphomas most commonly involve the superolateral aspect of the orbit,72 and bilateral disease is common.1 When multiple sites of disease are present, the intraconal space is most affected (85%). Lymphoma can involve virtually any part of the orbit, including the lacrimal gland, extraocular muscles, lacrimal sac, periorbital fat, and retrobulbar fat (Fig. 9).
Lymphoma can be very difficult to distinguish from orbital inflammatory disease (“pseudotumor”). However, diffusion-weighted imaging (DWI) is an MRI technique that has recently been proven effective in distinguishing these entities. Lymphoma typically shows brighter DWI signal and lower apparent diffusion coefficient (ADC) than normal orbital structures, due to its hypercellular nature. Inflammatory lesions, on the other hand, tend to show intermediate DWI and ADC signals, similar to normal lacrimal gland.27 Very low T2 signal (darker than normal white matter) should also suggest an inflammatory lesion.5 DWI is also helpful for identifying lacrimal gland lymphoma.
Differential diagnosis
Orbital inflammatory syndrome, optic nerve sheath meningioma, and orbital metastasis.
Key imaging features (lymphoma)
Homogeneous intermediate T2 signal, lobulated margins with molding around normal structures, homogeneous enhancement, brighter DWI signal and lower ADC than surrounding normal orbital tissues.
Metastatic tumors of the orbit
Clinicopathologic features
In adults orbital metastases usually arise from carcinomatous origin, in children sarcomatous or neural embryonal tumors are more common.74 The spread of tumor to the orbit and ocular adnexa is usually due to hematogenous spread of primary cancer, and is associated with a poor prognosis. Primary metastatic orbital tumors are estimated to account for 1–13% of all orbital tumors, and in cancer patients are prevalent from 2% to 4.7%.75 Improved cancer therapy, improved diagnostic imaging and increased awareness in medical literature have allowed for better diagnosis of these tumors.
Metastatic lesions are found more in the anterior orbit than the posterior orbit.76 The most frequent metastases to the orbit are from: breast, lung, prostate, melanoma, carcinoid, GI, renal cell, neuroblastomas and rhabdomyosarcomas. Rapidly progressive symptoms occur including: diplopia, proptosis, decreased vision, pain, ptosis, and palpable mass.75
Imaging findings
CT is usually performed for orbital imaging, although MR has superior soft tissue contrast. Tumor pattern can vary from diffusely infiltrative with obscured anatomical landmarks to a focal well-defined mass. Orbital metastases from breast cancer are often diffuse and irregular growing along the rectus muscles and fascial planes.76 Scirrhous (fibrotic) breast cancers are unique in their ability to produce enophthalmos and ophthalmoplegia. In these cases, the metastatic lesion is typically very T2 dark, reflecting its fibrotic nature (Fig. 10). Metastases from carcinoid (Fig. 11), renal cell carcinoma and melanoma tend to be circumscribed. All orbital metastases show some degree of MR enhancement.77 Metastases may involve any structure in the orbit, including the intraconal or extraconal space, globe, extraocular muscles and bone (Fig. 12).
Differential diagnosis
Lymphoma, infection, dysthyroid orbitopathy, orbital inflammatory disease, carotid cavernous fistula, and cavernous sinus thrombosis.
Key imaging findings
Imaging findings vary widely. Globe retraction is highly suspicious for scirrhous breast cancer metastasis.
Rhabdomyosarcoma
Clinicopathologic features
This is the most common soft tissue malignancy of childhood and most common primary orbital malignancy.78,79 There is a bimodal distribution of affected ages, commonly occurring between 2 and 5 years80 but is reported in all age groups.81 The embryonal subtype occurs in childhood (mean age 8 years; male to female ratio 5:3); the pleomorphic subtype occurs in adults; the alveolar subtype occurs in young children and adults and is associated with a poor prognosis.79
Primary rhabdomyosarcoma can occur from the conjunctiva, iris, ciliary body, or extension of primary orbital rhabdomyosarcoma.82 Secondary orbital rhabdomyosarcoma occurs from direct extension to orbits from the paranasal sinus, pterygopalatine fossa, infratemporal fossa or nasopharynx. Orbital metastases can also occur from other head and neck rhabdomyosarcomas and carries a poor prognosis. The orbital location varies: extraconal (37%), intraconal (17%), both (47%). The upper outer quadrant is involved in 67%. Presenting complaints include: unilateral proptosis (80–100%), globe displacement (80%), ptosis (30–50%), conjunctival and eyelid swelling (60%), palpable mass (25%), and pain (10%).83,84
Imaging findings
CT shows moderately well defined to ill-defined margins, irregular shape, and mild-moderate contrast enhancement. Adjacent bony destruction occurs in 40% (Fig. 13). Globe distortion85 and extension to the paranasal sinuses82,86 may also be seen. Regional lymph nodes are involved later in the disease course. Calcification is rare in untreated tumors.
MR typically shows bright T2 signal, distinguishing rhabdomyosarcoma from other tumors such as chloroma (granulocytic sarcoma), lymphoma and metastatic neuroblastoma (Fig. 14). MR may also better delineate the true extent of disease if surgical resection is planned.87 On occasion, a pyogenic abscess may have a subacute presentation that mimics a necrotic rhabdomyosarcoma clinically and by imaging.88 In such cases, MRI with DWI is critical in distinguishing these entities, through demonstration of restricted diffusion of pus in an abscess cavity,89 as opposed to elevated diffusion in the necrotic portion of a tumor.90
Differential diagnosis
Leukemic metastatic deposits; Langerhans cell histiocytosis; aggressive fibromatosis; ruptured dermoid cyst; subperiosteal hematoma; plexiform neurofibromatosis, capillary hemangioma.
Key imaging features
CT: variable definition, bony destruction; MR: bright T2 signal.
Optic nerve glioma
Clinicopathologic features
Optic nerve glioma comprises 1.5–3.0% of orbital tumors, 0.6–7.0% of all intracranial tumors, 1.7–7.0% of gliomas, and 2–5% of gliomas in the pediatric age group.91,92 The peak incidence is in 2–8 year old but can occur at any age and has been reported at up to 79 years of age.93,94 Females are more affected than males. There is a high association (12–37%) with type I neurofibromatosis type 195, which frequently shows bilateral lesions. Notably, optic gliomas associated with NF-1 typically have more indolent behavior than isolated optic nerve gliomas. Symptoms include: proptosis, decreased visual acuity,96 nystagmus, strabismus, central/paracentral field defects, dyschromatopsia, and afferent pupillary defect.92 Disk edema and atrophy may be evident by 8 weeks. The tumors are slow growing with periods of growth and dormancy,97,98 some may regress completely.99,100 In an analysis of affected orbital locations, 48% involved the orbital optic nerve, 24% the orbital and intracranial optic nerve, 10% intracranial optic nerve and chiasm, and just chiasm in up to 5%.101 Surgery is typically reserved for cases where there is significant mass effect.
Imaging findings
MR is optimal for imaging optic nerve glioma.102 The classic finding of optic nerve glioma is sharply circumscribed fusiform thickening and tortuosity of the optic nerve. Optic gliomas are typically T2 hyperintense, and usually show some enhancement, though a wide range of signal intensities and enhancement patterns may be encountered.103 Diffuse involvement of the substance of the nerve differentiates optic nerve glioma from optic nerve sheath meningioma, which surrounds the optic nerve. Any part of the optic nerve may be involved, from the globe to the optic chiasm. Peripheral enhancement of chiasmatic gliomas represents extraneural growth of tumor into the subarachnoid space, mixed with gliomatous tissue and non enhancing central tumor. Optic nerve gliomas that extend to the chiasm through the optic canal form a dumbbell shape (Fig. 15).
Differential diagnosis
Optic nerve sheath meningioma and medulloepithelioma.
Key imaging features
Diffuse enlargement of the substance of the optic nerve, with thickening and tortuosity.
Conclusion
A wide variety of lesions may be encountered in the orbit. Knowledge of the most common lesions, their typical clinical presentation, typical imaging appearance, and key features that distinguish from similar lesions should allow the reader to make the best use of imaging.
Footnotes
Peer review under responsibility of Saudi Ophthalmological Society, King Saud University.
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