Tumors: Vascular

Jacqueline R. Carrasco


BASICS


DESCRIPTION


• Orbital vascular tumors encompass a wide variety of pathology. They can be subdivided by histopathology and growth characteristics (1).


• Capillary hemangiomas


• Venous vascular malformations (cavernous hemangiomas and orbital varices)


• Venous lymphatic malformations


• Arteriovenous lesions


• Neoplasms (i.e., hemangiopericytoma)


• Orbital vascular tumors may also be classified by hemodynamic characteristics (2)


– No flow (lymphangioma)


– Venous flow (varix)


– Arterial flow (arteriovenous malformation)


EPIDEMIOLOGY


Incidence


• Vasculogenic tumors make up 17% of all orbital tumors (3).


– Capillary hemangioma (3%)


– Cavernous hemangioma (6%)


– Varix 2%


– Lymphangioma (4%)


– Hemangiopericytoma (1%)


Prevalence


Unknown


PATHOPHYSIOLOGY


See below


ETIOLOGY


• Capillary hemangiomas: Unknown but current theory is that they are of placental origin and may be either emboli or direct extension of placental tissue.


• Cavernous hemangioma: Some have proposed that these are of venous origin; others theorize that they are low flow arteriovenous malformations. Growth may be mediated by angiogenic or cytokine induced factors due to association between growth of hemangioma and puberty and pregnancy.


• Lymphangioma: A vascular malformation that occurs during embryogenesis from sequestration of lymphatic tissue.


• Varix: Results from presumably a congenital weakness in the postcapillary venous wall, which leads to proliferation of venous elements and massive dilation of the valveless orbital veins.


• Arteriovenous lesions: Derived from multiple congenital microvascular connections between arteries and veins without a capillary bed.


• Hemangiopericytoma: There is controversy as to the existence of these as a distinct entity versus part of the category of solitary fibrous tumors. Thought to be derived from vascular pericytes, contractile. 15% metastasize.


COMMONLY ASSOCIATED CONDITIONS


• Kasabach-Merritt syndrome is a syndrome of platelet entrapment and thrombocytopenia associated with hemangiomas.


• Blue rubber bleb nevus syndrome is associated with multiple cavernous hemangiomas.


• Orbital lymphangiomas have been seen with orbital and intracranial arteriovenous malformations.


• Orbital varices can be associated with defects in orbital bone and cephaloceles.


• PHACE syndrome (posterior fossa brain malformations, large facial hemangiomas, arterial anomalies, cardiac anomalies, and eye abnormalities) is associated with capillary hemangiomas.


• Wyburn-Mason syndrome is associated with arteriovenous malformations.


DIAGNOSIS


HISTORY


• Capillary hemangiomas appear in the first few months of life as a soft bluish mass deep to the eyelid. Typically they progress during the first few months of life, then stabilize at 6–12 months, and regress over the next 5–7 years. Often they have accompanying skin changes, “strawberry hemangiomas” that help make the diagnosis. More common in females.


• Cavernous hemangioma is most common in the fifth decade (40s). Patients typically present with unilateral painless proptosis without involution. More common in women.


• Lymphangiomas are probably present at birth but don’t become apparent until years later when the lesions abruptly present due to hemorrhage.


• Varix: Presents in young adulthood associated with positional proptosis (worse with Valsalva or bending over).


• Hemangiopericytoma: Mean age of presentation is fifth decade with slowly progressive proptosis, sometimes painful. More common in orbit in males.


• Arteriovenous malformation: These are rare in the orbit. Typically present with periorbital swelling.


PHYSICAL EXAM


• External skin changes


• Ptosis


• Astigmatism


• Amblyopia


• Lagophthalmos/corneal exposure


• Afferent pupillary defect


• Restriction of motility


• Proptosis


• Color vision deficit


• Visual field constriction


• Dilated exam: Choroidal folds


• Glaucoma


• Changes in vascular tumors in size can occur with crying, coughing, and upper respiratory infections due to vascular or lymphatic engorgement.


DIAGNOSTIC TESTS & INTERPRETATION


Imaging


• CT or MRI will help delineate characteristic features of the tumor (4)


– Capillary hemangioma: Round, ovoid circumscribed mass.


– Cavernous hemangioma: Round, ovoid mass usually in the muscle cone. May also occur in bone. Progressive accumulation of contrast in late phases due to low flow.


– Lymphangioma: «Chocolate cysts» as seen on imaging are hemorrhagic cystic structures. Sometimes these have fluid–fluid levels within the cyst based on different ages of the fluid.


– Varix: Round or irregular mass that becomes apparent with Valsalva.


– Hemangiopericytoma: Typically lobulated and well-circumscribed but may have infiltrative margins.


– Arteriovenous malformation: May need angiographic protocol or traditional angiography to diagnose.


Diagnostic Procedures/Other


Fine needle aspiration or total excision may be necessary if there is diagnostic uncertainty or a concern for malignancy.


Pathological Findings


• Capillary hemangioma: Capillary sized vascular spaces surrounded by proliferating benign endothelial cells.


• Cavernous hemangioma: Dilated congested vascular channels lined by flattened endothelial cells with an intervening fibrous interstitium.


• Lymphangioma: Dilated ecstatic vascular channels filled with clear fluid or blood.


• Varix: Dilated veins with hyalinization and thrombosis.


• Hemangiopericytoma: Staghorn-like capillary spaces lined by proliferating pericytes. One-third are histopathologically consistent with malignancy.


• Arteriovenous malformation: Multiple congenital connections between arteries and veins without a capillary bed.


DIFFERENTIAL DIAGNOSIS


• Orbital tumors (meningioma, glioma)


• Metastatic orbital tumors


• Idiopathic orbital inflammation


• Thyroid-related orbitopathy


TREATMENT


MEDICATION


First Line


• Capillary hemangiomas: Observation; topical, oral or intralesional corticosteroids; interferon alpha; laser; surgical. A new noninvasive therapy is beta-blocker propanolol (5)[C].


• Cavernous hemangioma: Typically conservative unless unacceptable proptosis or visual deficit makes surgery an option.


• Varix: Surgical excision if unacceptable cosmetic changes or visual compromise.


• Lymphangioma: Observation is typical, but if there is unacceptable proptosis or visual compromise, treatment includes intralesional injection of sclerosing agents (6)[C], carbon dioxide ablation (7)[C], and intralesional injection of steroids. Surgical management is very difficult as it is challenging to remove the entire growth.


• Hemangiopericytoma: Standard treatment is wide surgical excision with postoperative irradiation for incompletely excised tumors.


• Arteriovenous malformation: Embolization and in some cases, surgery.


SURGERY/OTHER PROCEDURES


• Observation is acceptable for vascular tumors that do not cause visual deficit or facial deformity.


• Surgical removal or debulking can be difficult with vascular tumors because of the risk of bleeding.


• Surgical treatment is recommended when the tumor becomes symptomatic, visually or cosmetically.


ONGOING CARE


PROGNOSIS


Varies with the type of vascular tumor and its location in the orbit


COMPLICATIONS


Incomplete excision of hemangiopericytoma may lead to malignant transformation.



REFERENCES


1. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children. A classification based on endothelial characteristics. Plast Reconstr Surg 1982;69(3):412–422.


2. Harris GJ. Orbital vascular malformations: A consensus statement on terminology and its clinical implications. Orbital Society. Am J Ophthalmol 1999:127(4):453–455.


3. Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions. Ophthalmology 2004;111(5):997–1008.


4. Smoker W, Gentry L, Yee N, et al. Vascular lesions of the orbit: More than meets the eye. Radiographics 2008;28(1):185–204.


5. Leaute-Labreze C, Dumas de la Roque E, Hubiche T, et al. Propanolol for severe hemangiomas of infancy. N Engl J Med 2008; 358(24):2649–2651.


6. Burrows PE, Mason KP. Percutaneous treatment of low-flow vascular malformations. J Vas Interv Radiol 2004;15:431–445.


7. Kennerdell JS, Maroon JC, Garrity JA, et al. Surgical management of orbital lymphangioma with the carbon dioxide laser. Am J Ophthalmol 1986;102:308–314.

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Nov 9, 2016 | Posted by in OPHTHALMOLOGY | Comments Off on Tumors: Vascular

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