TABLE 1. Vascular Tumors of the Orbit


Lesion Equivalent Terms Flow Clinical Features
Age at Diagnosis Typical Presentation Proptosis Strabismus Decreased Vision Other Associations
Hamartoma
Infantile capillary hemangioma Benign hemangioendothelioma
Strawberry nevus
Hemangioblastic hemangioma		 High ½ at birth; almost all by 6 mo Diffuse subcutaneous ± upper eye-lid dimpled, red dermal lesion 38% 34% 44–64% Amblyopia 29% Dermal, laryngeal, visceral or intracerebral hermangiomas
Thrombocytopenia and bleeding; diathesis with large visceral lesions (Kasabach-Merritt syndrome)
Cavernous hemangioma   Low 40–50s(Children-78 years) Gradual painless axial proptosis 70% Rare 16% Optic neuropathy Blue rubber bleb nevus syndrome
Lymphangioma   None First decade (Birth–73 years) Eyelid cysts ± conjunctival cysts ± deep component       17% involvement hard and soft palate, paranasal sinuses
Intracranial vascular anomalies
Congenital ocular malformations
Neoplasma
Hemangiopericytoma   High 40–50s (20 mo–87 years) Gradual painless nonaxial proptosis 76% 21% 24% Involvement retroperitoneum or lower limbs
Malignant hemangioendothelioma Angiosarcoma
Malignant angioma
Malignant endothelioma
Angioendothelioma
Hemangioendothelial sarcoma		 High Children (2 wk–68 years) Rapid painful proptosis       25% Associated local sensory or motor neurological deficits
Kaposi' sarcoma   None   Painless reddish blue mass eyelids/conjunctiva        
Angiolymphoid hyperplasia Kimura' disease None Teens and 20s Painless violaceous adnexal lesions       AIDS
Vascular leiomyoma Angiomyoma
Hemangioleiomyoma		   Usually ≤40years Painless proptosis       Head and neck angiomas ± systemic eosinophilia
Lesion Test(s) if suspect Ultrasound CT MRI (Compared to Gray Matter) Angiography Encapsulated Natural History Management
IR IS SA Typical Calcification
Infantile capillary hemangioma Clinical diagnosis ± enhanced MRI High and low Irregular Moderate Well-defined to irregular margins; intraconal/extraconal; moderate to intense enhancement ± globe indentation ± orbital bony enlargement No T1: Isointense Multiple feeding vessels ICA ECA No Complete resolution up to 60% age 4 years and 76% age 7 years ± Intralesional steroids
± Systemic steroids, laser, surgery
Cavernous hemangioma Enhanced CT High Regular Moderate Oval or rounded intraconal mass; progressive homogeneous or inhomogeneous enhancement ± globe indentation ± orbital bony enlargement Rarely T1: Isointense
T2: Hyperintense		 Not indicated Yes Slowly enlarge ± Surgical excision
Lymphangioma Unenhanced MRI Low Regular Marked Poorly defined intraconal/extraconal mass with rim enhancement ±globe indentation ± orbital bony enlargement Yes T1: Hypoisointense(lymphatic cyst)
Hyperintense (hemorrhagic cyst)
T2: Hyperintense		   No Slowly enlarge until adulthood; spurts of growth with RTI and hemorrhages ± Surgical debuilding
Hemangiopericytoma CT or MRI ± cerebral angiography Low to medium Regular/slightly irregular Mild to moderate Oval or rounded extraconal superior mass; intense early enhancement ± orbital bone erosion Yes T1: Isointense
T2: Isotense
Heterogeneous enhancement		 Intense blash; multiple feeding vessels Pseudocapsule Aggressive local behavior ± distant metastases ± Surgical excision
Malignant hemangioendothelioma CT or MRI ± cerebral angiography   Irregular   Circumscribed or infiltrative orbital mass; intense early enhancement ± orbital bone erosion       Pseudocapsule Aggressive local behavior ± distant metastases Surgical excision
Kaposi's sarcoma Clinical ± flourescein angiography               No Slowly enlarge ± Surgical excision, cryotherapy, radiation
Intralesional chemotherapy
Systemic chemotherapy
Angiolymphoid hyperplasia         6       No   Surgical excision ± radiation
Vascular leiomyoma Enhanced MRI       Intraconal mass   T1: Isointense
T2: Hyperintense		   Yes Slowly enlarge Surgical excision

IR, internal reflectivity; IS, internal structure; SA, sound attenuation; ICA, internal carotid artery; ECA, external carotid artery; RTI, respiratory tract infection