Vasoproliferative tumors of the ocular fundus. Classification and clinical manifestations in 103 patients
C. L. Shields, J. A. Shields, J. Barrett and P. De Potter
Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pa., USA.
OBJECTIVE: To describe the clinical features of vasoproliferative tumors of
the ocular fundus and to propose a comprehensive classification of these
tumors. METHODS: A retrospective review of all cases that were diagnosed as
acquired retinal hemangioma or vasoproliferative retinal tumor was
conducted on the Ocular Oncology Service at Wills Eye Hospital,
Philadelphia, Pa. RESULTS: There were 129 vasoproliferative tumors in 113
eyes of 103 patients. The tumors were classified as idiopathic in 84 eyes
(74%) and secondary to preexisting ocular disease in 29 (26%).
Subclassification into solitary (88 eyes), multiple (17 eyes), and diffuse
(eight eyes) involvement was made. Of the 84 eyes with idiopathic tumors,
73 (87%) had solitary tumors, five (6%) had multiple tumors, and six (7%)
had diffuse tumors. The lesion was located in the inferior, inferotemporal,
or temporal region of the fundus in 78% and developed within 6 mm of the
ora serrata retinae in 88%. Associated vitreoretinal findings included
intraretinal exudation (82%), secondary exudative retinal detachment (48%),
vitreous cells (46%), vitreous hemorrhage (21%), preretinal macular
fibrosis (31%), and macular edema (18%). Of the 29 eyes with secondary
tumors, the tumor was solitary in 15 (52%), multiple in 12 (41%), and
diffuse in two (7%). The most common preexisting ocular disease included
intermediate uveitis (pars planitis) in eight eyes (28%), retinitis
pigmentosa in six (21%), toxoplasmic retinitis in two (7%), toxocariasis in
two (7%), retinochoroidal coloboma in two (7%), and traumatic
chorioretinopathy in two (7%). Retinal pigment epithelial hyperplasia was a
prominent feature that was adjacent to 58% of the secondary tumors.
Overall, management of the 129 tumors consisted of observation in 63 (49%),
cryotherapy in 54 (42%), laser photocoagulation in seven (5%), plaque
radiotherapy in three (2%), and other modes of treatment in two (2%).
CONCLUSIONS: Vasoproliferative retinal tumors can be idiopathic, or they
can develop secondary to congenital, inflammatory, vascular, traumatic,
dystrophic, and degenerative ocular diseases. They can produce a variety of
complications. Awareness and recognition of these tumors and
differentiation from other retinal vascular tumors are important.
