The ultimate treatment has not yet been determined, but it seems advisable to do fundus photography and examine the lesion periodically if it is small and asymptomatic. The other 4 patients we previously reported are currently being managed by observation only. However, the patient reported here required active treatment because the tumor was enlarging and was producing vision-threatening complications. Although the best treatment for symptomatic cases is unknown, options include treating the macular edema medically or with grid laser photocoagulation, or treating the tumor with laser photocoagulation, cryotherapy, plaque brachytherapy, or local resection. Local resection was performed in our patient with the hope that removing the tumor completely would stabilize the progressive complications.
Most tumors are relatively small and show little change in the early stages.
With time, however, there is slow enlargement of the mass and progressive exudative retinopathy, often with preretinal macular gliosis and cystoid macular edema causing visual loss. The systemic prognosis is excellent. To our knowledge, no adenocarcinoma of the RPE has exhibited distant metastasis. Accepted for publication September 8, 2000. The authors acknowledge Eric Gurwin, MD, Steven Cohen, MD, and Carl Regillo, MD, for their assistance in the care of this patient, and Ramon Font, MD, for confirming the histopathologic diagnosis in consultation.