Von Hippel–Lindau disease with concomitant Hodgkin’s disease and congenital hypertrophy of the retinal pigment epithelium

Abstract

The article presents a rare case of combination of von HippelLindau disease and Hodgkins disease. The disease began with neurological symptoms with gradual progression over the next 3 years. The diagnosis of von HippelLindau disease was made after MRI of brain and spinal cord, abdominal MICTs, and detection of brain stem and spinal cord tumors, multiple pancreatic cysts. We performed resection trepanation of the posterior cranial fossa and microsurgical total removal of hemangioblastoma of the medulla oblongata. After 1.5 years the patient is diagnosed with Hodgkins disease and several courses of chemotherapy are carried out, reaching full remission, confirmed by PET with CT. 14 months later, the patient consulted an ophthalmologist due to visual impairment and floating opacities in her left eye. The ophthalmologic examination for the first time revealed multiple bilateral retinal hemangiomas and vitreal hemorrhages from tractional retinal tears caused by posterior hyaloid detachment and unrelated to hemangiomas in the left eye. The barrier laser coagulation of the left eye retinal tears was performed, and the observation tactics was adopted.