Sequential branch retinal artery occlusions following embolization of an intracranial meningioma.
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Central retinal artery occlusion (CRAO) is uncommon in those younger than 50. We present a 42-year-old man who complained of sudden loss of vision in the left eye. Fundus examination showed typical findings of CRAO. Extensive medical work up showed no evidence of diabetes or of cardiovascular, hematologic or collagen disease. However, a malignant tumor of the right testis was found, with metastases in the lung, retroperitoneal lymph nodes and brain. The right testis was resected and chemotherapy and brain irradiation resulted in remission during a follow-up of 2.5 years. The differential diagnosis of CRAO in younger patients is difficult. We believe that metastases from the primary tumor were the cause of CRAO in our patient. The possibility of malignancy with metastatic spread causing CRAO should be considered, particularly in relatively young patients with no other evidence of systemic disease.
BACKGROUND: Very little documentation of spontaneous regression of an angiomatous retinal lesion in v. Hippel-Lindau disease (VHL) exists. It is commonly believed that a spontaneous change of hemangiomas into fibrotic lesions occurred. PATIENTS/METHODS: Follow-up examinations of four patients with VHL in the Freiburg VHL study were carried out. RESULTS: A 16-year-old girl revealed a vascular lesion at the border of the optic disc. Control examination nine years later revealed complete spontaneous regression of the retinal vascular changes. A slight retinal vascular change at the superior border in her right eye was found in a 36-year-old woman. A control examination 20 years later revealed regression of the lesion. A 41-year-old woman showed in the retinal periphery a small fibrotic white hemangioma with a pigmented feeder vessel as sign of spontaneous tumor regression. A 12-year-old boy had a retinal microaneurysm inferior to the optic disc that disappeared several years later. CONCLUSION: Documentations of spontaneous regression of minor angiomatous retinal lesions in VHL exist. Such vascular changes are rare. Every retinal lesion should be controlled by follow-up examination and documentation. In case of retinal lesion growth, treatment is necessary.
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The three classic angiomatoses of the retina are described according to the clinical picture. If early diagnosis is possible, then the inevitable progression to exudative retinal detachment and secondary glaucoma may be avoided by extensive coagulation with laser, xenon or cryopexy. Prognosis is best with Leber's retinitis, much less so in cases of von Hippel-Lindau angiomatosis and primary Coats' disease.
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OBJECTIVE: To study the clinical features and natural history of 17 patients with retinocytoma. DESIGN: A retrospective case series. SETTING: Tertiary referral center. PATIENTS: Data on 17 patients with retinocytoma were reviewed for clinical features. The natural history of retinocytoma and its risk for malignant transformation were also evaluated. RESULTS: Among 920 consecutive patients who had retinoblastoma, retinocytoma, or both, we identified 24 tumors in 17 patients (1.8%) with clinical features compatible with retinocytoma. The median age at diagnosis was 15 years (range, 4-45 years). Of the 24 tumors, the retinocytoma was bilateral in 3 cases (13%) and the family history of retinoblastoma was positive in 3 cases (13%). Seventeen (71%) of the tumors were extramacular in location, and 7 (29%) were located in the macular area. Ophthalmoscopic features characteristic of retinocytoma included the presence of a translucent retinal mass in 21 (88%), calcification in 15 (63%), and retinal pigment epithelial alteration in 13 (54%) of the 24 tumors. A combination of all 3 features was observed in 8 (33%) of the 24 tumors. In 13 (54%) of the tumors, a zone of chorioretinal atrophy could be observed. In 1 patient, subtle tumor regression was documented photographically. Only 1 retinocytoma (4%) underwent malignant transformation into retinoblastoma. At the last follow-up visit, none of the patients had developed a pineoblastoma or another second malignant neoplasm. CONCLUSIONS: Retinocytoma is a rare benign retinal tumor that has characteristic clinical features. The areas of chorioretinal atrophy were suggestive of tumor regression. In our series, the risk for malignant transformation of retinocytoma into retinoblastoma was 4%; therefore, patients with a presumed diagnosis of retinocytoma should be closely observed.
Hematogenous retinal metastases from non-small cell lung cancer are rare, and are even more uncommonly observed bilaterally. Non-small cell lung cancer usually metastasizes to the liver, adrenal glands, lung, bone, central nervous system, and kidney. We report the case of a 41-year-old male patient with advanced lung adenocarcinoma heavily pretreated with polychemotherapy and palliative radiotherapy up to June 2003, when synchronous bilateral retinal metastases were diagnosed. The patient's prognosis was worsened by the onset of the retinal metastases and he died three months later.
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PURPOSE: To determine the safety of pars plana vitrectomy in eyes containing a treated posterior uveal melanoma. DESIGN: Interventional case series. METHODS: Retrospective case series of patients with posterior uveal melanoma who underwent pars plana vitrectomy. Complications, vitreous cytology, local tumor control, and metastasis were assessed. RESULTS: Nine patients met study criteria. Tumors were treated with (125)I plaque radiotherapy (seven patients) or transpupillary thermotherapy (two patients). Vitrectomy was performed for vitreous hemorrhage (five patients), macular pucker (two patients), macular hole (one patient), and rhegmatogenous retinal detachment (one patient). Vitrectomy was performed at a mean of 24.7 months (range, 7-47 months) after melanoma treatment. Dispersion of tumor cells at vitrectomy was not observed in any patients. Melanoma cells were detected in the vitreous aspirate in one of seven cases examined cytologically. This patient had intratumoral and vitreous hemorrhage before plaque radiotherapy, underwent combined vitrectomy/cataract extraction, and developed intraocular tumor dissemination 56 months after vitrectomy. No other patients developed intraocular tumor dissemination. At mean follow-up of 24 months (range, 3-63 months) after vitrectomy, none of the nine patients developed systemic metastasis. CONCLUSIONS: Pars plana vitrectomy rarely may lead to intraocular tumor dissemination, although the risk of this complication is probably low if the tumor has been treated and has responded to therapy before vitrectomy. Vitrectomy should be approached with caution if a vitreous hemorrhage is present, especially if the hemorrhage occurred before tumor treatment, as this may seed tumor cells into the vitreous cavity.
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A family with Von Hippel-Lindau syndrome has been investigated with Ultrasound, CT and MRI. The major lesions found were posterior fossa haemangioblastomas, retinal haemangioblastomas and renal cell carcinoma. Screening of family members has been undertaken due to the known autosomal dominant inheritance of this condition.
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