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Retinal astrocytoma is a rare and benign tumor of the retina most frequently seen in patients with tuberous sclerosis or, less freqently , neurofibromatosis. Sporadic cases occur in less than 30% of the total cases reported.

These lesions are often referred to as hamartomas.

Astrocytoma of the retina are probably best regarded as (benign) pilocytic astrocytomas, comparable to the tumours seen in the optic nerve.

Longstanding cases may be severely calcified and thus may radiologically resemble a retinoblastoma .

 Retinal astrocytoma can show progressive enlargement, retinal detachment, and vitreous seeding, findings that can mislead the clinician toward the diagnosis of retinoblastoma or choroidal melanoma.     

   

Optic nerve pilocytic astrocytoma with retinal involvement.Arch Soc Esp Oftalmol. 2005 Dec;80(12):733-6

INTRODUCTION: This is an atypical case of a pilocytic astrocytoma that involved the optic nerve (ON) and the retina. CLINICAL CASE: The patient was a 30-year-old male, who had attended ONCE since his early childhood because of the suspicion of an intraocular tumor. The ophthalmology exploration showed an ON and retinal coloboma in the right eye and microphthalmy, shutting of the pupil, retinal detachment and proof of an intraocular tumor in the left eye. The MR revealed an ON tumor that involved the retina. The histopathological study after enucleation was pilocytic astrocytoma. DISCUSSION: Long standing congenital pathology in a blind patient does not exclude the possibility of the patient suffering from other pathology as well.

Intraocular astrocytoma without phacomatosis. Eur J Ophthalmol. 2004 Jul-Aug;14(4):350-4.

PURPOSE: Astrocytic tumors occur in the retina or in the optic disc usually as a part of tuberous sclerosis complex or other phacomatosis and their isolated occurrence is rare. The authors present two adult patients in whom the diagnosis of intraocular astrocytoma was established but no signs of phacomatosis were revealed.

Progressive enlargement of acquired retinal astrocytoma in 2 cases.Ophthalmology. 2004 Feb;111(2):363-8

PURPOSE: To document 2 cases of progressively enlarging retinal astrocytoma that caused exudative retinal detachment, vitreous hemorrhage, and tumor seeding, simulating retinoblastoma in 1 case and choroidal melanoma in the other. DESIGN: Interventional case reports. PARTICIPANTS: Two patients. METHODS: Two patients with visual loss from an atypical, enlarging amelanotic retinal mass were evaluated. The first patient, a 14 year-old otherwise healthy girl, had a noncalcified gelatinous retinal mass with prepapillary involvement and surrounding retinal traction, as well as overlying vitreous hemorrhage and vitreous seeds. Over 37 months, the tumor grew in basal dimension from 4 mm to 10 mm. Retinal astrocytoma was suspected, but the presence of tumor enlargement and vitreous seeding raised concern for possible retinoblastoma. Fine-needle aspiration biopsy was performed. The second patient, a 33-year-old woman, had an amelanotic mass develop in the macula of her amblyopic left eye. Over 2 years, the mass enlarged to 9.5 mm in basal dimension and 6.3 mm in thickness, and total serous retinal detachment developed. Choroidal melanoma with retinal invasion was clinically suspected. Enucleation was performed. RESULTS: In patient 1, cytologic examination revealed bland spindle cells with fibrillar cytoplasm consistent with an astrocytic tumor of the retina. Enucleation was subsequently performed because of continued tumor growth, progressive retinal detachment, and visual loss. Histopathologic examination confirmed astrocytoma of the retina and optic disc. In patient 2, enucleation revealed astrocytoma of the retina and optic disc with total retinal detachment. There was no evidence of systemic tuberous sclerosis in either patient. CONCLUSIONS: Retinal astrocytoma can show progressive enlargement, retinal detachment, and vitreous seeding, findings that can mislead the clinician toward the diagnosis of retinoblastoma or choroidal melanoma.

Invasive giant cell astrocytoma of the retina in a patient with tuberous sclerosis.Ophthalmology. 1999 Mar;106(3):639-42.

OBJECTIVE: To report an unusual case of giant cell astrocytoma of the retina. DESIGN: Case report. INTERVENTION: A 10-month-old girl with tuberous sclerosis was found to have bilateral astrocytic hamartomas, the right eye being prominently involved by elevated and pedunculated lesions. At 7 years of age, she had posterior subcapsular cataract, retinal detachment, and subretinal exudation develop in the right eye. At 12 years of age, her blind, painful right eye had to be enucleated because of neovascular glaucoma and a spontaneous scleral perforation. RESULTS: Histopathologic examination showed that the entire vitreous cavity was filled with a mixture of tumor, granulation tissue, and necrotic debris. Part of the tumor was composed of spindle-shaped glial cells. The remainder was composed of large gemistocytic cells that contained large atypical nuclei and copious amounts of cytoplasm, which was intensely eosinophilic in some areas. The tumor contained foci of necrosis and rare mitotic figures. It had infiltrated the parenchyma of the retrolaminar nerve and extended to the surgical margin. Areas of unequivocal choroidal invasion were also identified. The tumor cells were intensely immunoreactive for neuron-specific enolase and S-100 protein. In contrast, glial fibrillary acidic protein was only minimally positive. CONCLUSIONS: The histologic and immunohistochemical features of this retinal tumor resemble those of subependymal giant cell astrocytoma, a characteristic lesion in tuberous sclerosis. Although this unusual giant cell astrocytoma of the retina had atypical histopathologic features and local aggressive behavior, the systemic prognosis was excellent.

Solitary retinal astrocytoma with "acoustic shadowing".Klin Monatsbl Augenheilkd. 1995 Mar;206(3):188-9.

PATIENT: A polycyclic lesion slightly prominent and yellowish in colour with a mulberry-like surface was incidentally detected in the left eye of a 69-year-old white female. It was located in the nasal horizontal sector of the fundus in a distance of about 5.5 mm from the optic disk. It was surrounded by a bright halo. Retinal arterioles penetrated the lesion superficially and more deeply. The vitreous body was unremarkable. Visual acuity was 0.8 to 1.0 p with a hyperopia of 3.5 dpt. Intraocular pressure was 116 mm Hg. In both eyes partially confluent drusen of the retinal pigment epithelium were present without a serous detachment of the retina. Ultrasound examination revealed an ovally shaped, 1.3-mm prominent solid tumor consisting of highly reflective granular internal echoes with acoustic shadowing behind. X-ray of the skull showed punctate calcifications in the medial orbit. The brain was unremarkable (incl. MRI). In fluorescein angiography the whole tumor was diffusely stained without signs of leakage or subretinal neovascularization. On red-free wide-angle photographs of the retinal nerve fiber layer no localized defects were detected. Neurological and dermatological examination were unremarkable without any hints for tuberous sclerosis or neurofibromatosis. All these findings led to the diagnosis of a solitary retinal astrocytoma. With no therapy performed, the functional and morphological status of the eye remained unchanged one year later. CONCLUSION: A retinal astrocytoma is a hamartoma that usually shows no tendency to grow and there is no need for treatment. It can rarely be the reason of a serous retinal detachment or vitreous hemorrhage. Especially solitary astrocytomas have sometimes been reported to show a tendency towards severe intraocular damage. Additional calcifications showing acoustic shadowing in ultrasound examination are important for the differential diagnosis of retinoblastoma.

Astrocytic tumors of the retina. Differentiation of sporadic tumors from phakomatosis-associated tumors. Arch Pathol Lab Med. 1984 Feb;108(2):160-3.

A single, 1.3-cm, intraocular tumor, composed of interlacing, spindle-shaped astrocytes, involved the retina and optic disc of a 13-month-old girl. Neither the patient nor her family had stigmas of a phakomatosis. We found 42 previous cases of histologically documented astrocytic tumors of the retina. Twenty-four patients (57%) had tuberous sclerosis, six patients (14%) had neurofibromatosis, and 12 patients (29%) were otherwise normal. Patients with tuberous sclerosis usually had multiple, peripheral, retinal tumors containing giant "astrocytes." Patients with neurofibromatosis and otherwise normal patients more frequently had disc-based tumors. However, three patients (50%) with neurofibromatosis had multiple tumors, whereas multiplicity occurred in only one of the patients in the sporadic group. We conclude that if the tumor is single, is disc based, and lacks giant cells, the patient is not at great risk of tuberous sclerosis.

Giant cell astrocytoma of the retina. A tumor of possible Mueller cell origin.Ophthalmology. 1983 Dec;90(12):1565-76.

A 5-month-old boy presented with a right retrolental mass and glaucoma. Both B-scan ultrasonography and CAT scanning failed to disclose any evidence of intralesional calcification, the absence of which is unusual for retinoblastoma. An aqueous lactic dehydrogenase (LDH) determination disclosed elevated levels of this enzyme, which were six times that detected in the blood. Pathologic examination of the enucleated globe revealed rubeotic glaucoma, and a massively necrotic retinal tumor that had filled the vitreous cavity. A small focus of viable tumor in the posterior pole of the lesion displayed a population of rounded astrocytic giant cells, with abundant eosinophilic cytoplasm and eccentrically placed vesicular nuclei possessing prominent nucleoli. The cytoplasm of these tumors cells contained a moderate number of PTAH-positive fibrils, and these were identified positively as glial in nature by immunoperoxidase reaction for the presence of glial fibrillary acidic protein (GFAP). Mueller cells in a portion of surviving retina also stained positively for GFAP. Electron microscopic studies performed on a rim of surviving tumor cells abutting the subretinal space demonstrated features consistent with a Mueller cell origin, namely, abundant profiles of smooth and rough-surfaced endoplasmic reticulum and elaborate villous processes, the latter being typically produced by Mueller cells as they project beyond the external limiting membrane of the retina.

Retinal phacomas. Apropos of 10 cases of astrocytoma.J Fr Ophtalmol. 1983;6(3):275-90.

Clinical and angiographic findings in 10 cases of retinal astrocytomas seen at the Clinique Ophtalmologique de Creteil are described. Classical features concerning onset of the disease, clinical aspects, and evolution of these tumors are confirmed. Some atypical findings are reported, including the marked proportion of isolated astrocytomas unrelated to phacomatosis, and the high frequency of complications of these lesions, usually considered to be stable.

Localization of glial fibrillary acidic protein in retinal astrocytoma: an immunohistochemical study.Curr Eye Res. 1982-1983;2(8):523-7.

Glial fibrillary acidic protein (GFA) is found in glial filaments, and is specific for reactive or neoplastic astrocytes and ependyma. Using a commercially available antibody to GFA protein, a retinal neoplasm from a patient with neurofibromatosis is intensely stained with the brown immunoperoxidase reaction product. Rather than the less specific phosphotungstic acid hematoxylin (PTAH) or Holzer's stain, antibody to GFAP protein is a relatively rapid, simple, and reliable immunohistochemical technique for formalin-fixed and paraffin-embedded tissue.

Retinal astrocytoma.Am J Ophthalmol. 1979 Jul;88(1):32-6.

A 41-year-old man had a 6 x 6 x 5-disk diameter amelanotic tumor in the posterior fundus. The clinical and fluorescein angiographic appearance suggested a benign retinal vascular tumor, although amelanotic choroidal melanoma and retinoblastoma were diagnostic possibilities. An incisional 48-hour radioactive phosphorus (32P) uptake test was performed and the result showed an increased uptake over the tumor mass of 100% as compared to the control quadrants. The globe was enucleated and the pathologic diagnosis was isolated astrocytic glioma of the retina with minimal, if any, malignant potential. The highly developed vascular system of the tumor probably contributed to the false-positive test result.


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