ACTA O P H T H A L M O L O G I C A 69 (1991) 113-116

CASE REPORT

Solitary retinal astrocytoma

Ashok Sharma, Jagat Ram and Arnod Gupta

Department of Ophthalmology (Head: A. Gupta), Postgraduate Institute of Medical Education and Research, Chandigarh, India

Abstract. A two-year-old female child presented with a left retinal mass in front of the optic disc and total serous retinal detachment. Enucleation was performed for sus- pected retinoblastoma. Histopathologically the tumour was composed of astrocytic giant cells with a few spindle- shaped astrocytes. Presence of multiple necrotic foci in the tumour resembling micro-abscesses and extension of tumour cells beyond lamina cribrosa were additional un- usual features.

Key words: retinal astrocytoma - serous retinal detach- ment - giant cell astrocytes.

Astrocytic tumours of the retina and optic disc are unusual aordano et al. 1974). Retinal astrocytomas most commonly occur in association with tuberous sclerosis (Wolter & Mertus 1969; Harley & Grover 1970; Barsky & Wolter 1971). These tumours are extremely slow-growing and are generally re- garded as clinically silent (Atkinson et al. 1973). Rarely, a solitary retinal astrocytoma unassociated with phakomatosis may follow an atypical course, resulting in severe intraocular damage due to tu- mour necrosis (Jordan0 et al. 1974). Clinico-patho- logical features of this atypical form of retinal as- trocytoma are not widely described. We report a case of solitary retinal astrocytoma with extensive tumour necrosis to illustrate clinical and histopa- thological features.

Case Report

A two-year-old female presented with squinting of the left eye of one year’s duration. The child’s growth and development had been normal. She

had no systemic manifestation of phakomatosis. Visual acuity could not be assessed on Snellen’s chart. Anterior segment examination of the left eye revealed a transparent cornea (11.5 X 10 mm), normal depth of anterior chamber and mid-di- lated non-reacting pupil. The iris was normal. The intraocular pressure was 20 mmHg in the left eye and 14 mmHg in the right eye. Fundus examin- ation revealed a whitish mass in front of the optic disc with total serous retinal detachment. The right eye was normal. X-ray of the left orbit showed no intraocular calcification. A clinical diagnosis of retinoblastoma in the left eye was made and the eye was enucleated under general anaesthesia. After a follow-up of 2V2 years no recurrence of tu- mour was seen.

Histopathological findings The enucleated eye was fixed in formaldehyde and glutaraldehyde (2% each). The eye measured 23.5 X 22.5 mm and the cornea 11.3 X 10.0 mm. On opening of the globe horizontally, a large 11.5 X 8 X 4 mm white f m tumour attached to a totally de- tached retina was observed (Fig. 1). The subretinal space contained extensive proteinaceous exudates. The angle of the anterior chamber was shallow and the iris and lens appeared to have been pushed for- ward.

The tumour was composed of many giant, gan- glion-like, gemistocytic astrocytes with a few spindle-shaped glial cells (Fig. 2a). These gemisto- cytic astrocytes had abundant eosinophilic cyto- plasm and large eccentric nuclei with prominent nucleoli (Fig. 2b). Throughout the tumour, ne-

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Fig. 1. Retinal tumour in front of optic disc (arrows) with involvement

beyond lamina cribrosa (arrow heads) (HEX 27).

F i q . 2a and 26. Fig. 2a. Tumour composed of spindle-shaped cells with scattered gemistocytic astrocytes (HE X 140).

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F i p . ?a and ?b. Fig. 3a. Necrotic focus infiltrated with neutrophils (HE X 140). Fig. 3b. Focal calcification (HE X 140).

crotic foci, infiltrated with neutrophils, resembled micro-abscesses (Fig. 3a). Special stains for micro- organisms were negative. Focal calcification was noted in the juxtapapillary area (Fig. 3b). Scattered tumour cells in the optic nerve beyond the poste- rior lamina cribrosa were observed. The histopa- thological findings were characteristic of a retinal astrocytoma.

Discussion

Astrocytic tumours were first described in associ- ation with tuberous sclerosis by Van der Hoeve in 1921. Retinal astrocytomas are so rare that these tumours escape clinical suspicion. In a series of 500 children with suspected retinoblastoma not a single case of astrocytoma was found (Howard & Ellsworth 1965). Classically, retinal astrocytomas present either as flat semi-translucent or nodular white tumours (Arnold et al. 1985). On long-term follow-up these tumours have been found to grow very slowly, and flat lesions have been observed to acquire a nodular mulberry appearance (Atkinson et al. 1973). Rarely, a solitary retinal astrocytoma

may progress and result in severe intraocular dam- age due to massive tumour necrosis (Jordan0 et al. 1974). Ten cases of histopathologically do- cumented retinal or optic disc astrocytomas of this unusual form have been reported in the literature (Boles et al. 1958; Foos et al. 1965; Cleasby et al. 1967; Wolter & Mertus 1969; Jordan0 et al. 1974; Reeser et al. 1978; Ramsay et al. 1979; Coppeto et al. 1982; Jakobiec et al. 1983; Arnold et al. 1985). Our case represents this atypical form of retinal as- trocytoma.

Solitary retinal astrocytomas unassociated with phakomatosis in children may simulate retinoblas- toma or Coats' disease (Jakobiec et al. 1983). Massive subretinal exudation around the retinal mass has been described as characteristic of a reti- nal astrocytoma (Cleasby et al. 1967). Microscopic calcification, rarely detectable on radiological in- vestigations, is a common feature of retinal astro- cytoma (Arnold et al. 1985). On computerised axial tomography the anterior border of a subretinal ex- udation is less distinct than in Coats' disease (Reeser et al. 1978). In spite of the various clinical features described, diagnosis of retinal astrocy- toma is extremely difficult. Of ten previously re-

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ported cases, enucleation in four was performed for a painful blind eye due to neovascular glau- coma and in the remaining six for suspicion of in- traocular neoplasms. Central retinal vein occlu- sion in two cases and neovascular glaucoma in five of ten cases have been previously reported (Arnold et al. 1985). Neither of these features were present in our case. Our patient had total exudative retinal detachment surrounding the retinal mass and the clinical diagnosis was retinoblastoma.

Astrocytomas of the retina are typically com- posed of spindle-shaped glial cells (Reeser et al. 1978; Ramsay et al. 1979; Coppeto et al. 1982). Rarely, these tumours may contain mainly polygo- nal, gemistocytic astrocytes, also described as giant retinal astrocytes (Jordano et al. 1974; Font 8c Ferry 1982). The tumour in our case was mainly com- posed of giant polygonal cells. Jakobiec et al. (1983) have postulated that Muller cells of the retina were a possible origin of these giant cell astrocytes. Microscopic calcification as seen in our patient is usual in retinal astrocytomas. Massive tumour ne- crosis resembling micro-abscesses as seen in our patient is unusual (Arnold et al. 1985). Extension of tumour beyond the cribriform plate is most un- usual and has been reported in only one case (Foos et al. 1965). Although there was extension of tu- mour cells beyond lamina cribrosa in our patient, no recurrence of tumour was seen after a follow-up of 2'12 years.

Retinal astrocytoma, if diagnosed accurately, does not warrant enucleation. Enucleation should be performed only when ocular complications de- velop or suspicion of retinoblastoma cannot be eliminated.

Acknowledgment

We are grateful to the staff of the Armed Forces Institute of Pathology, Washington DC for histopathological examination of the left eye.

References

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90: 1565-1576.

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Received on March 21st, 1990.

Author's address:

Amod Gupta, MD, Department of Ophthalmology, Postgraduate Institute of Medical, Education and Research, 160 012 Chandigarh, India.

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