Trilateral Retinoblastoma

E. TasËdemirogÆlu

EyuÈp Social Security Hospital, Neurosurgery Service, IÇstanbul, Turkey

Case Report

A two year old girl was diagnosed with a bilateral retinoblastomas

on March 1986. Her left eye was enucleated and the right eye was

treated by radiation therapy, with 4000 rads (Figure 1). She was in

remission until April 1991. She started complaining with headache,

nausea, vomiting and lethargy, ®ve years after the diagnosis and

treatment of bilateral retinoblastomas.

Her neurological examination showed a dilated right pupil, right

papilloedema and nystagmus on right lateral gaze. Her MR imaging

of the brain with gadolinium-DTPA enhancement (Figures 2A±B)

showed a 1.25 cm by 2.5 cm enhancing pineal mass with tectum and

upper midbrain compression and mild obstructive hydrocephalus.

CSF cytopathology was positive for malignant cells. Chemotherapy

was instituted on June 1991. Regardless of chemotherapy her disease

progressed and she died on January 1992, before the cranial and

spinal axis radiation therapy started.

Discussion

The term trilateral retinoblastoma has been used to

refer to the presence of bilateral retinoblastomas with

Acta Neurochirurgica> Springer-Verlag 1999Printed in Austria

Acta Neurochir (Wien) (1999) 141: 893±894

Fig. 1. CAT scan of the cranium showed an intra-ocular hyperdense

lesion ®lling the ocular bulb

a

b

Fig. 2. T-1 weighted axial (a), and sagittal (b) MR images following

G-DTPA enhancement showed a 1.25 cm by 2.5 cm pineal mass

with tectum and upper midbrain compression along with mild

hydrocephalus

an associated primary intracranial neoplasm of primi-

tive type that is usually located in the pineal, supra-

sellar, parasellar region [3] or posterior fossa [1]. A

second primary tumour occurs in 15% to 20% of pa-

tients with bilateral retinoblastomas, but most are soft

tissue tumours or osteogenic sarcomas, and 71% occur

within the radiotherapy ®elds [4]. However this co-

existent intracranial neoplasm if found in about 3%

patients with bilateral retinoblastoma [5] and this in-

tracranial tumour is considered a lesion distinct from

the intra-ocular neoplasm, not a metastasis [4]. Com-

pared with spontaneous retinoblastomas, the ocular

tumours in patients with trilateral retinoblastoma

present at an earlier age, such as less than a year old

[2]. A positive family history of retinoblastoma is

present in about 65% of cases [2]. Although, there is

usually a latent period of a few years between the di-

agnosis of an intra-ocular tumour and a coexistent

intracranial lesion [2], Finelli et al. [1] showed that

intracranial tumour in trilateral retinoblastoma may

present at any time, in any midline location. More

than 50 cases have been reported in the literature, so

far [1]. Most of the patients die soon after the diagnosis

of trilateral retinoblastoma, with extensive central

nervous system dissemination. Because of the lethality

of the third (intracranial) lesion in trilateral retino-

blastoma, early detection of preclinical lesions is very

important. Patients with hereditary bilateral retino-

blastoma presenting for orbital MR imaging should

also have scans including pineal, parasellar, supra-

sellar and fourth ventricular regions. Monitoring with

brain MR examinations at 6 to 12 months intervals

may be of bene®t to this small group of patients with a

high propensity for every aggressive intracranial ma-

lignant neoplasm.

References

1. Finelli DA, Shurin SB, Bardenstein DS (1995) Trilateral retino-

blastoma two variations. AJNR 16: 166±170

2. Holladay DA, Holladay A, Montebello JF, Redmond KP (1991)

Clinical presentation, treatment, and outcome of trilateral reti-

noblastoma. Cancer 67: 710±715

3. Jakobiec FA, Tso MO, Zimmerman LE, Danis P (1977) Retino-

blastoma and intracranial malignancy. Cancer 39: 2048±2058

4. Johnson DL, Chandra R, Fisher WS, Hammock MK, McKeown

CA (1985) Trilateral retinoblastoma: ocular and pineal retino-

blastomas. J Neurosurg 63: 367±370

5. Pesin SR, Shields JA (1989) Seven cases of trilateral retino-

blastoma. Am J Ophthalmol 107: 121±126

Correspondence: Erol TasËdemirogÆlu, M.D., Incirili Caddesi,

Deniz Apt. no � 74=7, BakõrkoÈy, Istanbul, 34750 Turkey.

894 E. TasËdemirogÆlu: Trilateral Retinoblastoma