Patient Details

Age: 6 months

Sex: Male

Date of initial presentation: 29/1/1975

Clinical Diagnosis

? Retinoblastoma.
Eye bisected equatorially in theatre.
? Normal front position fixed in glutaraldehyde for electron microscopy.
Posterior position (photographed by [surgeon] 10/1/1975) fixed in formal saline for routine histology.
Portion of optic nerve removed for sectioning separately in paraffin. Rest of specimen decalcified in formaldehyde - formic acid. (Carleton & Leach p. 211 [possibly Schafer's Essentials Of Histology by H. M. Carleton & E. H. Leach]) (Culling p. 48 [possibly Handbook of Histopathological and Histochemical Techniques by C. F. A. Culling])
*Send L.V.N. section to [consultant pathologist] when stained.

21/2/1975 - Letter from [surgeon] to [consultant pathologist]

I would be very grateful for your further opinion on the specimens which I enclose. I would like your confirmation, not only that this is a retinoblastoma but that the optic nerve is not involved. The two slides included preliminary preparation of a large tumour mass and a preparation of the proximal end of the optic nerve stump attached to the enucleated globe. We are preparing celloidin sections and will send you further sections through the optic nerve in due course. 

The specimen comes from a most interesting African baby who shows other somatic abnormalities and in whom the diagnosis of retinoblastoma was suspected when he presented last month with left convergent squint and a white pupil reflex. Clinically and in the cut specimen it appeared that the optic nerve was involved in the tumour mass. This boy incidentally has a glucose-6-P-D deficiency but of greater interest is the fact that he has a deletion in the long arm of chromosome 13 and appears to be an example of the "Dq" deletion syndrome of Orbeli. My impression is that the tumour is well differentiated and it seems from the literature that the retinoblastomas associated with this condition have been more benign in behaviour that usual. 

27/2/1975 - Report from [consultant pathologist]

Many thanks for referring this most interesting case. I should be very glad to see a section of the whole eye when it is available.

Sections from the calotte show a well-differentiated retinoblastoma arising from the retina and growing endophytically. The lesion is giving rise to well-formed rosettes, and at the level of these preliminary sections, there is no evidence of a choroidal invasion. The tumour seems to be close to the macula but a few seedlings are present on the inner surface of the peripheral retina on one side, close to the ora serrata. The other section is of the optic nerve: it is surrounded by haemorrhages and by normal meningeal coverings. At this level of study there is no evidence of invasion of the optic nerve nor of surrounding structures by tumour. 

No evidence of a developmental defect can be found in these sections, but it may be of value to examine the optic disc and papilla carefully, and possibly to check the presence of nerve fibres in the inner layers of the retina and the optic nerve, and possibly myelin in the latter.

20/5/1975 - Letter from [surgeon] to [consultant pathologist]

Would you please be kind enough to write pathology reports on the enclosed slides.

[Unrelated cases]

These are more sections of the eye you reported on 27th February, 1975. The anterior part of the eye was used for electron microscopy. I have done a Loyez stain for normal melanin and a modified Bielchowsky (Glees and Marsland) for nerve fibres without much success as you can see. 

2/6/1975 - Further report from [consultant pathologist]

Further examination of the tumour, the remaining sensory retina, and special stains of the zone surrounding and including the optic disc and never-head, do not seem to demonstrate the presence of nerve fibres in the sensory retina nor in the optic nerve, and also fail to show the presence of myelin in the portion of optic never available for study. This is subject to interpretation, as histochemical techniques often show a degree of variation. It is difficult to say if the absence of never fibres in the sensory retina, and of myelin in the optic nerve, represents a developmental element, or whether they may be a direct result of the tumour, which probably has been present for some time and is obviously involving the papilla rather heavily.