An
18 month-old baby was referred because of the following appearance.
a. Name four possible diagnosis.
b. Examination of the eye under
anaesthesia showed an unifocal lesion as
above.
Name five typical
histological features which may be seen in this
condition.
The picture shows a white
dome-shaped lesion typical of
Flexner-Winsteiner rosettes they
have not central lumen instead
the centre of the rosettes are filed
by neurofibrillary tangles.
Fleurettes are also seen which are
flower-like grouping of tumor
cells which look like photoreceptors
and are a marker of
well-differentiated retinoblastoma. Calcifiction
is common as
is necrosis because the tumour rapidly outgrows its
blood supply.
c. There was no family history of the
same condition. Examination of
the left eye
was normal. What is the risk of his parents having a similarly
affected child?
d. Enucleation was carried out
in this patient. Name 2 features in the
enucleated specimen
which are assocaited with poor prognosis.
e. What is the risk of second tumour
in this patient compared with one with
the same lesion
in both eye?.