History: A 40-year-old man presented with severe headache, nausea and acute
blindness. A CT-scan of the skull revealed a huge tumor along the basis of
the skull. The patient was referred to our clinic for diagnostic and thera
peutic evaluation.
Investigations: MRT showed a large right-sided paramedian tumour displacing
the brain stem with signs of increased intracranial pressure. Routine labo
ratory tests were normal except a normochromic anaemia. Endocrine tests dem
onstrated partial hypopituitarism with alteration of the somatotropic, gona
dotropic and corticotropic axis and moderate hyperprolactinaemia.
Treatment and course: On the day of admission a transnasal biopsy was taken
. The preliminary histopathological diagnosis was a low differentiated carc
inoma. Because of this diagnosis and because of the infiltrative tumour gro
wth an operation was not performed but emergency irradiation was begun and
dopamine agonist therapy was started because of hyperprolactinaemia. Severa
l days later the final microscopic diagnosis of the transnasal biopsy speci
men was reported to be an invasive prolactinoma. Under dopamine agonist the
rapy prolactin levels rose to a maximum of 6460 ng/ml to decline thereafter
to normal values, and the visual disturbances recovered. After 5 weeks of
therapy considerable shrinkage of the tumor was demonstrated by MRT.
Conclusion: The differential diagnosis of acute visual deterioration caused
by a large tumour along the basis of the skull includes an invasive prolac
tinoma. The diagnosis is made by demonstrating grossly elevated prolactin l
evels. To avoid falsely low prolactin measurements, caused by a hook-effect
in the prolactin assay, serum dilution is mandatory in the diagnostic work
up. In the case of a prolactinoma medical treatment with dopamine receptor
agonists is the therapy of choice because it causes rapid tumour shrinkage
and symptomatic improvement in most patients, so that irradition of the tum
our is not indicated. As dopamine agonist therapy is rapidly effective and
well tolerated, it should be started even in case of doubt to lose no time
until final diagnosis.