PMC6112072_01
(a) MRI images of Case 1 at diagnosis (1), initial response to dopamine agonist (2), and recurrence of dopamine agonist resistant tumour (3); (b) MRI images of Case2 at diagnosis and showing tumour shrinkage to subcentimeter level after 5 years of dopamine agonist therapy.
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His imaging revealed a giant pituitary tumour (59 x 45 x 42 mm) with extrasellar extension (Figure 1(a)) with initial prolactin of 91,800 mug/L confirming the diagnosis of giant prolactinoma. A few years later, he developed recurrence of the tumour, which was resistant to cabergoline therapy (Figure 1(a)), and underwent transcranial excision of the tumour in 2013. She was diagnosed to have a giant prolactinoma (40 x 45 x 30 mm, Figure 1(b)) with hyperprolactinaemia (serum prolactin 8930 ng/dL). She responded well to medical therapy with cabergoline (3.5 mg/week) with normalization of prolactin over 1 year and tumour shrinkage over 5 years (Figure 1(b)).
Family trees of the two MEN-1 cases. Cascade genetic testing needs to be followed in both families. Carriers need referral to specialist endocrinology clinic for clinical follow-up. Children of carriers need genetic testing.
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On family screening, the proband's father and brother were positive for the same mutation, and father was found to have hyperparathyroidism (Figure 2). Both the families are in the process of undergoing cascade genetic screening (Figure 2).