Akademik Veri Yönetim Sistemi
60th Annual Meeting of the European Society for Paediatric Endocrinology, Rome, İtalya, 15 - 17 Eylül 2022, cilt.95, sa.2, ss.374
Giant prolactinomas are rare tumours, with a prevalence of
0.5–4.4% among all pituitary tumours. In the literature, relatively
few series describing their management and outcomes are available; moreover, only individual case reports of giant prolactinomas
in children have been documented.
We report a 10-year-old boy presented with painless progressive vision loss, insidious in onset, and bitemporal headache of six
mounths duration. On physical examination; height: 134.2cm
(3-10p), weight: 34 kg (25-50p), BMI: 20kg/m2 (50-75p), prepubertal (Tanner stage 1) other system examinations were found to
be normal. Magnetic resonance imaging showed supra-parasellar
area, cavernous sinus, both internal carotid arteries, right temporal
fossa and optic chiasm a mass with an extending cystic component
was detected. The tumour had a maximum diameter of 42x40x46
mm. The laboratory investigations detected elevated prolactin levels (8730ng/ml), TSH: 2.83µıu/mL, fT4: 0.83 ng/dl, FSH: 0.41 ıu/l,
LH: <0.2 ıu/l, ACTH: 17.9 pg/ml, Cortisol: 14.2 µg/dl, IGF-1:
141ng/ml(-2;-1SD), IGFBP-3: 4440ng/ml(0;-1SD). The patients
were firstly partial reduction in size of the tumours and started on
dopamine agonist therapy Histopathological and immunoisthochemical exams described prolactin-secreting adenoma features.
Proliferation markers, Ki-67 expression:15 % (≥ 3% high) and
mitotic count:8 (The number of mitoses observed is >2 per 10
high-power fields).
Nowadays, after nine months from diagnosis, dopamine agonist treatment leading to reductions in tumor sizeş (12x5 mm) and
prolactin levels (13 ng/ml) in the normal range. After resection and
dopamin agonist therapy visual function stabilized.
Conclusion: Giant cystic prolactinomas in childhood are
extremely rare. Having very high prolactin levels, diffuse invasion
of the mass and High KI-67 index that is expected to have a bad
prognosis. Short-term therapy with cabergoline together with a
pituitary surgery in our patient was the key for the successful management, achieving biochemical and clinical remission in our
patients.
Keywords: Cystic Giant Prolactinoma, Hyperprolactinemia,
KI-67 Index, Dopamine Agonist Treatment, Endoscopic
Transsphenoidal Surgery