Endocrine Abstracts

Introduction: Pituitary adenomas are a rare condition in children and young people. The prolactinoma represents the most frequent of them. We report a case of an atypically evolution of aggressive macroprolactinoma in young patient.

Case: It’s about a boy of 14 years old with previous history of headache from 5 years without visual disturbance. He was admitted for intracranial hypertension syndrome. In clinical examination no dysmorphic syndrome, a growth retardation and a bilateral gynecomastia. MRI showed a macroadenoma with lateral extension to the cavernous sinuses with envelopment of carotid arteries. The hormonal test showed a hyperprolactinemia of 470 ng/ml with a secondary gonadal, corticotropic and thyrotropic deficiency. Partial transsphenoidal adenomectomy was performed and the histological assessment confirmed a macroprolactinoma with upper Ki67 at 7%. Cabergoline was started at 1 mg/week to 2 mg/week. The evolution done by the progressive normalization of blood prolactin and reduction of the size of the residual tumor.

Discussion: Pituitary adenomas in young people are frequently hormonally active. These patients typically presented with endocrine symptoms related to their adenoma type, in our case he presented gynecomastia and tumoral syndrome. Prolactinoma is a more common one.

The radiological caracteristics and the Hight Ki67 proliferation index gave an idea of aggressive tumor. Taking in account the young age it can be part of AIP mutation or another type of genetic predisposition syndrome. These two conditions tend to predict recurrence and resistance to conventional therapy. The good evolution by dopaminergic agonist was atypical. Long-term follow-up will make it possible to report on the recurrence or otherwise of the process.

Conclusion: The management of pituitary adenoma in young patients must take in account the progress on histopathology and molecular fields. The specific medical treatment of prolactinoma may be try as part of complex management.

Reference: 1 Öngürü Ö, Düz B, Şimşek H, Günal A, Gönül E. Pituitary macroadenomas (>3 cm) in young adulthood: Pathologic and proliferative characteristics. Neurol Neurochir Pol. 2015;49(4):212-6.

2 Cuny T, Chanson P. Adénomes hypophysaires agressifs et résistants au traitement Aggressive and resistant-to-treatment pituitary tumors. Ann Endocrinol (Paris). 2013 Oct;74 Suppl 1:S3-12.