Multiple Endocrine Neoplasia Type 1 Presenting with an Invasive Giant Prolactinoma / 대한내과학회지
Korean Journal of Medicine
; : 300-305, 2016.
Article
de Ko
| WPRIM
| ID: wpr-20328
Bibliothèque responsable:
WPRO
ABSTRACT
Pituitary tumors occur in 15-50% of patients with multiple endocrine neoplasia of type 1 (MEN1). To the best of our knowledge, no MEN1 case in which the initial lesion was an invasive giant prolactinoma has been reported from Korea. We describe a patient in whom a skull-base tumor involved the sellar and parasellar spaces. A 49 year-old female presented with headache and diplopia. The tumor was ultimately identified as a giant prolactinoma; the serum prolactin concentration increased from 155.6 ng/mL to 3,234.3 ng/mL after cranial irradiation. She was evaluated in terms of incidental hypercalcemia and was found to have parathyroid hyperplasia. Genetic analysis revealed a missense mutation in the MEN1 gene (c.643G>A, p.Val215Met). Two years of treatment with a dopamine agonist reduced, but did not normalize, the serum prolactin concentration. We highlight the aggressive behavior of the giant skull-base tumor, and the diagnostic delay caused by a high-dose hook effect of the MEN1-related prolactinoma.
Mots clés
Texte intégral:
1
Indice:
WPRIM
Sujet Principal:
Tumeurs de l'hypophyse
/
Prolactine
/
Néoplasie endocrinienne multiple
/
Prolactinome
/
Irradiation crânienne
/
Néoplasie endocrinienne multiple de type 1
/
Agonistes de la dopamine
/
Mutation faux-sens
/
Diplopie
/
Céphalée
Limites du sujet:
Female
/
Humans
Pays comme sujet:
Asia
langue:
Ko
Texte intégral:
Korean Journal of Medicine
Année:
2016
Type:
Article