Multiple Endocrine Neoplasia Type 1 Presenting with an Invasive Giant Prolactinoma.
10.3904/kjm.2016.91.3.300
- Author:
Jinhoon CHA
1
;
Jin Seo KIM
;
Jung Suk HAN
;
Yeon Won PARK
;
Min Joo KIM
;
Yun Hyi KU
;
Hong Il KIM
Author Information
1. Department of Internal Medicine, Korea Cancer Center Hospital, Korea Institute of Radiological and Medical Sciences, Seoul, Korea. ngugi@kirams.re.kr
- Publication Type:Case Report
- Keywords:
Multiple endocrine neoplasia type 1;
Giant prolactinoma;
High-dose hook effect
- MeSH:
Cranial Irradiation;
Diplopia;
Dopamine Agonists;
Female;
Headache;
Humans;
Hypercalcemia;
Hyperplasia;
Korea;
Multiple Endocrine Neoplasia Type 1*;
Multiple Endocrine Neoplasia*;
Mutation, Missense;
Pituitary Neoplasms;
Prolactin;
Prolactinoma*
- From:Korean Journal of Medicine
2016;91(3):300-305
- CountryRepublic of Korea
- Language:Korean
-
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.
