ID 112137
Author
Nagata, Yuichi Toranomon Hospital
Inoshita, Naoko Toranomon Hospital
Fukuhara, Noriaki Toranomon Hospital
Yamaguchi-Okada, Mitsuo Toranomon Hospital
Nishioka, Hiroshi Toranomon Hospital
Yamada, Shozo Toranomon Hospital
Keywords
Growth hormone-producing pituitary adenoma
Young
Gigantism
Clinical feature
Pathology
Acromegaly
Content Type
Journal Article
Description
Purpose
Growth hormone (GH)-producing pituitary adenomas (PAs) in childhood or young adulthood are rare, and the details surrounding these tumors remain enigmatic. We present the clinical, pathological and genetic features of this disease.
Methods
We identified 25 patients aged 20 years or younger with GH-producing PAs who underwent surgery between 2003 and 2016 at Toranomon Hospital in Tokyo. We retrospectively reviewed the clinical data, treatment outcomes and pathological features of these patients to shed light on childhood acromegaly.
Results
The cohort comprised 14 male and 11 female patients whose average age at the time of surgery was 17.3 years. Germline AIP mutations were present in 5 of 13 patients examined, and Carney complex was identified in 2 of 25 patients. The mean maximum tumor diameter was 26.7 mm, and total resection assessed during surgery was achieved in 17 patients. Based on their respective pathological findings, patients were divided into the following 4 groups: sparsely granulated adenomas (5), densely granulated (DG) adenomas (6), plurihormonal adenomas (9), and silent subtype 3 (SS3) adenomas (5). During the mean follow-up period of 50.3 months, complete endocrinological remission was achieved in 14 of 25 patients (56%) by surgery alone and in 19 patients (76%) after postoperative adjuvant therapy.
Conclusions
GH-producing PAs in young patients are intriguing and difficult to treat due to their distinct tumor characteristics, including a lower incidence of the DG subtype and a higher incidence of SS3 adenomas and genetic abnormalities. Therefore, multi-modal therapies are essential to achieve optimal clinical outcomes.
Journal Title
Pituitary
ISSN
1386341X
15737403
NCID
AA11488255
AA12528741
Publisher
Springer
Volume
21
Issue
1
Start Page
1
End Page
9
Published Date
2017-08-28
Rights
This is a post-peer-review, pre-copyedit version of an article published in Pituitary.
The final authenticated version is available online at: DOI https://doi.org/10.1007/s11102-017-0836-4
EDB ID
DOI (Published Version)
URL ( Publisher's Version )
FullText File
language
eng
TextVersion
Author
departments
Oral Sciences