Growth Hormone-Producing Pituitary Adenomas in Childhood or Young Adulthood

Presentation Number: SUN 447
Date of Presentation: April 2nd, 2017

Yuichi Nagata*1, Noriaki Fukuhara2, Mitsuo Okada1, Hiroshi Nishioka1 and Shozo Yamada2
1Toranomon Hospital, Tokyo, Japan, 2Toranomon Hosp, Tokyo, Japan

Abstract

Introduction

Growth hormone (GH)-producing pituitary adenoma (PA) in childhood or young adulthood is a rare clinical entity, and the detail about the clinical and pathological features are not well understood. We present herein the treatment outcome and pathological features of this entity.

Methods

We experienced the 22 consecutive patients with GH-producing PA aged 20 years old or younger, treated between February 2003 and September 2016 at Toranomon Hospital. All the patients had undergone transsphenoidal surgery (TSS), and the pathological diagnosis confirmed GH-producing PAs. We retrospectively reviewed the clinical presentation, treatment outcome and pathological features of these patients.

Results

Our cohort included 13 men and 9 women with mean age of 16.8 years old (7-20 years old) at surgery. According to Knosp-Steiner classification, 7 cases were classified as Grade 0, 5 were Grade 1, 4 were Grade 2, 1 was Grade 3, and 5 were Grade 4. The average tumor size was 24mm in diameter. Gross total resection was achieved in 14 of 22 cases (63.6%). By immunohistochemistry, 8 cases were diagnosed as GH producing adenoma and 14 cases were GH and prolactin (PRL) producing adenoma (immunoreactive cells for PRL> 1%). Among 12 cases examined, 4 were AIP mutation-positive, and 2 were diagnosed as Carney complex. In 11 cases, complete remission of the disease was achieved only by surgical treatment at the last follow-up (50%). In other 9 cases, GH was also controlled well and stabilized by TSS followed by additional radiotherapy and/or medication during the mean follow-up period of 48.4 months (1-156 months).

Discussion

In our institute, the clinical outcome of patients with GH-producing PA in their childhood or young adulthood was worse than that of all ages. Current our study indicates that genetic mutations of AIP or PRKAR1Awere significantly more common in younger patients (< 20 years) than in adult patients, suggesting our poor surgical outcomes in this series.

Conclusion

In young patients with acrogigantism, complete remission is less likely to be achieved by surgery alone. Multidisciplinary treatment: radical resection of the tumor followed by additional radiotherapy and/or medication is mandatory to achieve optimal clinical outcome.

 

Nothing to Disclose: YN, NF, MO, HN, SY