Central precocious puberty: management and long-term outcome

Objective: Treatment of metastatic adrenocortical carcinoma (ACC) is challenging and long-term survival rates are exceedingly low. Long-term outcome data for pediatric patients who received mitotane is very limited. Methods: We describe the case of a 2-year-old boy with ACC with a lung metastasis. He was treated with surgery, chemotherapy, and mitotane, and remains disease-free 13 years after diagnosis. Results: The key endocrine issues learned from this case include: adrenal-derived sex-steroid and insulin-like growth factor-2 levels are correlated with disease status; very high doses of glucocorticoid and mineralocorticoid are required while on treatment of mitotane; and central precocious puberty needs to be detected and treated in a timely manner to preserve final adult height. Conclusion: We report a case of pediatric ACC with metastasis that was successfully treated with surgery, chemotherapy, and adjuvant therapy with mitotane. Appropriate endocrine testing and management are important for long-term survival and quality of life.

Download Full-text

Long-Term Outcome after Depot Gonadotropin-Releasing Hormone Agonist Treatment of Central Precocious Puberty: Final Height, Body Proportions, Body Composition, Bone Mineral Density, and Reproductive Function

The Journal of Clinical Endocrinology & Metabolism ◽

10.1210/jc.84.12.4583 ◽

1999 ◽

Vol 84 (12) ◽

pp. 4583-4590 ◽

Cited By ~ 42

Author(s):

S. Heger

Keyword(s):

Final Height ◽

Reproductive Function ◽

Central Precocious Puberty ◽

Gonadotropin Releasing Hormone ◽

Body Proportions ◽

Mineral Density ◽

Term Outcome ◽

Long Term Outcome ◽

Gonadotropin Releasing Hormone Agonist

Download Full-text

Use of Gonadotropin-Releasing Hormone Analogs in Children: Update by an International Consortium

Hormone Research in Paediatrics ◽

10.1159/000501336 ◽

2019 ◽

Vol 91 (6) ◽

pp. 357-372 ◽

Cited By ~ 11

Author(s):

Kanthi Bangalore Krishna ◽

John S. Fuqua ◽

Alan D. Rogol ◽

Karen O. Klein ◽

Jadranka Popovic ◽

...

Keyword(s):

Precocious Puberty ◽

Qualitative Evaluation ◽

Outcome Data ◽

Evidence Based ◽

Pediatric Endocrinology ◽

Term Outcome ◽

Long Term Outcome ◽

Future Studies ◽

Hormone Analogs

This update, written by authors designated by multiple pediatric endocrinology societies (see List of Participating Societies) from around the globe, concisely addresses topics related to changes in GnRHa usage in children and adolescents over the last decade. Topics related to the use of GnRHa in precocious puberty include diagnostic criteria, globally available formulations, considerations of benefit of treatment, monitoring of therapy, adverse events, and long-term outcome data. Additional sections review use in transgender individuals and other pediatric endocrine related conditions. Although there have been many significant changes in GnRHa usage, there is a definite paucity of evidence-based publications to support them. Therefore, this paper is explicitly not intended to evaluate what is recommended in terms of the best use of GnRHa, based on evidence and expert opinion, but rather to describe how these drugs are used, irrespective of any qualitative evaluation. Thus, this paper should be considered a narrative review on GnRHa utilization in precocious puberty and other clinical situations. These changes are reviewed not only to point out deficiencies in the literature but also to stimulate future studies and publications in this area.

Download Full-text

Cranial MRI abnormalities and long-term follow-up of the lesions in 770 girls with Central Precocious Puberty

The Journal of Clinical Endocrinology & Metabolism ◽

10.1210/clinem/dgab190 ◽

2021 ◽

Author(s):

Didem Helvacıoğlu ◽

Serap Demircioğlu Turan ◽

Tülay Güran ◽

Zeynep Atay ◽

Adnan Dağçınar ◽

...

Keyword(s):

Precocious Puberty ◽

Central Precocious Puberty ◽

Final Decision ◽

Low Grade ◽

Long Term Outcome ◽

Neoplastic Lesions ◽

Cranial Mri ◽

Cns Lesions

Abstract Context Central precocious puberty(CPP) may arise from central nervous system(CNS) lesions in a few affected girls. Recently, the incidence of girls with CPP has increased mostly in 6-8 year-olds, in whom the necessity of magnetic resonance imaging(MRI) is debated. Objective To investigate the frequency, long-term outcome and potential predictors of CNS lesions in a large cohort of girls with CPP. Design and Method A multi-center cohort of 770 Turkish girls with CPP who had systematic cranial MRI between 2005-2017. Age at puberty onset was < 6 years in 116 and 6-8 years in 654. CNS lesions were followed until final decision(6.2±3.1years). Potential predictors of CNS lesions were evaluated by univariate analyses. Results 104/770(13.5%) girls had abnormal brain MRI. Of these, 2.8% were previously known CNS lesions, 3.8% had newly detected and causally related CNS lesions, 3.1 % were possibly related and 3.8% were incidental. Only two(0.25%) neoplastic lesions (one low grade glioma and one meningioma) were identified; neither required intervention over follow-up of 6 and 3.5 years respectively. Age at breast developmen <6 years [OR(95%CI); 2.38(1.08-5.21)] and the peak LH/FSH ratio >0.6 [OR(95%CI); 3.13 (1.02-9.68)] were significantly associated with CNS lesions. However, both patients with neoplastic lesions were >6 years old. Conclusion Although age and LH/FSH ratio are significant predictors of CNS lesions, their predictive power is weak. Thus, systematic MRI seems to be the most efficient current approach to avoid missing an occult CNS lesion in girls with CPP, despite the low likelihood of finding a lesion requiring intervention.

Download Full-text