Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2017;88(6):423-426.
doi: 10.1159/000479107. Epub 2017 Jul 26.

Occurrence of Cranial Neoplasms in Pediatric Patients with Noonan Syndrome Receiving Growth Hormone: Is Screening with Brain MRI prior to Initiation of Growth Hormone Indicated?

Kanthi Bangalore Krishna  1 , Pedro Pagan  2 , Oscar Escobar  2 , Jadranka Popovic  2
Affiliations

Occurrence of Cranial Neoplasms in Pediatric Patients with Noonan Syndrome Receiving Growth Hormone: Is Screening with Brain MRI prior to Initiation of Growth Hormone Indicated?

Kanthi Bangalore Krishna et al. Horm Res Paediatr. 2017.

Abstract

Noonan syndrome (NS) is associated with short stature. Growth hormone treatment has been FDA approved for use in these patients. Children with NS are at a higher risk of developing benign and malignant proliferative disorders, primary brain tumors being one of them. Since growth hormone therapy can worsen the tumor burden, screening with a brain MRI prior to growth hormone initiation in NS patients is strongly recommended. Here we present two NS patients who developed different primary brain tumors while being on growth hormone therapy.

Keywords: Brain tumors; Growth hormone; Noonan syndrome.

PubMed Disclaimer

Conflict of interest statement

Disclosure Statement

All authors have no disclosures/conflict of interest except as mentioned below: Oscar Escobar served as a member of an Advisory Board meeting for Versartis, Inc.

Figures

Fig. 1.
Fig. 1.
Case No. 1. Transverse view (a) and sagittal view (b) of MRI of brain showing two separate lesions in the right anteromedial temporal lobe (yellow arrow) and right medial cerebellum (red arrow) with similar signal characteristics.
Fig. 2.
Fig. 2.
Case No. 2. Sagittal view (a) and transverse view (b) of MRI of brain showing a heterogeneously enhancing tumor within the fourth ventricle with drop metastasis in the anterior recess of the third ventricle (indicated by arrows).
See this image and copyright information in PMC

References

    1. van der Burgt I: Noonan syndrome. Orphanet J Rare Dis 2007; 2:4. - PMC - PubMed
    1. Fryssira H, Leventopoulos G, Psoni S, KitsiouTzeli S, Stavrianeas N, Kanavakis E: Tumor development in three patients with Noonan syndrome. Eur J Pediatr 2008; 167:1025–1031. - PubMed
    1. Karafin M, Jallo GI, Ayars M, Eberhart CG, Rodriguez FJ: Rosette forming glioneuronal tumor in association with Noonan syndrome: pathobiological implications. Clin Neuropathol 2011; 30:297–300. - PMC - PubMed
    1. Perry JK, Emerald BS, Mertani HC, Lobie PE: The oncogenic potential of growth hormone. Growth Horm IGF Res 2006; 16:277–289. - PubMed
    1. Schuettpelz LG, McDonald S, Whitesell K, Desruisseau DM, Grange DK, Gurnett CA, Wilson DB: Pilocytic astrocytoma in a child with Noonan syndrome. Pediatr Blood Cancer 2009; 53:1147–1149. - PubMed

Substances