A case of central precocious puberty due to concomitant hypothalamic hamartoma and juvenile pilocytic astrocytoma

• Published in: Journal of clinical research in pediatric endocrinology Vol. 6; no. 3; pp. 183 - 186
• Main Authors: Çatlı, Gönül, Abacı, Ayhan, Anık, Ahmet, Güleryüz, Handan, Özer, Erdener, Öcal, Irfan, Yüceer, Nurullah, Mutafoğlu, Kamer
• Format: Journal Article
• Language: English
• Published: Turkey Galenos Publishing 01-09-2014
• Subjects: Astrocytoma - complications; Astrocytoma - diagnosis; Astrocytoma - surgery; Biopsy; Case Report; Child; Hamartoma - complications; Hamartoma - diagnosis; Hamartoma - surgery; Humans; Hypothalamic Diseases - complications; Hypothalamic Diseases - diagnosis; Hypothalamic Diseases - surgery; Magnetic Resonance Imaging; Male; Neoplasms, Multiple Primary - complications; Neoplasms, Multiple Primary - diagnosis; Neoplasms, Multiple Primary - surgery; Puberty, Precocious - diagnosis; Puberty, Precocious - etiology; Treatment Outcome
• ISSN: 1308-5727; 1308-5735
• DOI: 10.4274/jcrpe.1306

Central precocious puberty (CPP) is caused by premature activation of the hypothalamo-pituitary-gonadal axis. More than 50% of boys with CPP have an identifiable etiology. Hypothalamic hamartoma (HH), hydrocephalus, tumors, infections, congenital defects, ischemia, radiation, or injury of the brain are the most common causes of secondary CPP. In this report, we present the case of a 2 years and 9 months old male patient who had a 30x40 mm contrast-enhancing suprasellar mass and was histopathologically diagnosed with giant HH. However, since HHs are designated as non-enhancing masses, considering the possibility of an incomplete diagnosis of a glial tumor, the patient was followed up. Clinical and radiological follow-up revealed stable findings with no evidence of tumor growth until the third year after surgery when he presented with neurological deficit due to the rapid growth of the suprasellar mass. After the second surgery, histopathological examination of the biopsy specimen revealed the lesion to be a juvenile pilocytic astrocytoma (PA). The concomitance of HH and juvenile PA is very rare. To our knowledge, this is the first report of a patient with concomitant juvenile PA and HH who developed CPP and did not have gelastic epilepsy despite the rapidly growing giant mass.