ISSN: 1308-5727 | E-ISSN: 1308-5735
Volume: 16 Issue: 1 Year: 2024
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Turkish Society for Pediatric Endocrinology and Diabetes
A Case of Central Precocious Puberty Due to Concomitant Hypothalamic Hamartoma and Juvenile Pilocytic Astrocytoma [J Clin Res Pediatr Endocrinol]
J Clin Res Pediatr Endocrinol. 2014; 6(3): 183-186 | DOI: 10.4274/jcrpe.1306

A Case of Central Precocious Puberty Due to Concomitant Hypothalamic Hamartoma and Juvenile Pilocytic Astrocytoma

Gönül Çatlı1, Ayhan Abacı2, Ahmet Anık1, Handan Güleryüz3, Erdener Özer4, İrfan Öcal5, Nurullah Yüceer6, Kamer Mutafoğlu7
1Dokuz Eylül University Faculty Of Medicine, Department Of Pediatric Endocrinology, İzmir, Turkey
2Dokuz Eylül University Faculty of Medicine, Department of Pediatric Endocrinology, İzmir, Turkey
3Dokuz Eylül University Faculty Of Medicine, Department Of Radiology, İzmir, Turkey
4Dokuz Eylül University Faculty Of Medicine, Department Of Pathology, İzmir, Turkey
5İzmir Atatürk Training Research And Hospital, Department Of Pathology, İzmir, Turkey
6Dokuz Eylül University Faculty Of Medicine, Department Of Neurosurgery, İzmir, Turkey
7Dokuz Eylül University Faculty Of Medicine, Department Of Pediatric Oncology, İzmir, Turkey

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.

Keywords: Hypothalamic hamartoma,pilocytic astrocytoma,Central precocious puberty

Gönül Çatlı, Ayhan Abacı, Ahmet Anık, Handan Güleryüz, Erdener Özer, İrfan Öcal, Nurullah Yüceer, Kamer Mutafoğlu. A Case of Central Precocious Puberty Due to Concomitant Hypothalamic Hamartoma and Juvenile Pilocytic Astrocytoma. J Clin Res Pediatr Endocrinol. 2014; 6(3): 183-186
Manuscript Language: English
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