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A 10-year-5-month-old boy presented with signs of precocious puberty including facial hair development, voice deepening, and increased growth velocity that began before 9 years old. Physical examination showed height of 140.4 cm (47%), Tanner 4 genitalia, Tanner 3 pubic hair, and testicular volumes of 15 cc (left) and 12 to 15 cc (right). Bone age (BA) was 13.5 years with a predicted adult height (PAH) by Bayley-Pinneau method of 63 inches vs mid-parental target height of 67 inches. Serum luteinizing hormone (LH) was 2.9 mIU/mL (2.9 IU/L) and serum testosterone level was 3.78 nmol/L (109 ng/dL). Thyroid-stimulating hormone (TSH), glucose, serum and cerebral spinal fluid β-human chorionic gonadotropin (CSF β-HCG), dehydroepiandrosterone sulfate (DHEAS), and cerebral spinal fluid alpha-fetoprotein (CSF AFP) were normal. Magnetic resonance imaging (MRI) of the pituitary showed a small, heterogeneous and fat-containing lesion extending inferiorly from the tuber cinereum consistent with teratoma (Fig 1A), a rare cause of central precocious puberty (CPP) [1]. Figure 1B shows hypothalamic hamartoma, a common organic cause of CPP [1, 2]. Figure 1C shows a normal pituitary gland. Treatment consisted of gonadotropin-releasing hormone (GnRH) agonist Lupron (30 mg) every 3 months [2]. Repeat evaluation 1 year after therapy showed BA of 14 years, PAH of 64.4 inches, and growth velocity of 5.3 cm/year. Given response to GnRH agonist injections, the absence of central nervous system (CNS) symptoms referable to the teratoma, and no change in MRI, surgical intervention was not recommended.
Figure 1.
T1-weighted MRI in sagittal (left) and coronal (right) planes. (A) Our patient, hypothalamic teratoma indicated by white arrows. Areas of hyperintensity (fat sebum), hypointensity (small calcifications), and isointense signal. (B) Hypothalamic hamartoma, lesion is isointense compared to gray matter. (C) Normal hypothalamus and pituitary.
Abbreviations
- BA
bone age
- CPP
central precocious puberty
- GnRH
gonadotropin-releasing hormone
- MRI
magnetic resonance imaging
- PAH
predicted adult height
Contributor Information
Renee Robilliard, Division of Pediatric Endocrinology and Diabetes, Rhode Island/Hasbro Children's Brown University Health, Providence, RI 02903, USA.
Alexandra Delano, Division of Pediatric Endocrinology and Diabetes, Rhode Island/Hasbro Children's Brown University Health, Providence, RI 02903, USA.
Glenn A Tung, Department of Radiology, Rhode Island Brown University Health, Providence, RI 02903, USA.
Jose Bernardo Quintos, Division of Pediatric Endocrinology and Diabetes, Rhode Island/Hasbro Children's Brown University Health, Providence, RI 02903, USA.
Funding
No public or commercial funding.
Disclosures
None declared.
Informed Patient Consent for Publication
Signed informed consent obtained directly from the patient's relatives or guardians.
References
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