ESPE Abstracts

Background: Precocious puberty is common in boys occasionally in girls with beta human chorionic gonadotropin (β-hCG)-secreting intracranial tumors, and is reported to be peripheral precocious puberty exclusively.

Object: To described a girl with intracranial β-hCG-secreting germ cell tumor（GCT） who presented with central precocious puberty（CPP）.

Method: We described the clinical data of the 8 years and 9months girl with intracranial GCT and reviewed the relative literatures.

Result: The girl was admitted to our hospital because of breast development for 1.5 years without experience of menarche, and was diagnosed with CPP concurrently with elevated serum β-hCG levels(56.45-20.7 mIU/mL) for 7 months at local hospital. On admission, physical examination revealed precocious puberty(breast B3, axillary hair A2, pubic hair PH1), without neurological findings. Serum and cerebrospinal fluid(CSF) β-hCG was 40.03 and 103.22 mIU/ml, respectively. A magnetic resonance imaging scan of the head with enhancement revealed a 7*2mm lesion at the lenticular nucleus in the right basal ganglia considered to be the GCT focus, and an 11*8mm pineal cyst. Intracranial non-germinomatous germ cell tumor was diagnosed, and combination chemotherapy was initiated with GnRHa therapy for CPP. The girl was transferred to another brain hospital due to the stable response of chemotherapy, and the pathology of pineal lesion was ‘central neurocytoma’.

Conclusion:This is the first female case of intracranial β-hCG-secreting germ cell tumor concomitant with CPP. In this case, CPP seems unlikely to attribute to the elevated β-hCG secreted by the tumor. CNS lesion due to the tumor in the right lenticular nucleus and the pineal lesion may contribute to CPP. Detection of serum HCG (AFP) is indispensable for the diagnosis of precocious puberty, regardless of boys or girls. Cranial MRI/CT is also necessary in girls with CPP even older than 6 years of age under certain circumstances.