Objective: To analyze the clinical characteristics and treatment of non-germinomatous germ cell tumor (NGGCT) in one girl with the initial onset of homosexual peripheral precocious puberty (PPP) and central diabetes insipidus (CDI) and eventually progressed into central precocious puberty (CPP).
Methods: The clinical data of a girl who was diagnosed as NGGCT with the onset of PPP and CDI and eventually progressed into CPP was analyzed. Related literature up to November 2021 was searched with the terms of “(precocious puberty OR PP) AND (non-germinomatous germ cell tumor OR NGGCT)”in Wanfang, CNKI and PubMed database in Chinese and English. The characteristics of the disease were summarized.
Results: A 6 years and 9 months old girl with complaining of polyuria, polydipsia and breast development was admitted to the First Affiliated Hospital of Sun Yat-sen University Pediatric Department in June 2020. Upon admission, the initial serum showed low luteinizing hormone (LH), low Follicle-stimulating hormone (FSH) and high estradiol (E2). β-human chorionic gonadotropin（β-HCG）level was extremely high in both serum and cerebrospinal fluid (CSF). Pelvic ultrasound showed enlarged uterus and premature follicle. The baseline cerebral MR Perfusion showed occupation of suprasellar and pineal gland. The girl was diagnosed as the β-HCG secreting NGGCT. After the chemotherapy treatment course, her breast return to TannerⅠ, the follow-up cerebral MR Perfusion showed the occupation had almost disappeared and the β-HCG level of CSF and serum had descended to normal. After 7 months of the cessation of chemotherapy, breast development showed again and finally diagnosed as CPP after GnRHa stimulation test and treated with GnRHa.
Conclusion: We reported a girl of β-HCG secreting NGGCT with onset of precocious puberty which was very rare. For those PP girls especially in the young age, β-HCG should also be screened to rule out NGGCT. Meanwhile, for the NGGCT patients，routine follow-up of clinical manifestation, hormones, hypophysis MR，β-HCG level in serum and CSF are necessary.
15 Sep 2022 - 17 Sep 2022