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题名:Endocrine dysfunction in Taiwanese children with human chorionic gonadotropin-secreting germ cell tumors.
作者:LIN CM; LEE CT; TUNG YC; WU MZ; TSAI WY; YANG YL; LU MY; JOU ST; LIN DT; LIN KH;
来源:J Formos Med Assoc. 2014 Feb;113(2):102-5. doi: 10.1016/j.jfma.2012.04.007. Epub [ IF= 0.00 ] ]
URL :10.1016/j.jfma.2012.04.007
日期:20140317
摘要:BACKGROUND/PURPOSE: Human chorionic gonadotropin (HCG)-secreting germ cell tumors
(GCTs) are rare childhood malignancies with unique clinical manifestations but
delayed diagnosis is common. The purpose of this study is to investigate the
clinical manifestations and endocrine dysfunction of Taiwanese children with
HCG-secreting GCTs. METHODS: From 1991 to 2011, 24 children (19 boys and five
girls) with HCG-secreting GCTs were evaluated for their clinical findings and
endocrine functions. RESULTS: The mean age at diagnosis of the study patients was
10.8 +/- 3.1 years. Of the 24 patients, 20 had central nervous system (CNS) GCTs
and four had primary mediastinal GCTs (PMGCTs). The most common pathologic
findings were germinomas and mixed type GCTs. The common initial symptoms and
signs included polyuria, polydipsia, rapid growth, neurologic deficit,sexual
precocity, and growth retardation. There was a delay in diagnosis in about 60% of
patients. Diabetes insipidus and hypopituitarism were common endocrine
dysfunctions in patients with CNSGCTs. Twelve boys had gonadotropin-independent
puberty upon diagnosis, which were related to their high serum beta-hCG levels.
None of the five girls had this disorder despite their high serum beta-hCG
levels. Three of the four PMGCTs patients had the classic form of Klinefelter
syndrome. CONCLUSION: Taiwanese children with HCG-secreting GCTs often have
clinical manifestations related to endocrine dysfunction. High index of suspicion
is important to avoid delayed diagnosis in these children.

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