Takahashi H, Tokuda N, Kariya H
Department of Pediatrics, Kanazawa Medical University, Ishikawa-ken, Japan.
Acta Paediatr Jpn. 1990 Feb;32(1):88-93. doi: 10.1111/j.1442-200x.1990.tb00789.x.
We report a 7-year-old boy who developed incomplete sexual precocity due to a human chorionic gonadotropin (HCG)-producing tumor in the pineal region. The patient presented enlarged testes (3 x 2 x 2 cm) bilaterally, enlarged penis, pubic hair development of Tanner Stage III, advanced bone age and growth spurt. Initial hormonal studies showed an adult male level of testosterone (13 ng/ml) and a high level of HCG as well as HCG-beta subunit. A high basal level of LH, probably due to immuno-cross-reactivity with HCG, and low basal level of FSH, probably suppressed by testosterone, did not respond to LH-RH infusion. Search for the site of HCG production failed at the initial workup, but calcification without definite signs of tumor in the pineal region was found by conventional brain CT scan. Because of subsequent progression of clinical and laboratory findings of sexual precocity, nuclear magnetic resonance computed tomographic (NMR-CT) scan was performed, which confirmed the presence of a pineal tumor three months later. The patient was treated with 4,500 rad. of radiation therapy, and responded dramatically to this regimen. He has been followed for more than two years without any signs of recurrence. We have reported here a very rare case of incomplete sexual precocity due to an HCG-producing intracranial tumor in the pineal region. An NMR-CT scan is a very useful tool for the diagnosis of some types of pineal tumor, such as germinoma, which are highly radiosensitive.
我们报告一名7岁男孩,因松果体区产生人绒毛膜促性腺激素(HCG)的肿瘤而出现不完全性性早熟。患者双侧睾丸增大(3×2×2cm),阴茎增大,坦纳Ⅲ期阴毛发育,骨龄提前且有生长加速。初始激素研究显示睾酮水平达到成年男性水平(13ng/ml),HCG以及HCG-β亚基水平升高。LH基础水平较高,可能是由于与HCG存在免疫交叉反应,而FSH基础水平较低,可能是被睾酮抑制,对LH-RH输注无反应。在初始检查中未能找到HCG的产生部位,但通过常规脑部CT扫描发现松果体区有钙化但无明确肿瘤迹象。由于性早熟的临床和实验室检查结果随后进展,进行了核磁共振计算机断层扫描(NMR-CT),三个月后证实存在松果体肿瘤。患者接受了4500拉德的放射治疗,对该治疗方案反应显著。对他进行了两年多的随访,没有任何复发迹象。我们在此报告了一例非常罕见的因松果体区产生HCG的颅内肿瘤导致的不完全性性早熟病例。NMR-CT扫描是诊断某些类型松果体肿瘤(如对放疗高度敏感的生殖细胞瘤)的非常有用的工具。