Human chorionic gonadotropin (hCG)-producing tumors cause peripheral precocious puberty (PP) in boys, but generally not in girls. Homology between LH and hCG activates the LH receptor in testicular Leydig cells, increases testosterone production, and causes virilization. However, since FSH action is required for follicle development, hCG action alone does not increase estradiol (E2) production and does not cause feminization. Only a few cases of peripheral PP with hCG tumors in girls have been reported. We describe the case of a 7-year-old Japanese girl with peripheral PP associated with an hCG-producing tumor. She had prolonged vomiting, loss of appetite, and Tanner stage III breast development. Although no apparent increase in growth rate, bone age was advanced at 9.8 years. Serum E2 was slightly elevated and LH and FSH were below the measurement sensitivity, and abdominal ultrasonography and computed tomography images showed no abnormal findings in the uterus or ovaries. Subsequently, she developed visual field disturbance and loss of consciousness, and brain magnetic resonance imaging revealed an intracranial tumor. Based on pathological findings and abnormally high serum hCG-β level (48,800 IU/L), intracranial choriocarcinoma was diagnosed. 2.5 months after the start of chemotherapy, the hCG-β level became almost negative and the breast development disappeared synchronously. Tissue immunostaining of the tumor showed strong positivity for aromatase and hCG, indicating that the choriocarcinoma cells themselves may have produced estrogen via aromatase. This unique case highlights the possibility that hCG-producing tumors can cause peripheral PP in girls as well as boys.

产生人绒毛膜促性腺激素 (hCG) 的肿瘤会导致男孩出现外周性性早熟 (PP)，但女孩通常不会。LH 和 hCG 之间的同源性激活睾丸间质细胞中的 LH 受体，增加睾酮的产生，并导致男性化。然而，由于卵泡发育需要 FSH 作用，单独的 hCG 作用不会增加雌二醇 (E2) 的产生，也不会导致女性化。仅有少数女孩外周 PP 伴 hCG 肿瘤的报道。我们描述了一名 7 岁日本女孩患有外周 PP 并伴有产生 hCG 的肿瘤的病例。她有长时间的呕吐、食欲不振和 Tanner III 期乳房发育。虽然生长速度没有明显增加，但骨龄提前了 9.8 岁。血清 E2 轻度升高，LH 和 FSH 低于测量灵敏度，腹部超声和计算机断层扫描图像显示子宫或卵巢未见异常发现。随后，她出现视野障碍和意识丧失，脑磁共振成像显示颅内肿瘤。根据病理结果和异常高的血清hCG-β水平（48,800 IU/L），诊断为颅内绒癌。化疗开始2.5个月后，hCG-β水平几乎转为阴性，乳房发育同步消失。肿瘤组织免疫染色显示芳香酶和hCG强阳性，表明绒毛膜癌细胞本身可能产生了雌激素 腹部超声和计算机断层扫描图像显示子宫或卵巢未见异常发现。随后，她出现视野障碍和意识丧失，脑磁共振成像显示颅内肿瘤。根据病理结果和异常高的血清hCG-β水平（48,800 IU/L），诊断为颅内绒癌。化疗开始2.5个月后，hCG-β水平几乎转为阴性，乳房发育同步消失。肿瘤组织免疫染色显示芳香酶和hCG强阳性，表明绒毛膜癌细胞本身可能产生了雌激素 腹部超声和计算机断层扫描图像显示子宫或卵巢未见异常发现。随后，她出现视野障碍和意识丧失，脑磁共振成像显示颅内肿瘤。根据病理结果和异常高的血清hCG-β水平（48,800 IU/L），诊断为颅内绒癌。化疗开始2.5个月后，hCG-β水平几乎转为阴性，乳房发育同步消失。肿瘤组织免疫染色显示芳香酶和hCG强阳性，表明绒毛膜癌细胞本身可能产生了雌激素 和脑磁共振成像显示颅内肿瘤。根据病理结果和异常高的血清hCG-β水平（48,800 IU/L），诊断为颅内绒癌。化疗开始2.5个月后，hCG-β水平几乎转为阴性，乳房发育同步消失。肿瘤组织免疫染色显示芳香酶和hCG强阳性，表明绒毛膜癌细胞本身可能产生了雌激素 和脑磁共振成像显示颅内肿瘤。根据病理结果和异常高的血清hCG-β水平（48,800 IU/L），诊断为颅内绒癌。化疗开始2.5个月后，hCG-β水平几乎转为阴性，乳房发育同步消失。肿瘤组织免疫染色显示芳香酶和hCG强阳性，表明绒毛膜癌细胞本身可能产生了雌激素通过芳香酶。这个独特的案例强调了产生 hCG 的肿瘤可能导致女孩和男孩的外周 PP。