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以性早熟为表现的肝母细胞瘤:一例报告

Hepatoblastoma presenting as precocious puberty: a case report.

作者信息

Marino Silvia, Caruso Manuela, Magro Gaetano, D'Amico Salvatore, La Spina Milena, Moscheo Carla, Russo Giovanna, Di Cataldo Andrea

出版信息

J Pediatr Endocrinol Metab. 2015 Mar;28(3-4):429-32. doi: 10.1515/jpem-2014-0167.

Abstract

Hepatoblastoma (HB), a primary liver tumor in childhood, is often accompanied by alpha-fetoprotein (AFP) secretion, and sometimes by β-human chorionic gonadotropin hormone (β-hCG) secretion, and this can cause peripheral precocious puberty (PPP). We describe a case of PPP associated with HB. Laboratory tests showed an increase in AFP, β-hCG and testosterone values, and suppression of follicle-stimulating hormone and luteinizing hormone levels. After chemotherapy and surgery, AFP, β-hCG and testosterone levels normalized and signs of virilization did not progress further. The child did not show evidence for tumor recurrence after 16 months of follow-up. New therapeutic approaches and early diagnosis may ensure a better prognosis of virilizing HB, than reported in the past. Assessment of PPP should always take into account the possibility of a tumoral source.

摘要

肝母细胞瘤(HB)是儿童期的一种原发性肝脏肿瘤,常伴有甲胎蛋白(AFP)分泌,有时还伴有β-人绒毛膜促性腺激素(β-hCG)分泌,这可导致外周性早熟(PPP)。我们描述了1例与HB相关的PPP病例。实验室检查显示AFP、β-hCG和睾酮值升高,促卵泡生成素和促黄体生成素水平受到抑制。化疗和手术后,AFP、β-hCG和睾酮水平恢复正常,男性化体征未进一步进展。随访16个月后,该患儿未出现肿瘤复发迹象。与过去报道的情况相比,新的治疗方法和早期诊断可能确保男性化HB有更好的预后。对PPP的评估应始终考虑到肿瘤来源的可能性。

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