Al-Jumaily Usama, Sammour Ibrahim, Al-Muhaisen Fadi, Ajlouni Fatenah, Sultan Iyad
Department of Pediatric Oncology, King Hussein Cancer Center, Queen Rania Al Abdullah St, Amman, 11941, Jordan.
J Med Case Rep. 2011 Aug 30;5:422. doi: 10.1186/1752-1947-5-422.
The syndrome of isosexual precocious puberty associated with primary malignant hepatic tumors is rare. All previously reported cases in the literature are old and prognosis was grim.
We present the case of a 15-month-old Asian male baby who presented with precocious puberty associated with hepatoblastoma. Serum concentrations of alpha-fetoprotein and free testosterone were elevated, as was beta human chorionic gonadotropin hormone. He was treated with six courses of chemotherapy and underwent surgery. His surface markers as well as free testosterone level returned to normal during therapy. The child has now been off therapy for 18 months with no evidence of tumor recurrence at follow-up.
Virilizing hepatoblastoma is rare and reported with poor outcome, but the development of new chemotherapeutic agents and complete surgical resection are promising.
与原发性恶性肝肿瘤相关的同性性早熟综合征较为罕见。文献中所有先前报道的病例年代久远且预后严峻。
我们报告一例15个月大的亚洲男婴,其患有与肝母细胞瘤相关的性早熟。血清甲胎蛋白和游离睾酮浓度升高,β人绒毛膜促性腺激素亦升高。他接受了六个疗程的化疗并接受了手术。治疗期间其表面标志物以及游离睾酮水平恢复正常。该患儿现已停止治疗18个月,随访期间无肿瘤复发迹象。
男性化肝母细胞瘤罕见且报道的预后不佳,但新型化疗药物的研发及完整的手术切除带来了希望。