Al-Jumaily Usama, Al-Hussaini Maysa, Ajlouni Fatenah, Abulruz Abdulrahman, Sultan Iyad
Department of Pediatric Oncology, King Hussein Cancer Center, Queen Rania Al Abdullah St,, Amman, 11941, Jordan.
J Med Case Rep. 2012 Jan 16;6:13. doi: 10.1186/1752-1947-6-13.
Development of a sarcomatous component in a germ cell tumor is an uncommon phenomenon. Most cases reported have a grim prognosis. Growing teratoma syndrome is also an uncommon phenomenon and occurs in approximately 2% to 7% of non seminomatous germ cell tumors and should be treated surgically.
We report the case of a 12-year-old Asian girl with an ovarian mixed germ cell tumor containing a rhabdomyosarcomatous component. She was treated with a germ cell tumor chemotherapy regimen and rhabdomyosarcoma-specific chemotherapy. Towards the end of her treatment, she developed a retroperitoneal mass that was increasing in size. It was completely resected, revealing a mature teratoma, consistent with growing teratoma syndrome. She is still in complete remission approximately three years after presentation.
The presence of rhabdomyosarcoma in a germ cell tumor should be treated by a combined chemotherapy regimen (for germ cell tumor and rhabdomyosarcoma). In addition, development of a mass during or after therapy with normal serum markers should raise the possibility of growing teratoma syndrome that should be treated surgically.
生殖细胞肿瘤中出现肉瘤成分是一种罕见现象。大多数报道的病例预后不良。生长性畸胎瘤综合征也是一种罕见现象,约2%至7%的非精原性生殖细胞肿瘤会出现该综合征,应进行手术治疗。
我们报告一例12岁亚洲女孩,患有卵巢混合性生殖细胞肿瘤,其中包含横纹肌肉瘤成分。她接受了生殖细胞肿瘤化疗方案和横纹肌肉瘤特异性化疗。在治疗接近尾声时,她出现了一个不断增大的腹膜后肿块。该肿块被完全切除,病理显示为成熟畸胎瘤,符合生长性畸胎瘤综合征。就诊约三年后,她仍处于完全缓解状态。
生殖细胞肿瘤中存在横纹肌肉瘤应采用联合化疗方案(针对生殖细胞肿瘤和横纹肌肉瘤)进行治疗。此外,在治疗期间或治疗后,血清标志物正常但出现肿块,应提高生长性畸胎瘤综合征的可能性,对此应进行手术治疗。