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伴有横纹肌肉瘤成分的卵巢生殖细胞肿瘤及生长性畸胎瘤综合征的后期发展:一例报告

Ovarian germ cell tumors with rhabdomyosarcomatous components and later development of growing teratoma syndrome: a case report.

作者信息

Al-Jumaily Usama, Al-Hussaini Maysa, Ajlouni Fatenah, Abulruz Abdulrahman, Sultan Iyad

机构信息

Department of Pediatric Oncology, King Hussein Cancer Center, Queen Rania Al Abdullah St,, Amman, 11941, Jordan.

出版信息

J Med Case Rep. 2012 Jan 16;6:13. doi: 10.1186/1752-1947-6-13.

Abstract

INTRODUCTION

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.

CASE PRESENTATION

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.

CONCLUSION

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.

摘要

引言

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。

病例报告

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。

结论

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。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8fb3/3296578/60f2bd152c6a/1752-1947-6-13-1.jpg

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