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卵巢幼年型颗粒细胞瘤和卵泡膜细胞瘤作为性早熟的罕见病因:病例报告及文献复习

Juvenile granulosa and theca cell tumor of the ovary as a rare cause of precocious puberty: case report and review of literature.

作者信息

Fleming Nathalie A, de Nanassy Joseph, Lawrence Sarah, Black Amanda Y

机构信息

Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada.

出版信息

J Pediatr Adolesc Gynecol. 2010 Aug;23(4):e127-31. doi: 10.1016/j.jpag.2010.01.003. Epub 2010 Apr 3.

Abstract

BACKGROUND

The differential diagnosis for precocious puberty in a young female includes peripheral causes. This case documents a rare cause of peripheral precocious puberty--a juvenile granulosa and theca cell ovarian tumor--and a brief review of the literature for this tumor type.

CASE

A 7-year-old girl presented with rapid onset of pubertal development and elevated estradiol levels. Menarche occurred 5 months after thelarche. A thorough workup revealed a large multicystic left ovary. Other causes of precocious puberty were excluded. She underwent an exploratory laparotomy and left salpingo-oophorectomy. Pathology reported a juvenile granulosa and theca cell tumor of the ovary, FIGO stage 1A. Postoperatively, she experienced a cessation of vaginal bleeding and estradiol levels normalized. A literature review found that early stage disease has an excellent prognosis and that adjuvant chemotherapy is not indicated in this setting.

SUMMARY AND CONCLUSION

Juvenile granulosa and theca cell tumor of the ovary is a rare cause of peripheral precocious puberty, even more so than juvenile granulosa cell tumor, due to the theca component. Treatment is surgical and an excellent prognosis is possible for early stage disease.

摘要

背景

年轻女性性早熟的鉴别诊断包括外周性病因。本病例记录了一种罕见的外周性性早熟病因——青少年颗粒细胞瘤和卵泡膜细胞瘤——并对该肿瘤类型的文献进行简要综述。

病例

一名7岁女孩出现青春期发育迅速且雌二醇水平升高。乳房发育5个月后月经初潮。全面检查发现左侧卵巢有一个大的多囊性肿物。排除了其他性早熟病因。她接受了剖腹探查术和左侧输卵管卵巢切除术。病理报告为卵巢青少年颗粒细胞瘤和卵泡膜细胞瘤,国际妇产科联盟(FIGO)分期为1A期。术后,她阴道出血停止,雌二醇水平恢复正常。文献综述发现早期疾病预后良好,在此情况下无需辅助化疗。

总结与结论

卵巢青少年颗粒细胞瘤和卵泡膜细胞瘤是外周性性早熟的罕见病因,由于存在卵泡膜成分,甚至比青少年颗粒细胞瘤更为罕见。治疗方法为手术,早期疾病预后良好。

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