Juvenile granulosa cell tumors and other sex cord-stromal tumors in childhood and adolescence

被引：0
作者：
Schneider D.T. [1 ,2 ,4 ]
Calaminus G. [1 ,2 ]
Harms D. [1 ,3 ]
Göbel U. [1 ,2 ]
机构：
[1] MAKEI Arbeitsgruppe, Deutschen Gesell. Padiat. Onkologie
[2] Klinik fur Kinder-Onkolologie, Heinrich-Heine-Universität, Düsseldorf
[3] Institut für Paidopathologie, Deutsches Kindertumorregister, Universität zu Kiel
[4] Klinik fur Kinder-Onkologie, Heinrich-Heine-Universität, 40225 Dußsseldorf
来源：
Der Gynäkologe | 2004年 / 37卷 / 9期
关键词：
Chemotherapy; Children; Germ cell tumors; Ovarian sex cord-stromal tumors; Ovary;
D O I：
10.1007/s00129-004-1581-0
中图分类号：
学科分类号：
摘要：
Ovarian sex cord-stromal tumors (OSCST) are rare but characteristic pediatric tumors that develop from the non-germinative component of the ovaries. This review summarizes the experience from 62 patients that were prospectively reported to the German MAKEI studies on non-testicular germ cell tumors and diagnosed and treated according to uniform standards. The event-free and overall survival of all patients is 0.87±0.05 and 0.88±0.05, respectively. Stage according to FIGO and mitotic activity have proven prognostically relevant. All events have occurred in patients stage Ic (with malignant ascites of pre-operative tumor rupture) or stage II-III, and all events occurred in patients with more than 20 mitoses per 120 high power fields. In the rare more advanced tumors stage II-III, adjuvant cisplatin-based chemotherapy has been effective. In conclusion, overall prognosis of children and adolescents with OSCST is favorable, particularly if they are diagnosed at low tumor stage. Patients, in whom an aggressive course of disease has to be expected, can be identified at diagnosis by a standardized clinical and histopathologic assessment and may benefit from adjuvant cisplatin-based chemotherapy. On this basis a prospective therapeutic protocol (MAKEI-SCST 2004) has been designed, which is opening for recruitment in 2004 (http://www.unikiinik-duesseldorf.de/scst).
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页码：815 / 821
页数：6
相关论文
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