Seltene maligne Ovarialtumore

Kernaussagen

Etwa 10 % aller Ovarialtumore werden den seltenen malignen Ovarialtumoren zugerechnet. Sie teilen sich in die beiden Gruppen der Keimzelltumore (mit einem typischen Erkrankungszeitraum im jungen Erwachsenenalter) und der Keimstrang-Stroma-Tumore (ohne besondere Altersabhängigkeit). Keimzelltumore sind überwiegend endokrin inaktiv, wohingegen einige Keimstrang-Stroma-Tumoren auch hormonell aktiv sind und zu erhöhten Östrogenwerten führen. Obwohl das Staging in Analogie zu den epithelialen Ovarialtumoren erfolgt, unterscheidet sich insbesondere die Therapie der Keimzell- und Keimstrangtumore beträchtlich vom epithelialen Ovarialkarzinom. So besteht die Therapie maligner Keimzelltumore zunächst in einer operativen kompletten Zytoreduktion (i. d. R. fertilitätserhaltend). In den meisten Fällen ist eine adjuvante Kombinationschemotherapie nach dem BEP-Schema indiziert. Bei Keimstrang-Stroma-Tumoren ist in vielen Situationen die chirurgische Therapie alleine ausreichend – auch in der Rezidivtherapie. Eine postoperativ anschließende Chemotherapie wird nur bei fortgeschrittenen Stadien empfohlen und ist hinsichtlich der Effektivität bisher nicht in Studien untersucht worden.

• Literatur

• 1 Quirk JT, Natarajan N. Ovarian cancer incidence in the United States, 1992–1999. Gynecol Oncol 2005; 97: 519-523
• 2 Gershenson DM. Management of early ovarian cancer: germ cell and sex cord-stromal tumors. Gynecol Oncol 1994; 55: S62-S72
• 3 American College of Obstetricians and Gynecologists. The role of generalist obstetrician-gynecologist in the early detection of ovarian cancer. ACOG Committee Opinion No. 280 American College of Obstetricians and Gynecologists. Obstet Gynecol 2002; 100: 1413-1416
• 4 Mann JR, Raafat F, Robinson K et al. ; The United Kingdom Childrenʼs Cancer Study Groupʼs second germ cell tumor study: carboplatin, etoposide, and bleomycin are effective treatment for children with malignant extracranial germ cell tumors, with acceptable toxicity. J Clin Oncol 2000; 18: 3809-3818
• 5 Evans 3rd AT, Gaffey TA, Malkasian jr. GD, Annegers JF. Clinicopathologic review of 118 granulosa and 82 theca cell tumors. Obstet Gynecol 1980; 55: 231-238
• 6 Benedet JL, Bender H, Jones 3rd H et al. FIGO staging classifications and clinical practice guidelines in the management of gynecologic cancers. FIGO Committee on Gynecologic Oncology. Int J Gynaecol Obstet 2000; 70: 209-262
• 7 NCCN Clinical Practice Guidelines Ovarian Cancer, Version 1.2013. http://www.nccn.org/professionals/physician_gls/pdf/ovarian.pdf Stand: 2013
• 8 Colombo N, Peiretti M, Garbi A et al. ESMO Guidelines Working Group. Non-epithelial ovarian cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 2012; 23 Suppl 7: vii20-vii26
• 9 Bafna UD, Umadevi K, Kumaran C et al. Germ cell tumors of the ovary: is there a role for aggressive cytoreductive surgery for nondysgerminomatous tumors?. Int J Gynecol Cancer 2001; 11: 300-304
• 10 Gershenson DM, Miller AM, Champion VL et al. Gynecologic Oncology Group. Reproductive and sexual function after platinum-based chemotherapy in long-term ovarian germ cell tumor survivors: a Gynecologic Oncology Group Study. J Clin Oncol 2007; 25: 2792-2797
• 11 Gershenson DM, Morris M, Cangir A et al. Treatment of malignant germ cell tumors of the ovary with bleomycin, etoposide, and cisplatin. J Clin Oncol 1990; 8: 715-720
• 12 Palenzuela G, Martin E, Meunier A et al. Comprehensive staging allows for excellent outcome in patients with localized malignant germ cell tumor of the ovary. Ann Surg 2008; 248: 836-841
• 13 Low JJ, Perrin LC, Crandon AJ, Hacker NF. Conservative surgery to preserve ovarian function in patients with malignant ovarian germ cell tumors. A review of 74 cases. Cancer 2000; 89: 391-398
• 14 Mahdi H, Swensen RE, Hanna R et al. Prognostic impact of lymphadenectomy in clinically early stage malignant germ cell tumour of the ovary. Br J Cancer 2011; 105: 493-497
• 15 Kumar S, Shah JP, Bryant CS et al. The prevalence and prognostic impact of lymph node metastasis in malignant germ cell tumors of the ovary. Gynecol Oncol 2008; 110: 125-132
• 16 Williams SD, Blessing JA, Moore DH et al. Cisplatin, vinblastine, and bleomycin in advanced and recurrent ovarian germ-cell tumors. A trial of the Gynecologic Oncology Group. Ann Intern Med 1989; 111: 22-27
• 17 Zanetta G, Bonazzi C, Cantù M et al. Survival and reproductive function after treatment of malignant germ cell ovarian tumors. J Clin Oncol 2001; 19: 1015-1020
• 18 Culine S, Lhomme C, Michel G et al. Is there a role for second-look laparotomy in the management of malignant germ cell tumors of the ovary? Experience at Institut Gustave Roussy. J Surg Oncol 1996; 62: 40-45
• 19 Loehrer PJ, Johnson D, Elson P et al. Importance of bleomycin in favorable prognosis disseminated germ cell tumors: an Eastern Cooperative Oncology Group trial. J Clin Oncol 1995; 13: 470-476
• 20 Murugaesu N, Schmid P, Dancey G et al. Malignant ovarian germ cell tumors: identification of novel prognostic markers and long-term outcome after multimodality treatment. J Clin Oncol 2006; 24: 4862-4866
• 21 Colombo N, Parma G, Zanagnolo V, Insinga A. Management of ovarian stromal cell tumors. J Clin Oncol 2007; 25: 2944-2951
• 22 Zhang M, Cheung MK, Shin JY et al. Prognostic factors responsible for survival in sex cord stromal tumors of the ovary – an analysis of 376 women. Gynecol Oncol 2007; 104: 296-400
• 23 Brown J, Shvartsman HS, Deavers MT et al. The activity of taxanes in the treatment of sex cord-stromal ovarian tumors. J Clin Oncol 2004; 22: 3517-3523
• 24 Fishman A, Kudelka AP, Tresukosol D et al. Leuprolide acetate for treating refractory or persistent ovarian granulosa cell tumor. J Reprod Med 1996; 41: 393-396
• 25 Schünke M, Schulte E, Schumacher U. Prometheus LernAtlas der Anatomie: Hals und Innere Organe. Stuttgart: Thieme; 2005