1 / 15

Gynaecological sarcomas

Gynaecological sarcomas. Dr Beatrice Seddon The London Sarcoma Service, University College Hospital, London, UK Connective Tissue Oncology Society Annual Meeting 13 th November 2008. Incidence of gynaecological sarcomas. < 1% of all gynaecological malignancies

sema
Download Presentation

Gynaecological sarcomas

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Gynaecological sarcomas • Dr Beatrice Seddon • The London Sarcoma Service, University College Hospital, London, UK • Connective Tissue Oncology Society Annual Meeting • 13th November 2008

  2. Incidence of gynaecological sarcomas • < 1% of all gynaecological malignancies • Majority are uterine tumours • Also cervix, vagina, vulva, ovary (all rare) • Includes: • Leiomyosarcoma (uterus, vagina, vulva, ovary) • Endometrial stromal sarcoma • Undifferentiated endometrial sarcoma • Rhabdomyosarcoma (vagina/cervix) • Other soft tissue sarcoma subtypes (MFH, angiosarcoma, ASPS, DFSP) • Does not include malignant mixed müllerian tumours/carcinosarcomas • Surgery is main component of management

  3. Uterine leiomyosarcoma • 6.4 cases per million in USA (total ~2000 cases) • Median age approx 50 years • 2 - 4 % of all uterine cancers • Majority (70%) >5 cm in diameter • Incidence of malignancy in uterine fibroids/leiomyomata 0.2 - 0.7% • Approximately 50% express ER and PR

  4. FIGO Staging Corpus Uteri • Stage I - tumour confined to corpus uteri • IA – tumour limited to endometrium • IB – invades ≤ ½ myometrium • IC – invades > ½ myometrium • Stage II - tumour invades cervix but does not extend beyond the uterus • IIA – endocrevical gland involvement only • IIB – cervical stromal invasion • Stage III - local and/or regional spread • IIIA – tumour involves serosa and/or adnexa and/or +ve peritoneal washings • IIIB – vaginal involvement (direct extension or metastatic spread) • IIIC – metastasis to pelvic and or para-aortic lymph nodes • Stage IVA - tumour invades bladder mucosa and/or bowel mucosa • Stage IVB - distant metastases

  5. FIGO stage at presentation

  6. Treatment outcome

  7. Role of adjuvant chemotherapy in uterine leiomyosarcoma • Outcome for uterine leiomyosarcoma is poor • Many relapses are distant • Could use of adjuvant chemotherapy improve outcome?

  8. Role of adjuvant chemotherapy in soft tissue sarcoma • Lancet meta-analysis of 1568 patients in 14 trials of adjuvant chemotherapy in soft tissue sarcoma • Total group - improved local RFS, distant RFS, overall RFS; but no overall survival benefit • EORTC adjuvant study also negative (ASCO 2007) • Subgroups: • 263 patients with uterine sarcoma - no survival benefit • Suggestion of benefit in meta-analysis for extremity tumours • ? Benefit for selected high risk patients

  9. Role of adjuvant chemotherapy • Retrospective series of 208 patients with uterine leiomyosarcoma • Identified prognostic factors: • Prognostic factor Score • Age <51 years 1 • Tumour >5 cm 1 • FIGO II-IV 1 • Intermediate/high grade 2 • Giuntoli et al, Gynaecol Oncol 2003; 89:460-9

  10. Role of adjuvant chemotherapy • Risk assessment: • Score Risk Median survival • 0-1 Low >25 years • 2-3 Intermediate 6.5 years • 4-5 High 2.1 years • Giuntoli et al, Gynaecol Oncol 2003; 89:460-9

  11. Role of adjuvant chemotherapy • Not routine • Consider treating high risk group in selected patients • Doxorubicin 60-75 mg/m2, ifosfamide 6-9 mg/m2, q.3/52, 5-6 cycles • Phase II SARC study of adjuvant gemcitabine and docetaxel in resected uterine sarcoma

  12. Endometrial stromal neoplasms • Stromal nodule (benign lesion) • Endometrial stromal sarcoma (‘low grade’ lesion) • Poorly differentiated endometrial sarcoma (‘high grade’ lesion) • Old classification of low grade and high grade ESS on basis of mitotic count no longer used

  13. Endometrial stromal sarcoma • Clinically indolent course, long natural history • High relapse rate, late relapses • Bland histology • Low mitotic count does not predict ‘bland’ behaviour • FIGO stage more accurately predicts outcome • Composed of cells identical to endometrial stromal cells in proliferative phase • High expression of ER and PR

  14. Poorly differentiated endometrial sarcoma • Anaplastic aggressive uterine tumour • Lacks endometrial stromal features - does not resemble proliferative phase endometrial cells • Mostly postmenopausal women >50 years • High recurrence rates, both locally and distant • Recurrences usually occur within 12 months of diagnosis • Outcome poor - 5 year survival 0-32%

  15. This morning’s presentations: • Staging of uterine leiomyosarcomas: • Stage specific outcomes of FIGO and AJCC systems (#34982) • Predictive value of FIGO and AJCC systems (#34970) • New prognostic marker in high grade uterine sarcoma – WT1 (#34855) • Role of adjuvant gemcitabine and docetaxel chemotherapy in uterine leiomyosarcoma (#34961) • Single institution experience of ovarian sarcoma (#35073)

More Related