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Gynaecological sarcomas

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  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)