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![Surgery : conservative
Ovarian preservation : in ESS and LMS :in younger age
group (chance of ovarian spread : 3%[Shah et al])
Most retrospective series : no difference in recurrence rate
whether ovaries preserved or not
Morcellation : Contraindicated :~50% increased risk of
recurrence and adversely affects survival.
If morcellation has been done re-surgery is indicated :In
re-surgery ~29% upstaging.
Coservative surgeries like myomectomy :High recurrence
rate](https://image.slidesharecdn.com/uterinesarcomafinalnight-151107183103-lva1-app6891/85/Uterine-sarcoma-13-320.jpg)
Uploaded byBeena K
Uterine sarcoma
Uterine sarcomas are a heterogeneous group of rare uterine cancers comprising 3% of uterine neoplasms. This document discusses the classification, management, and role of radiation therapy for various uterine sarcoma subtypes based on the 2014 WHO classification. It summarizes evidence from retrospective studies and phase III trials that adjuvant radiation improves local control for leiomyosarcoma and carcinosarcoma but does not provide a survival benefit. Ongoing research aims to determine optimal chemotherapy regimens and targeted therapies to improve survival by controlling metastasis.
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Uterine sarcoma
- 1. Uterine Sarcomas :Wheredo we stand? Dr.Beena.K. Radiation Oncologist AIMS,Cochin
- 2. Contents New WHOClassification Management options in each sub group and role of RT. Target Volumes of RT.
- 3. Introduction Uterine sarcomasare uncommon : 3% of all uterine neoplasm. Heterogeneous group with varying behavior. Insufficient data to make standard recommendations. Rarity of disease makes adequately powered randomized trials impractical.
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- 5. ESS LOW GRADE Endometrial stromal origin :resembles proliferative phase Low grade , <1% of uterine malignancy : (JAZF1rearrangement(7 ,17 translocation) Mostly indolent ,younger age group IHC :ER,PR,positive,CD10 +ve, hormones effective. 5yr DFS : Stage I and II :90%, stage III,IV:50%. Recurrence is common even in stage I
- 6. ESS HIGH GRADE Previously was part of undifferentiated endometrial ca . (WHO 2003 ) Now redefined in the present WHO as high grade ESS Uniform round cells ,with occasional low grade areas. ER,PR _ve,CD10 –Ve,Cyclin D1 +Ve YWHAE - FAM22 fusion +ve .(10:17 translocation) Hormone treatment is not effective . Prognosis better than undifferentiated sarcoma.
- 7. Adenosarcoma Only mesenchymalcomponent is malignant. Usually low grade ,ER ,PR positive. <6 % of uterine sarcomas Staging and treatment similar to ESS low grade 5 yr. survival :70%
- 8. LMS 40-60% uterinesarcoma : Most common. Smooth muscle origin Associated with tamoxifen therapy Desmin,+ve, ER,PR +ve ~ 30% Stage 1 and II 5 yr survival:40-70% Overall 5 yr survival: 15-25 %
- 9. UNDIFFERENTIATED UTERINE SARCOMA Nodifferentiation, pleomorphic. classified as Undifferentiated Endometrial Sarcoma WHO 2003. (UES). High grade ESS is now removed from this group (WHO 2014 ) Prognosis : bad Die within 2 yrs of diagnosis. PFS:7-10 mths,OS: 11-23 mths
- 10. CARCINOSARCOMA Previously knownas MMT :Both components are malignant high grade endometrial carcinoma with sarcomatous metaplasia. Behavior is similar to high grade endometrial papillary, clear cell carcinoma.(peritoneal, nodal mets) Staging and treatment is similar to high grade endometrial carcinoma with Sx, chemo and pelvic RT .
- 11. Evaluation: Diagnosis mostlypost op. Slide review by an experienced pathologist for typing. Pre op diagnosis : very rarely either from imaging or from FC. CT /MRI scan abdomen ,pelvis ,CT chest. If there is a highly vascular solid ,solitary tumor on USS , CT :heterogeneous enhancement, Hyper intense on MRI both T1,T2.
- 12. Role of surgery En bloc removal of tumor with hysterectomy. prophylactic lymphadenectomy is not recommended except in carcinosarcoma. Lymph nodal spread : 7-15% in uterine confined ESS and in LMS its rare. Lymphadenectomy is done in patients with suspicious nodes or gross extra uterine spread. R0 resection is associated with better PFS.
- 13. Surgery : conservative Ovarian preservation : in ESS and LMS :in younger age group (chance of ovarian spread : 3%[Shah et al]) Most retrospective series : no difference in recurrence rate whether ovaries preserved or not Morcellation : Contraindicated :~50% increased risk of recurrence and adversely affects survival. If morcellation has been done re-surgery is indicated :In re-surgery ~29% upstaging. Coservative surgeries like myomectomy :High recurrence rate
- 14. ESS /LMS/Adenosarcoma FIGO2009 staging
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- 16. RADIOTHERAPY Routine adjuvant,historically. Adjuvant : EBRT to sterilize the microscopic disease in pelvic nodes and tumor bed . Palliative RT to pelvis or sites of distant mets/ recurrence. Medically inoperable.
- 19. DRR LAT &AP Fields#33 3D PLAN
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- 22. PHASE III STUDYON ADJ RT EUROPEAN JOURNAL OF CANCER 44 (2008) 808–818
- 23. EORTC PHASE III Observation vs adj pelvic RT (51Gy/28 Fr):224 pts. , 112 in each arm 99 pts :LMS, 92 pts :CS, 30 pts :ESS Majority of patients were stage I:197 pts. LR, DFS, OS, Distant mets.
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- 25. OS P:0.92 FAILED TO PROVESURVIVAL ADVANTAGE.
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- 28. Largest Retrospective Review 3650patients :US National Oncology database.
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- 32. Trials in Uterinesarcoma with RT
- 33. CARCINO SARCOMA :Randomized Trials study stage yrs RT details N MFU OS LRec Dist met Reed et al EORTC NO RT RT 1-II N=91 1988- 2001 50.4 Gy/28 Fr 45 Vs. 46 NG NG 47% 24% 29% 35% Wolfson et al NO RT (chemo) RT I-IV N=206 1993- 2005 WA:30G y,pelvis: 49.8 101 Vs. 105 5.3 yrs. 45% 35% 24% 17% 53% 56%
- 34. study Arm s Stage yrs.N RT MFU yrs. Loco Reg. Distant mets. OS Sampath et al No RT RT I - IV 1980- 2005 638 490 EBRT +/- Brach y 5 20% Vs. 10% NA Smith SEER No Vs. RT I - IV 1973- 2003 1571 890 varyin g 3.9 33% 42% Gerszten et al No RT I-III 1977- 92 31 29 45- 50G 2.7 yrs. 55% 3% 53% 83% At 3yrs CARCINOSARCOMA -Non randomized Evidence for OS
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- 38. Role of RTESS Post op Adjuvant. No level I evidence :retrospective data better LC Medically inoperable. Palliative :stage IV, or recurrence NCCN Category 2B and ESMO guidelines suggest Adj RT in Stage II- IVA.
- 39. RADIATION IN LMS STAGE I-IVA :Improves LRC: Survival (No level I evidence ). No improvement in survival probably because of the high and early metastatic potential. Other indications :medically inoperable Palliative /recurrent cases :to reduce bleeding ,pain .
- 40. CARCINOSARCOMA Managed similarto endometrial high grade carcinoma Multi institutional retrospective reviews favour Chemo and RT ,with sandwitch regimen being slightly better. With chemotherapy taxol+ carbo 6 cycles and adjuvant RT. Level I data for adjuvant RT. Chemo EBRT vs. Chemo brachy has to be
- 41. Chemotherapy The roleof chemotherapy as an adjuvant is at best controversial. None of the published literature have shown any statistically significant benefit as regards DFS or OS in adjuvant setting, except the SARC GYN study, Most of the trials are underpwered.
- 42. Chemotherapy in LMS No randomized trial has shown a significant survival advantage. SARC GYN study – DFS improved with adjuvant chemo+RT compared to RT alone. Hensley et al reported a 2 yr PFS of 59% in stage I-IV 78% in stage I,II patients.( with Gem-doce followed by doxo ) GOG 277 – ongoing adjuvant chemo study (Gem +Doce x4 -Doxo x 4)
- 43. LMS -Chemo In recurrent/metastatic setting : PFS improvement with single agent/combination chemo . Agents being tried are Ifosfamide,platinum,gemcitabine ,doxorubicin Second line agents :Trabactedin with or without chemo /Pazopanib/bevacizumab /mTOR inhibitors.
- 44. HGUS :Chemo Highchance of local and systemic failure. In SARC GYN phase III : adjuvant pelvic RT vs Chemo (doxo,ifos,cis)-RT (9 cases were HGUS.) 3yr DFS 41% vs 55%(p=0.048) Data suggests the use of chemo followed by RT in high grade uterine sarcomas . Standard recommendation not possible. Participation in multicentre clinical trials is recommended.
- 45. Carcinosarcoma Retrospective reportsfavour sequential chemotherapy and RT in adjuvant setting (Menczer et al ,Wong et al) Managed similar to high grade endometrial cancer .
- 46. Hormones – Lowgrade ESS Progestins are preferred Aromatase inhibitors also show promise, in ESS.(ORR of 67%) Duration : Adjuvant setting 2yrs to 5 yrs and in recurrent/metastatic setting until progression. Chemotherapy is of limited use in low grade ESS.
- 47. ESS LOW GRADENCCN 2015
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- 49. CARCINOSARCOMA –NCCN 2015 Carcinosarcoma ,Clearcell ,papillary
- 50. RECURRENCE/METS Locoregional Rec:Resection : if feasible especially in ESS . Metastatectomy :for oligo mets :developing after good DFS, if R0 feasible. Palliative chemo ,agents tried are Doxorubicin ,Gemcitabine ,taxanes ,Ifosfamide Targeted agents as second line like Pazopanib ,bevacizumab ,mTOR and multikinase inhibitors with or without chemo. Best approach is participation in multicentre trials.
- 51. PRACTICE POINTS Benefitof adjuvant RT is limited to improved LRC. LRC with Radiation is not translated in to survival . Ongoing area of research is effective chemo regimens /targeted agents to control metastasis and to improve survival.
- 52. FUTURE Multi institutionalstudies IRC :International Rare Cancer Initiative : Exploring the role of CTRT vs. Adjuvant chemo /targeted treatments in each subgroup is warranted. Comparison of EBRT vs. brachytherapy with newer effective systemic treatment in each subgroup.
Editor's Notes
- #2 Uterine sarcomas are large fleshy tumours .