UTERINE SARCOMA ESMO Summit Africa 2018 Dr Thobile Goba-Mjwara Prof Hannah Simonds
I have nothing to declare.
Introduction • Uterine sarcomas are malignant mesenchymal tumours that include leiomyosarcoma (LMS), endometrial stromal sarcomas (ESS)and undifferentiated uterine sarcomas (UUS). • Accounts for 3-9% of all uterine malignant neoplasms. • Compared to the more common endometrial carcinomas, uterine sarcomas, particularly LMS, behave aggressively and are associated with a poorer prognosis.
Classification of uterine sarcomas 1. Stromal: -ESS -LMS -UUS 2. Mixed epithelial-stromal neoplasms: -Adenosarcomas are considered as sarcomas. -Uterine Carcinosarcoma is now classified as a carcinoma rather than a sarcomas though they have stromal differentiation.
Case presentation • Gynae history : P3G3, • Mrs AB, 57yrs menarche 16yrs, • Symptoms : menopause 46yrs, no Post menopausal previous pap smears. • Family history : nil bleeding refractory to • Past surgical history : cyclokapron with BTL 40yrs associated lower • Social history : smokes abdominal pain for 4/12. 4 cigarettes per day, no Past medical history : alcohol history, good HIV -ve, Asthmatic on family support. treatment.
Clinical examination Pre-op examination : ECOG PS1 Unremarkable general examination Thyroid and breast examination normal Chest: clear breath sounds Abdomen: soft, 14/40 weeks size uterus, no adnexal masses. Vulva and perineum normal Cervix normal with PVB PR: normal rectal mucosa, no masses palpated.
Investigations Pipelle biopsy 30/10/17: IHC profile compatible with part of a carcinosarcoma. Positive p16 & p57 favours a serous epithelial component while the focal myogenin positivity confirms heterologous rhabdomyoplastic differentiation in the sarcomatous component . Labs 21/11/17: Normal FBC,U&E and LFT LDH 259, AFP 2.9, BHCG 2 , CEA 5.3 CXR 20/11/17: Normal, no metastasis noted. Abdominal U/S 21/11/17: Normal liver and kidneys, no metastasis noted. Pelvic U/S 15/11/17- Enlarged uterus with a heterogeneous compressible structure of 64x51x69mm in size. Ovaries not visualized.
Histology TAH, BSO, PLND+Omentectomy 22/12/2017: Carcinosarcoma with heterologous differentiation (Rhabdoid), High grade. 80% sarcoma: 20% carcinoma >50% myometrium involved LVI present Cervix, adnexa, omentum not involved No nodes involved- 0/31
Questions 1. Does the percentage of sarcomatous elements influence treatment? 2. Is there a role for chemotherapy in view of the histology of the “Rhabdoid” heterologous differentiation , 80% sarcoma: 20% carcinoma ? 3. If the histology showed 100% rhabdomyosarcoma would the patient be treated as a sarcoma? 4. Does the panel agree that the best adjuvant treatment for this patient – Stage Ib G3 – is pelvic radiotherapy alone?
Planned Management In keeping with Stage pIbG3 pn0 Carcinosarcoma. Discussed at the MDT – treat as an epithelial endometrial carcinoma. For adjuvant whole pelvic radiotherapy as per preliminary results of PORTEC 3.
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Radiotherapy planning • Technique: 3D CRT • Data acquisition: CT scan 3mm slices L1-5cm below the vaginal introitus with IV contrast. • Modality: 10MV • Position: supine, arms on the side • Immobilization: neck rest, knee and ankle stocks. • Anterior and lateral tattoos marked with radio-opaque material aligned with lasers to prevent lateral rotation. • Bladder protocol used to maintain a constant bladder filling. • Prescription: 40Gy/20#, 5 days a week.
Plan evaluation
References 1. NCCN Guideline Uterine neoplasms - http://www.nccn.org/professionals/physician_gls/f_guidelines. 2. Uptodate 3. Practical Radiotherapy Planning (4th edition) – Barrett, Dobbs, Morris & Roques
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