UTERINE SARCOMA - European Society for Medical Oncology · • Uterine sarcomas are malignant...
Transcript of UTERINE SARCOMA - European Society for Medical Oncology · • Uterine sarcomas are malignant...
UTERINE SARCOMAESMO 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
• Mrs AB, 57yrs
• Symptoms:
Post menopausal
bleeding refractory to
cyclokapron with
associated lower
abdominal pain for 4/12.
Past medical history:
HIV -ve, Asthmatic on
treatment.
• Gynae history: P3G3, menarche 16yrs, menopause 46yrs, no previous pap smears.
• Family history: nil
• Past surgical history: BTL 40yrs
• Social history: smokes 4 cigarettes per day, no alcohol history, good family support.
Clinical examination
Pre-op examination:
ECOG PS1Unremarkable general examinationThyroid and breast examination normalChest: clear breath soundsAbdomen: soft, 14/40 weeks size uterus, no adnexal masses.Vulva and perineum normalCervix normal with PVBPR: 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 involvedNo 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.
THANK YOU!
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