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