A rare tumor of the uterus, sarcoma is discoursed in this presentation

1. Uterine Sarcoma
Dr Ibanda Hood
Dr Ongala Emmanuel
Tutor: Dr Nakalembe

2. • Uterine sarcoma refers to soft tissue tumors of the
uterus and the tumors are of mesenchymal origin.
• The tumors are very rare

3. Epidemiology
• Tumors are rare; 3-7% of uterine malignancies are uterine sarcoma.
• Behavior(severity) depends on tumor location, histological subtype,
and tumor grade.
• The Cause of uterine sarcoma is not clear.
• More in Black women (ratio >2).
• Incidence age is at least 50 years.

4. Clinical presentation
The symptoms of uterine sarcoma are vague/not specific. They include:
• Abnormal Vaginal bleeding
• Lower abdominal or pelvis pain
• Abdominal distension.
A Fibroid increasing in size in a postmenopausal woman should be
investigated as a likely case of Uterine sarcoma. 52.4% tumors removed
at surgery will not appear malignant.

5. Investigation
Diagnostic Challenges
• Most commonly used imaging (Ultrasound, MRI) can not differentiate
between uterine sarcoma and uterine fibroid
• MRI is better but it’s neither sensitive nor specific. It’s also too
expensive to be used for screening
• No tumor markers available for detecting uterine sarcoma
Histopathology gives the definitive diagnosis but it is done
postoperatively.

6. Histological sub-types of uterine Sarcoma
• Uterine Leiomyosarcoma (ULMS)
• Low grade endometrial stromal sarcoma (ESS)
• High grade endometrial stromal Tumor (HG-ESS)
• Undifferentiated Uterine Sarcoma
• Adenosarcoma.
• Rhabdomyosarcoma
NB: Uterine sarcoma can be also classified as Non-epithelia, Mixed
non-epithelia/Epithelial.

7. Figo staging of Uterine Sarcoma(ULMS & ESS)
STAGE DEFINITION
I Tumor limited to Uterus
1A
1B
Tumor than less/equal to 5cm
More than 5cm
II Tumor extends beyond the uterus, but it is within the pelvis
2A
2B
Adnexal involvement
Extrauterine pelvic tissue involved
III
3A
3B
3C
Tumor invades abdominal tissue(not just protruding in abdomen)
1 sites
> 1 site
Metastasis to pelvic or paraortic lymphnodes
IV
4A
4B
Tumor invades Bladder or Rectum
Distant Metastasis

8. FIGO staging of uterine sarcoma(Adenosarcoma)
STAGE DEFINITION
I
1a
1b
1c
Tumor limited to uterus
-limited to endometrium/endocervix no myometrial invasion
-Less than or equal to half of myometrium invasion
-More than half myometrial invasion
II
2a
2b
Tumor Extends to Pelvis
-Adnexal involvement
-Tumor extends to Extrauterine pelvic tissues
III
3a
3b
3c
Tumor invades abdominal tissues (not just protruding into the abdomen)
-1 site
-More than 1 site
-Metastasis to pelvic/para-aortic lymph nodes
IV
4a
4b
-Tumor invades bladder and/or Rectum
-Distant Metastasis

9. Management of uterine Sarcoma
• Early stage disease: TAH + BSO are the standard care. Debulking of
the tumor is also done if the tumor extends beyond uterus. Surgery
must be done well, but do risk stratification before choosing surgical
approaches like laparoscopy with morcellation that may lead to
dissemination of tumor. This can occur if we think the tumor is fibroid
• Follow surgery by reviewing operative findings, histology reports,
endocrine status and other results to aid in staging and determine
need for any other therapy.

10. Management of Advanced Uterine sarcoma
• Surgery is the best treatment. Other therapies are generally not effective.
• Chemotherapy: Choice of chemotherapy is guided by histology. Docetaxel/gemcitabine,
doxorubicin, and ifosfamide are all reasonable options for advanced or recurrent disease
with response rates ranging from 17% to 36%.
• In Carcinosarcoma, chemo is used since the tumor is very aggressive.
• Response to other treatment than Surgery is very poor and not in common use.
• Other treatment to consider include:
• Radiotherapy
• Judicious use of surgery
• Interventional radiology techniques like embolization
• Ablative treatment
• Immunotherapy
• Palliation
NB: patient-centered care is key, and we better have a multidisciplinary team.

11. prognosis
• Poor prognosis
• Tumor stage is the most important prognostic factor
• 56-71% recurrence rates; 40% recur in the lungs

12. References
Benson, C. & Miah, A. B. (2017). Uterine sarcoma: current perspective.
International journal of women’s health, 7, 597-606
Mbatani, N., Olawaiye, A.B., & Prat, J. (2018). Uterine sarcomas.
International journal of obstetrics & gynecology; I43, S2 (51-58).
Juhasz-Böss. I, Gabriel,L., Bohle, R.M., Horn, L.C., Solomayer, E.F.,
& Breitbach G.P. (2018). Uterine Leiomyosarcoma. Oncology research &
treatment; 41, 11, (680-686).