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Chapter 36 - Uterine Sarcoma and Rare Uterine Malignant Disease

from Section 8 - Uterus

Published online by Cambridge University Press:  24 November 2021

By
Edward Wight
Edited by
Tahir Mahmood ,
Charles Savona-Ventura ,
Ioannis Messinis  and
Sambit Mukhopadhyay
Tahir Mahmood
Affiliation:
Victoria Hospital, Kirkcaldy
Charles Savona-Ventura
Affiliation:
University of Malta, Malta
Ioannis Messinis
Affiliation:
University of Thessaly, Greece
Sambit Mukhopadhyay
Affiliation:
Norfolk & Norwich University Hospital, UK
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Summary

Uterine sarcomas are rare tumours with high recurrence rate and poor prognosis. Only low-grade endometrial stromal sarcomas and adenosarcoma without sarcomatous overgrowth occasionally show an indolent growth, but late recurrences occur even in early-stage disease. Total hysterectomy is the cornerstone of treatment. Tumour-free resection margins after primary surgery and prevention of intra-abdominal spillage of malignant tissue during the intervention are of utmost importance. Removal of the ovaries and retroperitoneal lymph node dissection remain controversial, but oophorectomy is recommended in postmenopausal women and in patients with hormone-sensitive disease. Influence of adjuvant radiotherapy and adjuvant systemic therapy on survival is uncertain, but may be indicated in cases with high risk of recurrence. Palliative systemic therapies comprise doxorubicin alone or in combination with gemcitabine or olaratumab and aromatase inhibitors in hormone-sensitive disease. Encouraging new therapeutic options are beginning to emerge with targeted therapies.

Keywords

uterussarcomacancermalignanttumours
Type
Chapter
Information
The EBCOG Postgraduate Textbook of Obstetrics & Gynaecology
Gynaecology
 , pp. 305 - 316
Publisher: Cambridge University Press
Print publication year: 2021

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References

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