Uterine sarcoma – current perspectives

Abstract

Uterine sarcomas comprise a group of rare tumors with differing tumor biology, natural history and response to treatment. Diagnosis is often made following surgery for presumed benign disease. Currently, preoperative imaging does not reliably distinguish between benign leiomyomas and other malignant pathology. Uterine leiomyosarcoma is the most common sarcoma, but other subtypes include endometrial stromal sarcoma (low grade and high grade), undifferentiated uterine sarcoma and adenosarcoma. Clinical trials have shown no definite survival benefit of adjuvant radiotherapy or chemotherapy and have been hampered by the rarity and heterogeneity of these disease types. There is a role of adjuvant treatment in carefully selected cases following multidisciplinary discussion at sarcoma reference centers. In patients with metastatic disease, systemic chemotherapy can then be considered. There is activity of a number of agents, including doxorubicin, trabectedin, gemcitabine-based chemotherapy, eribulin and pazopanib. Patients should be considered for clinical trial entry where possible. Close international collaboration is important to allow progress in this group of diseases.

MRI screening for uterine leiomyosarcoma

“…In addition to providing guidance to clinical management, one of the goals for screening is early diagnosis to allow for early treatment and decreased morbidity and mortality. It is a difficult task in the case of uterine sarcomas since presenting clinical symptoms are vague, comprised of abdominal and/or pelvic pain, abdominal distention, and vaginal bleeding, which overlap with symptoms of benign uterine fibroids . In our study, on initial MRI three of ten patients with LMS presented with distant metastasis, which is similar to other reported data of 33% initial presentation with metastases .…”

Retrospective analysis of treatment and prognosis for uterine leiomyosarcoma: 10‐year experience of a single institute

“…Notably, patients undergoing chemotherapy experienced no survival benefit compared with patients who underwent observation only. Moreover, several reports have stated that the efficacy of ACT in this patient population could not be confirmed …”

“…Moreover, several reports have stated that the efficacy of ACT in this patient population could not be confirmed. 5,[20][21][22] In contrast, the utility of ACT has been reported by other groups.…”

“…Uterine sarcomas are classified as either nonepithelial malignancies or mixed nonepithelial/epithelial malignancies, depending on the tissue of origin. 5 Smooth muscle tumors such as ULMS are the most common type of uterine sarcoma, followed by low-grade endometrial stromal sarcoma, high-grade ESS, undifferentiated uterine sarcoma and adenosarcoma. Pathologically, ULMS are characterized by moderate to severe cytological atypia, high mitotic indices (≥10 per 10 high-power fields), and coagulative tumor cell necrosis.…”

Long‐term impact of lymphadenectomies in patients with low‐grade, early‐stage uterine endometrial stroma sarcoma

“…However, others insist early and complete surgical resection including LND for HGESS is the best treatment . Though, the standard therapeutic strategy for ESS is TH plus BSO when the tumor is confined to the uterine corpus, with a risk of LNM being 10% in the entire population of ESS cases . Based on our study, there is no significant benefit on OS and PFS with LND ( P = 0.171 and 0.892, respectively).…”