Dataset Information

The systemic treatment of uterine leiomyosarcomas: A systematic review. No news is good news?

ABSTRACT:

Background

Uterine leiomyosarcomas are rare malignant mesenchymal tumors. The systemic treatment of these tumors includes chemotherapy and radiotherapy. However, there are still a lot of unanswered questions regarding the ideal therapeutic approach.

Methods

We have conducted a systematic review of the treatment strategies of uterine leiomyosarcomas for the last ten years.

Results

Adjuvant chemotherapy is still a matter of dilemma. Doxorubicin based chemotherapy or the combination of Gemcitabine-Docetaxel are the regimens of choice for the first line setting. Beyond the first line, there are several options;, including chemotherapy, targeted therapy, and recently efforts of introducing immunotherapy to the therapeutic armamentarium of clinicians treating uterine leiomyosarcomas.

Conclusions

Despite the efforts of the clinicians dealing with uterine leiomyosarcomas, the optimal therapeutic algorithm is yet to be described.

SUBMITTER: Kyriazoglou A

PROVIDER: S-EPMC8021365 | biostudies-literature | 2021 Apr

REPOSITORIES: biostudies-literature

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Publications

The systemic treatment of uterine leiomyosarcomas: A systematic review. No news is good news?

Kyriazoglou Anastasios A Liontos Michalis M Ntanasis-Stathopoulos Ioannis I Gavriatopoulou Maria M

Medicine 20210401 13

<h4>Background</h4>Uterine leiomyosarcomas are rare malignant mesenchymal tumors. The systemic treatment of these tumors includes chemotherapy and radiotherapy. However, there are still a lot of unanswered questions regarding the ideal therapeutic approach.<h4>Methods</h4>We have conducted a systematic review of the treatment strategies of uterine leiomyosarcomas for the last ten years.<h4>Results</h4>Adjuvant chemotherapy is still a matter of dilemma. Doxorubicin based chemotherapy or the combi ...[more]

PMID: 33787622

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Project description:BackgroundUterine leiomyosarcoma (uLMS) responds poorly to conventional chemotherapeutic agents, and personalized therapies have yet to be systematically explored. Comprehensive genomic profiling (CGP) can identify therapeutic targets and provide insight into the biology of this highly aggressive tumor. We report a case of uLMS treated with the CGP-matched therapy palbociclib, a CDK4/6 inhibitor, with sustained clinical benefit in this rare and deadly malignancy.Materials and methodsThis study analyzed 279 clinically advanced/recurrent uLMS samples. Median patient age was 54 years (range, 23-83 years). DNA was extracted from 40 µm of formalin-fixed, paraffin-embedded sections, and CGP was performed on hybridization-captured, adaptor ligation-based libraries for up to 405 cancer-related genes plus introns from up to 31 genes frequently rearranged in cancer. Sequencing data were analyzed for base pair substitutions, insertions/deletions, copy number alterations, and rearrangements.ResultsCGP shows that 97.1% of uLMS harbor at least one alteration, and approximately 57% harbor alterations in one or more therapeutically targetable pathways. CDKN2A mutations that inactivate p16INK4a were identified in 11% of uLMS. We report the first demonstration of clinical benefit in response to palbociclib treatment for a uLMS patient with a CDKN2A mutation, resulting in disease stabilization and significant symptom reduction.ConclusionA patient with uLMS harboring a CDKN2A mutation experienced clinical benefit from treatment with palbociclib, and genomic analysis of 279 uLMS samples revealed that 19% of patients had mutations affecting the cyclin-dependent kinase (CDK) pathway. These observations provide a rationale for a clinical trial investigating treatment with CDK pathway inhibitors for uLMS harboring relevant genomic alterations. The Oncologist 2017;22:416-421Implications for Practice: Comprehensive genomic profiling (CGP) of individuals with uterine leiomyosarcoma (uLMS) indicates that nearly 20% of patients may harbor a mutation affecting the cyclin-dependent kinase (CDK) pathway. The case presented demonstrates that a CDK inhibitory drug may provide clinical benefit to such individuals. Given the lack of curative therapies for uLMS, CGP could be performed on all cases of advanced uLMS and a CDK inhibitor could be recommended (preferably as part of a clinical trial) for individuals harboring a mutation in the CDK pathway.

Dataset Information

The systemic treatment of uterine leiomyosarcomas: A systematic review. No news is good news?

Background

Methods

Results

Conclusions

Publications

The systemic treatment of uterine leiomyosarcomas: A systematic review. No news is good news?

Similar Datasets

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