Primary Ovarian Leiomyosarcoma Is a Very Rare Entity: A Narrative Review of the Literature.

Cancers (Basel)

Unit of Obstetrics and Oncological Gynecology, Azienda USL-IRCCS di, 42122 Reggio Emilia, Italy.

Published: May 2023

AI Article Synopsis

  • * Out of 113 cases, the majority underwent surgical resection; about 40% received chemotherapy, with lymphadenectomy linked to better survival outcomes.
  • * Findings indicate that patients typically present in their 50s, with early-stage cases; however, factors like advanced stage and higher mitotic counts negatively impact survival, suggesting the need for a standardized international registry for better data on diagnosis and treatment.

Article Abstract

Background: Primary ovarian leiomyosarcoma is a very rare malignancy characterized by unclear management and poor survival. We reviewed all the cases of primary ovarian leiomyosarcoma to identify prognostic factors and the best treatment.

Methods: We collected and analyzed the articles published in the English literature regarding primary ovarian leiomyosarcoma from January 1951 to September 2022, using PubMed research. Clinical and pathological characteristics, different treatments and outcomes were analyzed.

Results: 113 cases of primary ovarian leiomyosarcoma were included. Most patients received surgical resection, associated with lymphadenectomy in 12.5% of cases. About 40% of patients received chemotherapy. Follow-up information was available for 100/113 (88.5%) patients. Stage and mitotic count were confirmed to affect survival, and lymphadenectomy and chemotherapy were associated with a better survival rate. A total of 43.4% of patients relapsed, and their mean disease-free survival was 12.5 months.

Conclusions: Primary ovarian leiomyosarcomas are more common in women in their 50s (mean age 53 years). Most of them are at an early stage at presentation. Advanced stage and mitotic count showed a detrimental effect on survival. Surgical excision associated with lymphadenectomy and chemotherapy are associated with increased survival. An international registry could help collect clear and reliable data to standardize the diagnosis and treatment.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10252074PMC
http://dx.doi.org/10.3390/cancers15112953DOI Listing

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