Primary Ovarian Leiomyosarcoma Is a Very Rare Entity: A Narrative Review of the Literature
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...
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| Format: | Article |
| Language: | English |
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MDPI AG
2023-05-01
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| Series: | Cancers |
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| Online Access: | https://www.mdpi.com/2072-6694/15/11/2953 |
| _version_ | 1797597816319639552 |
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| author | Vincenzo Dario Mandato Federica Torricelli Valentina Mastrofilippo Andrea Palicelli Luigi Costagliola Lorenzo Aguzzoli |
| author_facet | Vincenzo Dario Mandato Federica Torricelli Valentina Mastrofilippo Andrea Palicelli Luigi Costagliola Lorenzo Aguzzoli |
| author_sort | Vincenzo Dario Mandato |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-11T03:10:48Z |
| format | Article |
| id | doaj.art-ac00826be6d54a45afa48b4c38250a5d |
| institution | Directory Open Access Journal |
| issn | 2072-6694 |
| language | English |
| last_indexed | 2024-03-11T03:10:48Z |
| publishDate | 2023-05-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cancers |
| spelling | doaj.art-ac00826be6d54a45afa48b4c38250a5d2023-11-18T07:38:46ZengMDPI AGCancers2072-66942023-05-011511295310.3390/cancers15112953Primary Ovarian Leiomyosarcoma Is a Very Rare Entity: A Narrative Review of the LiteratureVincenzo Dario Mandato0Federica Torricelli1Valentina Mastrofilippo2Andrea Palicelli3Luigi Costagliola4Lorenzo Aguzzoli5Unit of Obstetrics and Oncological Gynecology, Azienda USL-IRCCS di, 42122 Reggio Emilia, ItalyLaboratory of Translational Research, Azienda USL-IRCCS di, 42122 Reggio Emilia, ItalyUnit of Obstetrics and Oncological Gynecology, Azienda USL-IRCCS di, 42122 Reggio Emilia, ItalyUnit of Pathology, Azienda USL-IRCCS di, 42122 Reggio Emilia, ItalyUnit of Obstetrics and Gynecology, Santa Maria delle Grazie Hospital, 80100 Naples, ItalyUnit of Obstetrics and Oncological Gynecology, Azienda USL-IRCCS di, 42122 Reggio Emilia, ItalyBackground: 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.https://www.mdpi.com/2072-6694/15/11/2953primary ovarian leiomyosarcomasymptomsstagemitotic countlymphadenectomychemotherapy |
| spellingShingle | Vincenzo Dario Mandato Federica Torricelli Valentina Mastrofilippo Andrea Palicelli Luigi Costagliola Lorenzo Aguzzoli Primary Ovarian Leiomyosarcoma Is a Very Rare Entity: A Narrative Review of the Literature Cancers primary ovarian leiomyosarcoma symptoms stage mitotic count lymphadenectomy chemotherapy |
| title | Primary Ovarian Leiomyosarcoma Is a Very Rare Entity: A Narrative Review of the Literature |
| title_full | Primary Ovarian Leiomyosarcoma Is a Very Rare Entity: A Narrative Review of the Literature |
| title_fullStr | Primary Ovarian Leiomyosarcoma Is a Very Rare Entity: A Narrative Review of the Literature |
| title_full_unstemmed | Primary Ovarian Leiomyosarcoma Is a Very Rare Entity: A Narrative Review of the Literature |
| title_short | Primary Ovarian Leiomyosarcoma Is a Very Rare Entity: A Narrative Review of the Literature |
| title_sort | primary ovarian leiomyosarcoma is a very rare entity a narrative review of the literature |
| topic | primary ovarian leiomyosarcoma symptoms stage mitotic count lymphadenectomy chemotherapy |
| url | https://www.mdpi.com/2072-6694/15/11/2953 |
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