Abstract
Carcinosarcoma, leiomyosarcoma, melanoma and carcinoid as primary tumors in the ovary are extremely rare. In this paper, the authors reviewed the literature from 2010 to 2021, based on specific criteria, to analyze the treatment of these rare ovarian neoplasms. We also aimed to verify whether modern therapies have been found in recent years. For this article, 80 papers were finally selected. The vast majority of the articles were clinical case reports. Despite single mentions of new potential pharmacological treatments, surgery (radical or fertility-sparing) is definitely the mainstay of treatment. There are currently no treatment guidelines for these tumors. A review of the literature has revealed the use of various adjuvant treatments. We, therefore, believe that a more detailed understanding of the biology of these tumors is necessary in order to find new target points for treatment. We would like to emphasize the importance of creating an international database of rare ovarian tumors which would make it possible to gather data from various oncological centers and enable further research into these neoplasms.
Highlights
The most common malignancy of the ovary is high grade serous carcinoma
As can be observed over the past several years, bevacizumab and poly(ADP-ribose) polymerase (PARP) inhibitors have been included in the treatment of selected groups of patients
Carcinosarcoma of the ovary is a very rare neoplasm accounting for 1–4% of cancers of this organ [1,2,3]
Summary
The most common malignancy of the ovary is high grade serous carcinoma. Due to its relatively high prevalence and low cure rate, ovarian cancer is a target for research, allowing the introduction of new drugs or increasing the number of indications of currently used drugs. As can be observed over the past several years, bevacizumab and poly(ADP-ribose) polymerase (PARP) inhibitors have been included in the treatment of selected groups of patients. Such progress in treatment is not observed in rare primary ovarian neoplasms. Primary carcinosarcoma (PCSO), leiomyosarcoma (PLSO), melanoma (POM) and carcinoid (PCO) of the ovary, each rare, together form a group of much more common neoplasms. These malignancies occur mainly in peri- and postmenopausal women.
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