Therapeutic Management of Rare Primary Ovarian Neoplasms: Carcinosarcoma, Leiomyosarcoma, Melanoma and Carcinoid

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.

Neoadjuvant chemotherapy in advanced epithelial ovarian cancer by histology: A SEER based survival analysis

“…[36][37][38][39] Primary ovarian carcinosarcomas are highly aggressive tumors with both carcinomatous and sarcomatous components that usually diagnosed at advanced stage (75%-80%). [40,41] Primary treatment guidelines for this subtype extrapolated from HGSC has traditionally been PDS followed by chemotherapy and/or radiotherapy. [42] No current evidence to guide clinical practice regards to NACT.…”

Intra-Abdominal Malignant Melanoma: Challenging Aspects of Epidemiology, Clinical and Paraclinical Diagnosis and Optimal Treatment—A Literature Review

“…In research published in 2021 [ 23 ], the significance of establishing an international database of rare ovarian tumors was underlined, as it would enable the collection of data from various oncological centers and further study of these tumors.…”

Histological Origin and Clinicopathological Analysis of Primary Ovarian Neuroendocrine Neoplasms

“…Although the pathological features of ovarian NENs are obvious, the preoperative clinical, imaging, and laboratory features are not speci c. Ovarian carcinoid tumors are small in size, with limited lesions and no clear necrosis or invasion. Imaging and tumor markers provide no clear suggestive effect, and the majority of the tumors are found unintentionally [12]. Even pathological examination may ignore very small lesions, and only one-third of ovarian insular carcinoid cancers are associated with carcinoid syndrome [13,14].…”

“…The clinical features and prognosis of ovarian NENs are related to pathological types. Ovarian carcinoids, island carcinoids, beam carcinoids, and thyroid-type carcinoids are usually small, rarely associated with metastasis, augur an acceptable prognosis [12], and the 10-year survival rate of stage I patients after surgery nears 100% [17]. Mucinous carcinoids may be a companion component of well-differentiated ovarian cancer, they and can be associated with metastasis and advanced tumor stage [18,19].…”