Abstract
Non-epithelial malignant ovarian tumors and clear cell carcinomas, Brenner tumors, transitional cell tumors, and carcinoid tumors of the ovary are rare ovarian tumors (ROTs). In this study, our aim was to determine the clinicopathological features of ROT patients and prognostic factors associated with survival. A total of 167 patients with ROT who underwent initial surgery were retrospectively analyzed. Prognostic factors that may influence the survival of patients were evaluated by univariate and multivariate analyses. Of 167 patients, 75 (44.9%) were diagnosed with germ-cell tumors (GCT) and 68 (40.7%) with sex cord-stromal tumors (SCST); the remaining 24 had other rare ovarian histologies. Significant differences were found between ROT groups with respect to age at diagnosis, tumor localization, initial surgery type, tumor size, tumor grade, and FIGO stage. Three-year progression-free survival (PFS) rates and median PFS intervals for patients with other ROT were worse than those of patients with GCT and SCST (41.8% vs 79.6% vs 77.1% and 30.2 vs 72 vs 150 months, respectively; p=0.01). Moreover, the 3-year overall survival (OS) rates and median OS times for patients with both GCT and SCST were better as compared to patients with other ROT, but these differences were not statistically significant (87.7% vs 88.8% vs 73.9% and 170 vs 122 vs 91 months, respectively; p=0.20). In the univariate analysis, tumor localization (p<0.001), FIGO stage (p<0.001), and tumor grade (p=0.04) were significant prognostic factors for PFS. For OS, the univariate analysis indicated that tumor localization (p=0.01), FIGO stage (p=0.001), and recurrence (p<0.001) were important prognostic indicators. Multivariate analysis showed that FIGO stage for PFS (p=0.001, HR: 0.11) and the presence of recurrence (p=0.02, HR: 0.54) for OS were independent prognostic factors. ROTs should be evaluated separately from epithelial ovarian cancers because of their different biological features and natural history. Due to the rarity of these tumors, determination of relevant prognostic factors as a group may help as a guide for more appropriate adjuvant or recurrent therapies for ROTs.
Highlights
Non-epithelial malignant ovarian tumors, clear cell carcinoma, Brenner tumors, transitional cell tumors, and carcinoid tumors of the ovary are defined as rare ovarian tumors (ROTs)
sex cord-stromal tumors (SCST) of the ovary are considered low grade malignancies with a relatively more favorable prognosis and are generally treated more often with repeat surgeries compared to epithelial ovarian cancers (Young, 2005; Ayhan et al, 2009)
Tumors that are mostly localized in the left ovary had germ-cell tumors (GCT) histology, but SCST and other ROTs were mostly localized in the right ovary
Summary
Non-epithelial malignant ovarian tumors (germ cell and sex cord-stromal tumors), clear cell carcinoma, Brenner tumors, transitional cell tumors, and carcinoid tumors of the ovary are defined as rare ovarian tumors (ROTs). Of these tumors, germ cell tumors (GCTs) and sex cord-stromal tumors (SCSTs) comprise approximately 15% of all ovarian malignancies, and they have a variety of histopathological subgroups (Quirk and Natarajan, 2005; Koulouris and Penson, 2009; Colombo et al, 2012). SCSTs, mostly granulosa cell tumors, are generally associated with a favorable prognosis, they are much less chemosensitive and grow much more slowly (Young, 2005; Ayhan et al, 2009) Both GCTs and SCSTs. Non-epithelial malignant ovarian tumors and clear cell carcinomas, Brenner tumors, transitional cell tumors, and carcinoid tumors of the ovary are rare ovarian tumors (ROTs). Due to the rarity of these tumors, determination of relevant prognostic factors as a group may help as a guide for more appropriate adjuvant or recurrent therapies for ROTs
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