Dataset Information

Primary neuroendocrine tumors of the ovary: Management and outcomes.

ABSTRACT:

Background

There is currently no recognized first-line treatment strategy for ovarian neuroendocrine tumors (NETs). Furthermore, because of the low incidence of ovarian NETs, no studies have reported prognostic statistics derived from large samples. This retrospective study aimed to investigate the clinical behavior of ovarian NETs.

Methods

The Surveillance, Epidemiology, and End Results database was used to identify women diagnosed with ovarian NETs from 2004 to 2015. Overall survival (OS), cancer-specific survival (CSS), and independent prognostic factors for ovarian NETs were evaluated. The effects of different treatments on prognosis were also compared, as were OS and CSS rates for histological subtypes.

Results

The 5-year OS rates were 83.3%, 30.0%, 20.3%, and 9.8% for patients in stages I (n = 159), II (n = 23), III (n = 101), and IV (n = 148), respectively. The 5-year CSS rates were 85.6%, 41.7%, 21.2%, and 9.8% for patients in stages I-IV, respectively. Age, American Joint Committee on Cancer (AJCC) stage, lymph node metastasis, treatment, and histological type were related to poor OS and CSS. In the early stage, the 5-year OS and CSS rates were 97.03% and 96.90%, respectively. For patients in the advanced stage receiving comprehensive treatment (surgery + chemotherapy + radiotherapy), the 5-year OS and CSS rates were 72.9% and 70.00%, respectively. When comparing low- and high-grade neuroendocrine carcinoma, 5-year OS rates were 93.96% and 7.01%, 5-year CSS rates were 97.44% and 7.31%, 10-year OS rates were 93.56% and 2.34%, and 10-year CSS rates were 97.44% and 4.88%, respectively.

Conclusion

Age, AJCC stage, treatment, and histological type are independent prognostic factors of ovarian NETs. OS and CSS are relatively good for early-stage cases treated with surgery alone, whereas more comprehensive treatment is required to improve OS and CSS in the advanced stage.

SUBMITTER: Pang L

PROVIDER: S-EPMC8633223 | biostudies-literature |

REPOSITORIES: biostudies-literature

ACCESS DATA

Similar Datasets

Project description:ImportanceEctopic adrenocorticotropic hormone secretion from lung tumors causing Cushing syndrome are associated with high rates of morbidity. Optimal management remains obscure because knowledge is based on rare reports with few patients.ObjectiveTo characterize the outcomes of lung neuroendocrine tumors associated with Cushing syndrome.Design, setting, and participantsAn observational case series review from 1982 to 2020 was conducted in a single institution referral center. Kaplan-Meier analysis estimated disease-free survival (DFS). Participants underwent curative-intent surgery for a lung neuroendocrine tumor causing Cushing syndrome.ExposuresLobectomy or pneumonectomy vs sublobar resection.Main outcomes and measuresDisease-free survival, disease persistence/recurrence.ResultsOf the 68 patients, the median age was 41 years (range, 17-80 years), 42.6% (29 of 68) were male, 81.8% (54 of 66) were White, with a mean follow-up after surgery was 16 months (range, 0.1-341 months). Lobectomy was the most common procedure (48 of 68 [70.6%]), followed by wedge resection (16 of 68 [23.5%]) and segmentectomy (3 of 68 [4.4%]). Video-assisted thoracoscopic surgery was performed in 19 of 68 (27.9%) of patients. Surgical morbidity was 19.1% (13 of 68), and perioperative mortality was 1.5% (1 of 68). Lymph node positivity was 37% (22 of 59) when evaluable. The overall incidence of persistence/recurrence was 16.2% (11 of 68) with a median time to recurrence of 55 months (range, 18-152 months). The median DFS was reached in 12.7 years (0.1-334 months). There were no statistical differences in DFS based on tumor size, stage (8th edition TNM), whether full systematic lymphadenectomy was performed or not, nodal status, or surgical approach.Conclusions and relevanceIn this case series study, neuroendocrine pulmonary tumors associated with Cushing syndrome had increased nodal metastasis, higher recurrence, and lower DFS than quiescent bronchopulmonary carcinoid tumors, but many patients experienced favorable outcomes. This observation is underscored by the discordance of TNM-stage classifications vs prognosis. Observing no difference in surgical techniques, the implication may be that a lung-sparing approach could suffice. These results may reflect the intrinsic importance of the hormone physiology instead of the carcinoid biologic factors.

Project description:IntroductionRecent classification of neuroendocrine neoplasms has defined well-differentiated high-grade neuroendocrine tumors (NET G3) as a distinct entity from poorly differentiated neuroendocrine carcinoma. The optimal treatment for NET G3 has not been well-described. This study aimed to evaluate metastatic NET G3 response to different treatment regimens.Materials and methodsThis was a retrospective study of patients with NET G3 within the Mayo Clinic database. Patients' demographics along with treatment characteristics, responses, and survival were assessed. Primary endpoints were progression-free survival (PFS) and overall survival. Secondary endpoints were objective response rate (ORR) and disease control rate (DCR).ResultsTreatment data was available in 30 patients with median age of 59.5 years at diagnosis. The primary tumor was mostly pancreatic (73.3%). Ki-67 index was ≥55% in 26.7% of cases. Treatments included capecitabine + temozolomide (CAPTEM) (n = 20), lutetium 177 DOTATATE (PRRT; n = 10), Platinum-etoposide (EP; n = 8), FOLFOX (n = 7), and everolimus (n = 2). CAPTEM exhibited ORR 35%, DCR 65%, and median PFS 9.4 months (95% confidence interval, 2.96-16.07). Both EP and FOLFOX showed similar radiographic response rates with ORR 25.0% and 28.6%; however, median PFS durations were quite distinct at 2.94 and 13.04 months, respectively. PRRT had ORR of 20%, DCR of 70%, and median PFS of 9.13 months.ConclusionAmong patients with NET G3, CAPTEM was the most commonly used treatment with clinically meaningful efficacy and disease control. FOLFOX or PRRT are other potentially active treatment options. EP has some activity in NET G3, but responses appear to be short-lived. Prospective studies evaluating different treatments effects in patients with NET G3 are needed to determine an optimal treatment strategy.Implications for practiceHigh-grade well-differentiated neuroendocrine tumors (NET G3) are considered a different entity from low-grade NET and neuroendocrine carcinoma in terms of prognosis and management. The oral combination of capecitabine and temozolomide is considered a good option in the management of metastatic NET G3 and may be preferred. FOLFOX is another systemic option with reasonable efficacy. Similar to other well-differentiated neuroendocrine tumors, peptide receptor radionuclide therapy seems to have some efficacy in these tumors.