Clinical features of ovarian large-cell neuroendocrine carcinoma: Four case reports and review of the literature.

The objective of the present study was to present 4 recently encountered ovarian large-cell neuroendocrine carcinoma (LCNEC) cases, and to evaluate their clinicopathological features in the context of the previously reported 29 LCNEC cases. First, we described the clinical features of 4 recently encountered cases. Routine H&E staining and immunohistochemistry for CD56, synaptophysin and chromogranin A were performed on sections of both the LCNEC and epithelial carcinoma components. Clinical data for the total of 33 LCNEC cases were summarized, and the Kaplan-Meier survival curve was estimated. Our cases were observed in women aged 42-81 years. One case is clinically classified as FIGO stage IV with multiple metastases, and the others are classified as FIGO stages Ic, IIc and IIIb by post-surgical findings. Pathological features, assessed by H&E staining, were similar to lung LCNEC, and at least one neuroendocrine marker was positive staining in both LCNEC and the epithelial component. One case was pure type LCNEC and the others were mixed carcinoma. Paclitaxel/carboplatin chemotherapy was performed for all cases and 3 of the 4 treatments were effective. The prognoses of our cases were as follows: 1 in stage Ic died from the disease after only 2 months, but the others survived, with or without recurrence, for 32-64 months, whereas the total 5-year survival of the 33 LCNEC cases was 34.9%. In summary, our 3 LCNEC cases revealed ordinary chemo-sensitivity, resulting in a better prognosis than those previously described, apart from 1 case which exhibited aggressive behavior. For the future, a retrospective survey to elucidate the prognostic factors and prospective clinical studies to evaluate the efficacy of treatment modalities of ovarian LCNEC are necessary, particularly for aggressive LCNEC cases.