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Composite Large-Cell Neuroendocrine Carcinoma and Surface Epithelial-Stromal Neoplasm of the Ovary

The clinicopathologic features of two cases of composite large-cell neuroendocrine carcinoma and surface epithelial-stromal neoplasm of the ovary are reported and those of eight previously published cases reviewed. The patients ranged in age from 22 to 77 years (mean, 56 years). The surface epithelial-stromal neoplasm was an endometrioid adenocarcinoma in one case, a mucinous cystadenoma in one case, and a mucinous adenocarcinoma in eight cases. The large-cell neuroendocrine carcinoma in these tumors may represent dedifferentiation of the neuroendocrine cells present in the surface epithelial-stromal tumor. This composite tumor type is highly aggressive. Of eight patients with follow-up information, all had died of disseminated tumor; six within 10 months, and two in 19 months and 3 years, respectively, after diagnosis. Only the neuroendocrine carcinoma component was found in the metastatic sites.