Large cell neuroendocrine carcinoma of the ovary: A pathologic entity in search of clinical identity

Large cell neuroendocrine carcinoma (LCNEC) of the ovary is a rare diagnosis and only a few dozen cases have been reported in the literature. It is characterized by large pleiomorphic cells with large round or oval nuclei, presence of mitoses and staining for neuroendocrine (NE) markers such as chromogranin A, synaptophysin, neuron specific enolase. This editorial gives a brief overview of this histologic type of ovarian carcinomas. LCNEC of the ovary is a pathologic entity that may not be diagnosed purely on clinical grounds due to the similarity of its clinical features with those of the more common epithelial ovarian cancers. Nevertheless the diagnosis is worth-making from a practical point of view in order to consider treatments tailored towards the NE component if it is dominant or it becomes dominant during the natural evolution of the disease. Establishment of an international tumor registry with an accompanying tumor tissue bank of ovarian LCNEC could be a means of obtaining further knowledge on clinical characteristics and advance research on this rare entity. This will further inform on treatment strategies and could identify future molecular treatment targets.