Primary pure large cell neuroendocrine carcinoma of the ovary

Abstract

Neuroendocrine tumors are more common in the female genital tract than in the male genital tract, and most are uterine small cell carcinomas or ovarian carcinoid tumors. Most ovarian tumors are associated with surface epithelial tumors, SertolieLeydig cell tumors, teratoma, small cell carcinoma, pulmonary-type and undifferentiated carcinoma, and nonsmall cell lung cancer [1]. Large cell neuroendocrine carcinoma (LCNEC) of the ovary is unusual and defined as an extremely malignant tumor with an aggressive lethal outcome. For this original and updated review, we searched the databases of PubMed Clinical Queries, MEDLINE, Cochrane Library and UpToDate. We also hand-searched relevant journals and reference lists of identified articles. The keywords used were “ovary”, “pure LCNEC”, and “immunohistochemistry”. We found that only 37 cases of ovarian tumor involving LCNEC associated with surface epithelial stromal tumors and/or teratoma have been reported to date [2e5]. In addition, seven cases of primary LCNEC of the ovary without any associated component have been described [2,3,6e8]. Herein, we present the case of a patient with stage IV primary pure LCNEC of the ovary with liver metastasis and carcinomatosis. A 50-year-old married, Taiwanese female, G3P3, was referred by a clinical oncologist for the evaluation of a pelvic mass. She denied specific systemic disease, a gynecological history, and undergoing major operations previously. She also denied any significant symptoms, such as compression sensation, lower abdominal pain, or abnormal vaginal bleeding. Because of an increased abdominal circumference and a palpable 20-cmmass in the abdomen that had been present for 2 weeks, she was referred by a clinical oncologist and underwent a suboptimal debulking operation with total abdominal hysterectomy, bilateral salpingo-oophorectomy, partial