Primary ovarian neuroendocrine neoplasia with concurrent large epithelial borderline ovarian tumor, coexistent with non-malignant pleural effusion and multiple uterine fibroids: a case report and review of the literature.

Abstract

Background: Neuroendocrine neoplasms are a group of neoplasms often originating from the neuroendocrine cells in the gastrointestinal tract, pancreas, and respiratory tract. Neuroendocrine neoplasms rarely occur in female reproductive organs and less than a hundred cases of ovarian high-grade lesions have been reported in the literature so far. Fewer still are cases reported in the literature associated with a borderline epithelial tumor in the same ovary. Owing to the rarity of the condition, there is a lack of specific guidelines for staging, and optimal management of these tumors.

Case presentation: We are reporting a case of primary ovarian neuroendocrine neoplasm in association with an epithelial borderline tumor. She is a 50-year-old Filipino woman who presented with nonspecific symptoms. Initial imaging revealed a large mass with suspicion of widespread metastasis. However, further imaging and laparotomy revealed early-stage neuroendocrine neoplasm, a large borderline epithelial tumor, with no evidence of pulmonary metastasis, despite having pleural effusion. She was lost to follow-up, presented again after a year with evidence of residual disease/metastasis, and was treated with chemotherapy.

Discussion and conclusion: The case posed significant difficulty owing to a lack of typical symptoms at presentation, nonmalignant changes in lungs in imaging, and therapeutic challenges due to the noncompliance of the patient. This report highlights the importance of considering the combination of borderline tumors of the ovary with neuroendocrine carcinoma as a possible differential diagnosis in ovarian tumors, the use of imaging and specific bio-markers for early identification, timely treatment, and follow-ups.