Rare large cell neuroendocrine tumor of the endometrium: A case report and review of the literature

INTRODUCTIONLarge cell neuroendocrine carcinoma (LCNEC) of the endometrium is a rare malignancy with an aggressive course. Although data is limited to case reports, the prognosis appears to be poor, similar to other type II uterine cancers. A total of 12 cases of LCNEC of the uterus have been published to date.PRESENTATION OF CASEA 71 year-old woman presented with postmenopausal vaginal bleeding. Endometrial biopsy was non-diagnostic for LCNEC. She underwent surgical debulking and staging of a 22 cm endometrial tumor with omental metastasis and positive lymph nodes. Her final FIGO stage was IVB.DISCUSSIONWe summarize all prior case reports of LCNEC of the endometrium and discuss the definition, presentation, imaging and surgical management. The pathology with immunohistochemical review, adjuvant therapy and prognosis of LCNEC of the endometrium are also reviewed.CONCLUSIONPathologic findings and immunohistochemistry are essential in making a diagnosis of LCNEC of the endometrium. Primary debulking and surgical staging is typically performed, but if a diagnosis of LCNEC can be made preoperatively with immunohistochemistry, surgeons should consider neoadjuvant chemotherapy due to its high grade histology and aggressive course. Otherwise adjuvant chemotherapy is usually given. Even with early stage disease, the prognosis seems poor. Due to the rarity of this aggressive malignancy, more data is needed to establish incidence.