Pure large cell neuroendocrine carcinoma originating from the endometrium: A case report

BACKGROUND: Large cell neuroendocrine carcinoma (LCNEC) of the endometrium is an uncommon and highly aggressive tumor that has not been comprehensively characterized. We report a case of pure endometrial LCNEC and review the current literature of similar cases to raise awareness of the histological features, treatment, and prognosis of this tumor. CASE SUMMARY: We report the case of a 73-year-old woman who presented with irregular postmenopausal vaginal bleeding. Ultrasonography showed an enlarged uterus and a 5.1 cm × 3.3 cm area of medium and low echogenicity in the uterine cavity. Biopsy by dilatation and curettage suggested poorly differentiated carcinoma. Magnetic resonance imaging revealed a heterogeneously enhanced uterine tumor with diffuse infiltration of the posterior wall of the uterine myometrium and enlarged pelvic lymph nodes. The patient underwent a hysterectomy and bilateral adnexal resection. Gross observation revealed an ill-defined white solid mass of the posterior wall of the uterus infiltrating into the serosa with multiple solid nodules on the serous surface. Microscopically, the tumor cells showed neuroendocrine morphology (organoid nesting). Immunohistochemistry revealed the tumor cells were diffusely positive for the neuroendocrine markers CD56, chromogranin A, and synaptophysin. Thus, the tumor was diagnosed as stage IIIC endometrial LCNEC. CONCLUSION: Pathologic findings and immunohistochemistry are essential in making a diagnosis of endometrial LCNEC.