High-grade neuroendocrine carcinoma of the corpus uteri

High-grade neuroendocrine carcinoma of the corpus uteri is an extremely rare and aggressive malignant tumor arising from the body of the uterus (endometrium and/or myometrium). This cancer is composed of neuroendocrine cells — specialized cells that share features of both nerve cells and hormone-producing cells. The tumor may present as small cell neuroendocrine carcinoma or large cell neuroendocrine carcinoma of the uterine corpus. It is classified under the broader category of neuroendocrine neoplasms of the gynecological tract and is distinct from the more common endometrial carcinomas (endometrioid, serous, or clear cell types). Patients typically present with abnormal uterine bleeding, pelvic pain, or a uterine mass, most commonly in postmenopausal women. Due to its highly aggressive nature, the disease frequently presents at an advanced stage with early lymphovascular invasion and distant metastases to organs such as the lungs, liver, and lymph nodes. The prognosis is generally poor, with rapid disease progression even after treatment. Treatment typically involves a multimodal approach including surgical resection (hysterectomy with bilateral salpingo-oophorectomy and lymph node dissection), combined with platinum-based chemotherapy regimens similar to those used for small cell lung cancer (such as cisplatin or carboplatin with etoposide). Radiation therapy may also be employed as adjuvant treatment. Despite aggressive therapy, recurrence rates are high and overall survival remains limited. Given the rarity of this tumor, there are no standardized treatment guidelines, and management is often guided by case reports, small case series, and extrapolation from treatment protocols for pulmonary neuroendocrine carcinomas. Enrollment in clinical trials is encouraged when available.

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