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Abstract：
Large cell neuroendocrine carcinoma (LCNEC) is a rare histological subtype of ovarian cancer. A few cases have been reported in the literature with extreme invasiveness and a poor prognosis. However, there still have not been accepted criteria for diagnosis and treatment of LCNEC. Here we report an unmarried 37 year-old woman who was diagnosed with LCNEC associated with clear cell carcinoma and the tumor index was manifested with a specific increase of AFP. The case received six courses of etoposide and carboplatin chemotherapy as an adjuvant therapy after primary curative surgery. However, she relapsed within 6 months after surgery and metastasized rapidly to distant organs despite combined chemotherapy of paclitaxel, cisplatin, and bevacizumab, and died 18 months after primary surgery. This is the first reported case of LCNEC manifested with a specific increase of AFP and characteristically metastasized to the spine as recurrence. Reviewing our case as well as previously reported cases, LCNEC present with aggressive malignancy and vulnerable to distant metastasis through a hematogenous approach, we conjectured that adding Bevacizumab in primary chemotherapy may be beneficial to extend disease-free survival. But so far there is no recommendation of this regimen for treatment of LCNEC in current guidelines. Further research is needed to confirm this view so as to find the best treatment of LCNEC and improve the prognosis of these patients.
摘要：
大细胞神经内分泌癌（LCNEC）是一种罕见的卵巢癌组织学亚型。文献中报道了一些具有极端侵袭性和预后不良的病例。然而,LCNEC的诊断和治疗标准尚未被接受.在这里，我们报告了一名未婚的37岁女性，她被诊断患有与透明细胞癌相关的LCNEC，并且肿瘤指数表现为AFP的特定升高。该病例在初次根治性手术后接受了六个疗程的依托泊苷和卡铂化疗作为辅助治疗。然而,术后6个月内复发，尽管紫杉醇联合化疗，但仍迅速转移至远处器官，顺铂,还有贝伐单抗,并在初次手术后18个月死亡。这是第一例报道的LCNEC病例，表现为AFP的特定增加，并特征性地转移到脊柱作为复发。回顾我们的案例以及以前报道的案例，LCNEC表现为侵袭性恶性肿瘤，易于通过血行途径进行远处转移，我们推测在初级化疗中加入贝伐单抗可能有利于延长无病生存期.但是到目前为止，在目前的指南中还没有推荐这种治疗LCNEC的方案。需要进一步的研究来证实这一观点，以找到LCNEC的最佳治疗方法并改善这些患者的预后。
