Department of Gastrointestinal Oncology, H. Lee Moffitt Cancer Center, Tampa, FL, USA.
Pancreas. 2010 Aug;39(6):799-800. doi: 10.1097/MPA.0b013e3181ebb56f.
Extrapulmonary poorly differentiated neuroendocrine carcinomas can originate in the gastrointestinal tract, bladder, cervix, and prostate. These high-grade malignancies are characterized by aggressive histological features (high mitotic rate, extensive necrosis, and nuclear atypia) and a poor clinical prognosis. They are infrequently associated with secretory hormonal syndromes (such as the carcinoid syndrome) and rarely express somatostatin receptors.Most poorly differentiated neuroendocrine carcinomas are locally advanced or metastatic at presentation. First-line systemic chemotherapy with a platinum agent (cisplatin or carboplatin) and etoposide is recommended for most patients with metastatic-stage disease; however, response durations are often short. Sequential or concurrent chemoradiation is recommended for patients with loco-regional disease. In patients with localized tumors undergoing surgical resection, adjuvant treatment (chemotherapy with or without radiation) is warranted in most cases.
肺外分化差的神经内分泌癌可起源于胃肠道、膀胱、宫颈和前列腺。这些高级别恶性肿瘤的特征为侵袭性组织学特征（高有丝分裂率、广泛坏死和核异型性）和不良的临床预后。它们很少与分泌性激素综合征相关（如类癌综合征），也很少表达生长抑素受体。大多数分化差的神经内分泌癌在就诊时已为局部晚期或转移。对于大多数转移性疾病患者，建议采用含铂药物（顺铂或卡铂）联合依托泊苷的一线全身化疗；然而，缓解持续时间通常较短。对于局部疾病患者，建议序贯或同期放化疗。对于接受手术切除的局限性肿瘤患者，大多数情况下需要辅助治疗（化疗加或不加放疗）。
