Pavlidis Efstathios T, Galanis Ioannis N, Pavlidis Theodoros E
The 2 Department of Propaedeutic Surgery, Hippokration General Hospital, School of Medicine, Aristotle University of Thessaloniki, Thessaloniki 54642, Greece.
World J Gastrointest Oncol. 2024 Dec 15;16(12):4559-4564. doi: 10.4251/wjgo.v16.i12.4559.
Mixed neuroendocrine non-neuroendocrine neoplasms constitute rare tumors that are located mainly in the gastrointestinal (GI) tract and have high degrees of malignancy, and the frequency of these tumors has been increasing. They consist of a neuroendocrine neoplastic component with another component of adenocarcinoma usually and have a dismal prognosis. The rare GI pure neuroendocrine carcinoma is highly aggressive and requires complex and extensive management since a genetic distinction exists between it and GI non-neuroendocrine neoplasms, which are generally slow-growing lesions. The most common GI-mixed neuroendocrine non-neuroendocrine neoplasms are colorectal, followed by gastric, mainly in the gastroesophageal junction. Current imaging modalities of nuclear medicine and radiology play important roles in the accuracy of diagnosis. Liquid biopsy may contribute to early detection and timely diagnosis. Ultrasonography, either endoscopic or abdominal, is a technique that contributes to a diagnosis; additionally, contrast-enhanced ultrasonography is very helpful in follow-up appointments. Histopathology establishes a definite diagnosis and stage by evaluating the cell differentiation grade and the cell proliferation index Ki67. The genetic profile can be valuable in diagnosis and gene therapy. Surgical resection with wide lymphadenectomy, whenever possible, and adjuvant chemotherapy constitute the main therapeutic management strategies. Targeted therapy and immunotherapy achieve encouraging results.
混合性神经内分泌-非神经内分泌肿瘤是一类罕见肿瘤，主要位于胃肠道（GI），恶性程度高，且其发病率呈上升趋势。这类肿瘤通常由神经内分泌肿瘤成分和腺癌成分组成，预后较差。罕见的胃肠道纯神经内分泌癌侵袭性很强，由于它与通常生长缓慢的胃肠道非神经内分泌肿瘤在基因上存在差异，因此需要复杂且广泛的治疗。最常见的胃肠道混合性神经内分泌-非神经内分泌肿瘤发生于结肠直肠，其次是胃，主要位于胃食管交界处。目前核医学和放射学的成像方式在诊断准确性方面发挥着重要作用。液体活检有助于早期发现和及时诊断。无论是内镜超声还是腹部超声，都是有助于诊断的技术；此外，超声造影在随访中非常有帮助。组织病理学通过评估细胞分化程度和细胞增殖指数Ki67来明确诊断和分期。基因图谱在诊断和基因治疗中可能具有重要价值。只要有可能，进行广泛淋巴结清扫的手术切除以及辅助化疗是主要的治疗策略。靶向治疗和免疫治疗取得了令人鼓舞的效果。
