Surjan Rodrigo Cañada Trofo, Paulino Jorge Francisco de Assis, Schleinstein Henrique Perobelli, Pereira Felipe Moraes Toledo, Figueira Estela Regina Ramos, Ardengh José Celso
Hospital Nove de Julho - São Paulo (SP), Brazil.
Universidade de São Paulo, Faculty of Medicine, Department of Gastroenterology, Pancreaticobiliar Surgery - São Paulo (SP), Brazil.
Arq Bras Cir Dig. 2024 Dec 2;37:e1840. doi: 10.1590/0102-6720202400046e1840. eCollection 2024.
Mixed neuroendocrine-non-neuroendocrine tumors (MiNEN) are a rare type of tumor formed by two components, a non-neuroendocrine component that is most often an adenocarcinoma and a neuroendocrine tumor, and each of these components must represent at least 30% of the tumor. The origin of this tumor on the ampulla of Vater or periampullary region is more infrequent. Usually, the lesions are highly aggressive and quickly metastasizing, and their biological behavior is dictated by the high grade of the neuroendocrine component. This is the first report of a patient with ampullary MiNEN treated employing a robotic pancreaticoduodenectomy. Although being submitted to aggressive treatment with complete surgical resection followed by systemic therapy, the patient developed early recurrence with hepatic metastatic disease, demonstrating the hostile nature of these tumors.
混合性神经内分泌-非神经内分泌肿瘤(MiNEN)是一种罕见的肿瘤类型,由两种成分组成,一种非神经内分泌成分,最常见的是腺癌,另一种是神经内分泌肿瘤,且每种成分必须占肿瘤的至少30%。这种肿瘤起源于 Vater壶腹或壶腹周围区域的情况更为少见。通常,这些病变具有高度侵袭性且转移迅速,其生物学行为由神经内分泌成分的高分级决定。这是首例采用机器人胰十二指肠切除术治疗壶腹MiNEN患者的报告。尽管患者接受了积极治疗,包括完整的手术切除及后续的全身治疗,但仍出现了早期复发并伴有肝转移,显示出这些肿瘤的恶性本质。