Mubeen Basharat, Eapen Malini, Sudhindran S, Haridas Nikhil Krishna
Department of Pathology, Amrita Institute of Medical Sciences, KERALA, INDIA.
Department of Surgical Gastroenterology, Amrita Institute of Medical Sciences, KERALA, INDIA.
Turk Patoloji Derg. 2025;41(1):21-29. doi: 10.5146/tjpath.2024.13492.
To highlight two cases mixed neuroendocrine non-neuroendocrine tumors (MINEN) of the liver and to review the literature till date. To present two cases of MINEN of the liver diagnosed in our centre with clinical & diagnostic workup, the treatment modalities, and follow up. Extensive review of the literature and compilation of the presentation and treatment modalities used in those cases.
Thirty-three cases of MINEN of the liver have been reported till date including ours. Our cases presented as incidental masses in liver during workup for other symptoms. AFP levels were normal in both cases but PIVKA (Protein induced by vitamin K absence) levels were increased. Resection was done in one of the cases while the other patient had to undergo transplantation. A diagnosis of MINEN was made on H&E, and confirmed on IHC. One patient was unfit for systemic chemotherapy whereas the other patient received cisplastin and etoposide based chemotherapy. Both patients developed metastasis on follow up but are still alive after 12-15 months.
MINEN is an uncommon tumor of the liver with a poor prognosis as shown by the few studies available. Recurrence and distant metastases are often described even after complete resection and the course is fatal. The role of adjuvant chemotherapy following surgical resection is not fully elucidated. Mean survival in the cases reported ranged from 1 month to 33 months. However, no significant differences were seen in the clinicopathologic profile of the cases described so far. Further multiinstitutional studies and follow up will help to further characterize this subtype for appropriate treatment.
重点介绍两例肝脏混合性神经内分泌-非神经内分泌肿瘤(MINEN)并回顾迄今的文献。展示在我们中心诊断的两例肝脏MINEN病例,包括临床及诊断检查、治疗方式和随访情况。广泛回顾文献并汇总这些病例中所采用的表现及治疗方式。
迄今已报道33例肝脏MINEN病例,包括我们的病例。我们的病例在因其他症状进行检查时作为肝脏偶然发现的肿块出现。两例患者的甲胎蛋白(AFP)水平均正常,但异常凝血酶原(PIVKA,维生素K缺乏诱导蛋白)水平升高。其中一例进行了手术切除,另一例患者不得不接受移植手术。通过苏木精-伊红染色(H&E)做出MINEN诊断,并通过免疫组化(IHC)得以证实。一名患者不适合进行全身化疗,而另一名患者接受了基于顺铂和依托泊苷的化疗。两名患者在随访中均发生转移,但在12 - 15个月后仍存活。
如现有少数研究所显示,MINEN是一种罕见的肝脏肿瘤,预后较差。即使在完全切除后,复发和远处转移也经常出现,病程致命。手术切除后辅助化疗的作用尚未完全阐明。所报道病例的平均生存期为1个月至33个月。然而,迄今为止所描述病例的临床病理特征未见显著差异。进一步的多机构研究和随访将有助于进一步明确该亚型以便进行适当治疗。