Cicerone Ottavia, Basilico Giorgia, Tassi Claudio, Antoniacomi Caterina, Lucev Federica, Corallo Salvatore, Vanoli Alessandro, Maestri Marcello
Department of Clinical-Surgical, Diagnostic and Pediatric Sciences, University of Pavia, Pavia 27100, Italy.
Department of Molecular Medicine, University of Pavia, Pavia 27100, Italy.
World J Clin Oncol. 2025 Aug 24;16(8):108557. doi: 10.5306/wjco.v16.i8.108557.
Mucinous cystic neoplasms of the liver (MCN-L) are rare cystic lesions characterized by mucin-producing epithelium and ovarian-like stroma. Although they constitute fewer than 5% of hepatic cystic lesions, MCN-L poses significant diagnostic challenges due to overlapping features with other cystic lesions and their potential for malignant transformation. Early recognition and definitive surgical intervention are therefore critical to ensure optimal patient outcomes. A literature review was conducted to summarize epidemiology, clinical presentation, diagnostic modalities, and management strategies for MCN-L. Additionally, from 2019 to 2025, 9 patients with MCN-L were identified at our center. Clinical data and outcomes were retrospectively analyzed. MCN-L predominantly affects middle-aged women and presents as large, multiloculated cystic lesions without biliary communication. The revised 2010 World Health Organization classification emphasizes the presence of ovarian-like stroma for definitive diagnosis. Contrast-enhanced computed tomography or magnetic resonance imaging are often suggestive but not pathognomonic, reinforcing the need for histopathological confirmation. MCN-L remains a diagnostic and therapeutic challenge due to its resemblance to other cystic liver lesions. Complete surgical resection is the treatment of choice to prevent recurrence and malignant transformation, reinforcing the importance of early intervention. Further research is needed to improve diagnostic accuracy and refine management strategies.
肝脏黏液性囊性肿瘤（MCN-L）是一种罕见的囊性病变，其特征为产生黏液的上皮和卵巢样间质。尽管它们在肝囊性病变中所占比例不到5%，但由于与其他囊性病变有重叠特征且有恶变潜能，MCN-L带来了重大的诊断挑战。因此，早期识别和确定性手术干预对于确保患者获得最佳预后至关重要。进行了一项文献综述，以总结MCN-L的流行病学、临床表现、诊断方法和管理策略。此外，2019年至2025年期间，我们中心共确诊9例MCN-L患者。对其临床资料和预后进行了回顾性分析。MCN-L主要影响中年女性，表现为无胆管相通的大的多房囊性病变。2010年世界卫生组织修订分类强调存在卵巢样间质对于明确诊断的重要性。增强计算机断层扫描或磁共振成像通常具有提示作用，但并非特异性表现，这进一步凸显了组织病理学确诊的必要性。由于MCN-L与其他肝囊性病变相似，它仍然是一个诊断和治疗难题。完整的手术切除是预防复发和恶变的首选治疗方法，这凸显了早期干预的重要性。需要进一步研究以提高诊断准确性并完善管理策略。
