Mucinous cystic neoplasms of the liver: Literature review and case series

Abstract

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.

Keywords: Cystadenoma; Cystic liver neoplasm; Hepatic cystic lesions; Liver surgery; Liver tumors; Mucinous cystic neoplasm of the liver; Ovarian-like stroma.

Figure 1

Contrast-enhanced computed tomography scan. A and B: Demonstrating arterial (A) and venous (B) phases of a mucinous cystic neoplasm of the liver characterized by multiple enhancing septations.

Figure 2

Histological aspect of a mucinous cystic neoplasms of the liver. A: Low-power view of the cystic wall showing an extensively eroded epithelium and underlying ovarian-like stroma (HE 2.5×); B: Cyst lining predominantly composed of mucinous epithelium, overlying a hypercellular ovarian-like subepithelial stroma (HE 15×); C: Immunohistochemical staining showing estrogen receptor expression within the stromal component (15×); D: Immunohistochemical staining showing progesterone receptor expression within the stromal component (15×).