Klaassen Zachary, Paragi Prakash R, Chamberlain Ronald S
Department of Surgery, Saint Barnabas Medical Center, Livingston, N.J., USA.
Case Rep Gastroenterol. 2010 Mar 13;4(1):84-92. doi: 10.1159/000260183.
Mesenchymal hamartoma of the liver (MHL) is a rare benign lesion occurring primarily in the pediatric population. While the precise pathogenesis of the tumor is not certain, the most common theory relates to aberrant mesenchyme development in the portal tract likely related to the bile ducts. A 53-year-old female was evaluated for an incidental liver mass. Initial CT scan showed a cystic lesion in the right lobe of the liver and follow-up imaging revealed an increase in size and the percent solid component within the mass. In view of these changes, a nondiagnostic biopsy was obtained followed by extirpation of the lesion. Gross pathological review of the lesion identified a 9 × 9 × 7.5 cm, pink-yellow-tan, gelatinous mass, with a >1 cm clear surgical margin. Histologically, the mass consisted of benign dilated bile ducts, as well as myxoid stroma with spindle cells showing smooth muscle differentiation. The patient was discharged home on postoperative day five. A review of the literature for MHL in adults reports 30 previous cases, predominantly published as individual case reports describing the size, lobe(s) of the liver affected, and the cystic/solid nature of the tumor. MHL in adults may represent a potentially premalignant lesion, as the emerging literature supports a potential relationship between MHL and malignant undifferentiated embryonal sarcoma in regards to cytogenetic analysis. Aggressive surgical management of MHL in adults is mandated when feasible.
肝脏间叶性错构瘤（MHL）是一种主要发生于儿童群体的罕见良性病变。虽然该肿瘤的确切发病机制尚不确定，但最常见的理论认为与门静脉区域可能与胆管相关的间叶组织异常发育有关。一名53岁女性因偶然发现肝脏肿物而接受评估。最初的CT扫描显示肝脏右叶有一个囊性病变，后续影像学检查显示肿物大小及实性成分比例增加。鉴于这些变化，进行了一次未明确诊断的活检，随后切除了病变。对病变进行大体病理检查发现一个9×9×7.5厘米、粉黄棕色、胶冻状肿物，手术切缘清晰，大于1厘米。组织学上，肿物由良性扩张的胆管以及含有显示平滑肌分化的梭形细胞的黏液样间质组成。患者术后第5天出院。对成人MHL的文献回顾报告了之前的30例病例，主要以个案报告形式发表，描述了肿瘤的大小、受影响的肝叶以及肿瘤的囊性/实性性质。成人MHL可能代表一种潜在的癌前病变，因为新出现的文献支持在细胞遗传学分析方面MHL与恶性未分化胚胎性肉瘤之间存在潜在关系。在可行的情况下，对成人MHL应采取积极的手术治疗。
