Pinelli Domenico, Guerci Claudio, Cammarata Francesco, Cirelli Riccardo, Scatigno Agnese, Colledan Michele
Department of Organ Failure and Transplantation, ASST Papa Giovanni XXIII Hospital, Bergamo 24127, Italy.
University of Milan, Via Festa del Perdono 7, Milano 20122, Italy.
J Surg Case Rep. 2024 Apr 1;2024(4):rjae184. doi: 10.1093/jscr/rjae184. eCollection 2024 Apr.
Mesenchymal hamartoma of the liver (MHL) is rare. Less than 50 adult cases have been described. Due to their potential degeneration or recurrence, a complete surgical resection must be performed. We describe a case of a 26-year-old with a palpable solid lesion, which displaced abdominal organs. Percutaneous needle biopsies suggested the diagnosis of MHL. A right hemi-hepatectomy without segment 1 was performed; the post-operative course was uneventful. The mesenchymal component of the tumour was reactive to desmin and smooth muscle actin. Low proliferation index was confirmed (MIB1). Genetic counselling: the sequencing analysis of DICER1 and CDKN1C gene was negative, DNA methylation analysis on the chromosome 11p15 region was normal. After 42 months, there was no recurrence. In conclusion, clinicians should consider MHL in the differential diagnosis. The dimension and the need of radicality impose major liver resections or liver transplantations, which should be performed in referral centres.
肝间叶性错构瘤（MHL）较为罕见。已报道的成人病例不足50例。鉴于其有潜在退变或复发风险，必须进行完整的手术切除。我们报告一例26岁患者，其可触及实性肿块，该肿块使腹部脏器移位。经皮穿刺活检提示MHL诊断。实施了不包括第1段的右半肝切除术；术后病程顺利。肿瘤的间叶成分对结蛋白和平滑肌肌动蛋白呈反应性。证实增殖指数较低（MIB1）。遗传咨询：DICER1和CDKN1C基因的测序分析为阴性，11号染色体p15区域的DNA甲基化分析正常。42个月后无复发。总之，临床医生在鉴别诊断时应考虑MHL。肿瘤大小及根治需求要求进行大范围肝切除或肝移植，此类手术应在转诊中心进行。
