Mesenchymal Hamartoma of the Liver in Children Single Center Experience

Artur Apanasiewicz, Małgorzata Markiewicz-Kijewska, Hor Ismail, Marek Stefanowicz, Adam Kowalski and Piotr Kalicinski

Department of Pediatric Surgery and Organ Transplantation, Children’s Memorial Health Institute, Warsaw, Poland

^*Correspondance to: Małgorzata Markiewicz-Kijewska 

 PDF  Full Text DOI: 10.25107/2474-1647.2541

Abstract

Mesenchymal Hamartoma (MH) of the liver is the rare congenital lesion, solid, cystic, or mixed type. It is usually recognized in early childhood (before 3 years of age). Histologically, the MH is a benign lesion but undifferentiated sarcoma in mesenchymal hamartoma was described in several cases. Some features of MH can be potentially locally malignant. Quick enlargement of the tumor may be the cause of compression of the liver, can lead to respiratory insufficiency, ileus or abdominal compartment syndrome. Differential diagnosis is difficult, especially when tumor consist of mesenchymal stroma elements. Resection of the tumor is usually the treatment of choice, but not in all cases is possible (it depends on type, size and location of the tumor). Between 1986 and 2015 we treated 21 patients with MH of the liver. Surgical treatment included cystic drainage and obliteration, marsupialisation, tumor resection (enucleation, liver resection and total hepatectomy with liver transplantation). Diagnosis of MH was confirmed in all cases, coexisting infantile hemangioma was found in one child. All patients, except one are alive in good general condition. One patient died due to secondary biliary cirrhosis after extensive tumor resection. Surgical resection of the MH is often difficult due to irregular form of the tumor and no clear border between tumor and normal liver. Complications after resection are possible especially if tumor is located in segment IV. Nonresectional treatment is usually not effective.

Keywords

Mesenchymal hamartoma of the liver; Diagnosis; Treatment; Outcome

Cite the article

Apanasiewicz A, Markiewicz-Kijewska M, Ismail H, Stefanowicz M, Kowalski A, Kalicinski P. Mesenchymal Hamartoma of the Liver in Children Single Center Experience. Clin Surg. 2019; 4: 2541.