Difficulties in the management of mesenchymal hamartomas

Abstract

Mesenchymal hamartoma of the liver is an entity with a varied presentation and frequent initial delay in diagnosis. The macroscopic appearance too is quite heterogeneous with solid, cystic and mixed variants being present with varying degrees of vascularity. Management will depend on presentation and expertise available. We look at a single centre experience with the mesenchymal hamartomas. Retrospective patient record review of the past 30 years, 1976-2006. Seventeen patients aged 1 day to 15 years were identified, with a histopathological diagnosis of mesenchymal hamartoma of the liver. The anatomical location in the liver was 12 in the right liver and the 5 in the left. All patients presented with abdominal distension, eight had significant anorexia and or vomiting. Ultrasound scan was done in all patients. Findings were that of a mass and or cysts. The cysts were multiple in all cases but one and were interspersed with solid elements. Calcification was noted in only two of the patients. Operative approaches were six right hepatectomy, four wedge excision, seven tumour excisions by division of its pedicle; two of these were done laparoscopically, by cyst drainage and excision of the solid component. The tumours were all confirmed as mesenchymal hamartomas; size ranged from 412 to 2,230 g. Complications included three related to misdiagnosis (hydatid disease, and hepatoblastoma). Intraoperative problems consisted of preoperative bleeding resulting in an on-table hypovolaemic arrest and in a second case a bile duct injury. Postoperative problems consisted of an initial incomplete resection, with residual tumour on the IVC. There was rapid regrowth of tumour and death after a second exploration. Two children developed fluid collections requiring re-exploration and drainage. The surviving children have been followed up for a median time period of 4 months (range 1 month-11 years) and are well. Although hamartomas of the liver are histologically benign, their clinical course and the complications of surgical treatment can be significant. They can often pose diagnostic dilemmas and may have a propensity for local recurrence and malignant degeneration.