Mesenchymal hamartoma of the liver: a systematic review.

Mesenchymal hamartoma of the liver is the second commonest benign liver tumor in children, yet its biology and pathogenesis are poorly understood. Cytogenetic studies have suggested that the tumor may be a neoplasm rather than a hamartoma. Typically, it presents as a large benign multicystic liver mass in a child younger than 3 years amenable to complete resection. However, its imaging characteristics are variable, ranging from a few large cysts to a solid mass occupying one or both lobes of the liver. In addition, the tumor occasionally contains angiomatous elements or is multifocal. Most tumors gradually increase in size, some reaching enormous proportions, which can make surgery challenging. Paradoxically, a few undergo incomplete spontaneous regression and, on rare occasions, others have shown malignant transformation to undifferentiated (embryonal) sarcoma. These unusual pathological and biological features must be taken into account when considering the management of affected individuals.