Hepatic mesenchymal hamartoma with translocation involving chromosome band 19q13.4: A recurrent abnormality
Department of Pathology, Children's Medical Center of Dallas and the University of Texas Southwestern Medical Center, 1935 Motor Street, Dallas, TX 75235, USA. Cancer Genetics and Cytogenetics
(Impact Factor: 1.93).
09/2004; 153(1):60-3. DOI: 10.1016/j.cancergencyto.2003.12.004
We report a case of mesenchymal hamartoma of the liver in an 8-month-old male child, in which the cytogenetic analysis revealed a balanced translocation, t(11;19)(q13;q13.4). This is the fifth description of a cytogenetic abnormality in mesenchymal hamartoma and is similar to the four cases reported previously in that one of the breakpoints involved chromosome band 19q13.4.
Available from: Hee Jin Chang
- "The pathogenesis of MH has been considered to be aberrant development of primitive mesenchyme in the portal tract (3). However, some recent evidences have raised the possibility of true neoplasm (4-6) and those are as follows: the balanced translocation involving chromosome 19q13.4 (4); rare cases of malignant transformation (5); and aneuploidy in flow cytometric analysis (6). "
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ABSTRACT: Mesenchymal hamartoma (MH) of the liver is an uncommon benign lesion related to ductal plate malformation. It is usually cystic and mainly composed of myxoid mesenchymal tissue with tortuous or cystic bile ducts. In order to characterize the clinicopathological features of MH, the Korean Gastrointestinal Pathology Study Group collected a total of 17 MH cases diagnosed in 7 hospitals from 1992 to 2002 and compared the clinicopathologic findings of cystic MH with those of solid variant. Among the 17 cases, 7 (41%) were solid. The solid form showed a higher serum level of alpha-fetoprotein (AFP), the smaller bile ducts, and more frequent proliferation of vessels. Serum AFP level was related to the amount of hepatocytes. Two of seven solid cases harbored a larger amount of evenly distributed hepatocytes and proliferation of small duct with focal hepatocyte-bile duct transition. These histologic findings are similar to those of mixed hamartoma. Therefore, the mixed hamartoma and the MH of both solid and cystic types could be the variants of one disease spectrum. And hepatocytes within MH might be rather a genuine tumor component than entrapped into the tumor. In conclusion, MH can show various clinicopathological features and recognition of these features will facilitate accurate diagnosis of MH.
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ABSTRACT: 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.
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