HEPATOBLASTOMA IN A CHILD WITH TRISOMY-18 - CYTOGENETICS, LIVER ANOMALIES, AND LITERATURE-REVIEW

A 26-month-old female with trisomy 18 and repaired omphalocele died of metastatic disease after resection of hepatoblastoma (HB) at 21 month s of age. Four other cases (three of them published) suggest that the association of trisomy 18 and HB may be nonrandom. Karyotype abnormali ties of the tumor in our case included duplication of 2q and +20, repo rted previously in HB arising in patients with normal karyotype. Antec edent growth disturbance of liver, either intrinsic (abnormal lobation ) or related to contiguous extrinsic anomalies such as omphalocele or local diaphragmatic hypoplasia and possibly augmented by unusual sensi tivity to noxious environmental agents, may predispose to hepatoblasto ma in trisomy 18. Longevity in trisomy 18 predisposes to both hepatobl astoma and Wilms tumor, possibly by a shared pathway.