Abstract

JUAREZ-VILLEGAS, Luis Enrique; ZAPATA-TARRES, Marta; LEZAMA DEL VALLE, Pablo  and  PALOMO-COLLI, Miguel Ángel. Pierre-Robin syndrome and hepatoblastoma: a case report. Bol. Med. Hosp. Infant. Mex. [online]. 2010, vol.67, n.6, pp.543-547. ISSN 1665-1146.

Background. Pierre-Robin Sequence (PRS) is a congenital disease characterized by micrognatia, glosoptosis and U-shaped posterior cleft palate. Its prevalence is 1:8500 newborns. Hepatoblastoma (HB) is the most frequent hepatic malignant tumor in childhood and affects patients <3 years of age. Case report. A 2-year old male with PRS was diagnosed with HB. The boy was the product of the second full term pregnancy. He was born by cesarean with a birth weight of 2750 g. The patient presented with a 1-month history of abdominal mass. Diagnosis of HB was done by biopsy. The patient received chemotherapy with cisplatin, vincristin and 5-fuorouracil as well as left lobectomy. Five years later he is alive without tumor activity. Conclusions. PRS and HB association has not been reported in the literature. Both entities have chromosome 2 alterations. Because cancer is associated with cytogenetic alterations, future considerations must be made to fnd a relationship between cancer patients and other genetic entities. This will be useful for fnding molecular targets. We can also detect the at-risk population in order to create follow-up programs that will allow an early diagnosis with an improved better survival. It is the frst case of PRS and HB reported in either the national or international literature.

Keywords : Pierre-Robin Sequence; hepatoblastoma; left lobectomy; cisplatin; vincristin; 5-fuorouracil.

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