Congenital gastric teratoma in Wiedemann-Beckwith syndrome.
Wiedemann-Beckwith syndrome (WBS) may be associated with abdominal tumors, including Wilms tumor, adrenocortical carcinoma, hepatoblastoma, gonadoblastoma, rhabdomyosarcoma, and neuroblastoma. We report on a newborn infant with WBS and a congenital teratoma of the stomach. This is the sole report of any teratoma being associated with WBS and also the first report of a tumor present at birth and visible prenatally in WBS. At birth this infant boy had the diagnostic findings of WBS with macroglossia, ear lobule creases and pits, nevus flammeus, and omphalocele, and an abdominal mass. Abnormalities were detected prenatally when ultrasound examination showed placental overgrowth, polyhydramnios, omphalocele, and posterior abdominal calcifications. Resection of the mass and partial gastrectomy were performed at age 10 days; histologic study showed an immature grade-II teratoma containing a mixture of mature and immature tissues from all germ layers. Results of cytogenetic studies of blood and teratoma were normal (46,XY). This congenital gastric teratoma in a newborn boy with classical WBS may represent either a tumor or an included twin. We discuss its implications for the association of WBS with neoplasia and monozygotic (MZ) twinning, review various neoplasias associated with WBS, and consider pathogenetic mechanisms.
Falik-Borenstein
T C
TC
Ahmanson Pediatric Center, USC School of Medicine.
Korenberg
J R
JR
Davos
I
I
Platt
L D
LD
Gans
S
S
Goodman
B
B
Schreck
R
R
Graham
J M
JM
D001506:Beckwith-Wiedemann Syndrome / Q000235:genetics* / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D013274:Stomach Neoplasms / Q000235:genetics* / Q000473:pathology; D013724:Teratoma / Q000235:genetics* / Q000473:pathology