Pancreatic hamartoma in a premature Trisomy 18 female Article / Autopsy Case Report

Case Autopsy, Reports
unpublished
How to cite: Delgado PI, Correa-Medina M, Rojas CP. Pancreatic hamartoma in a premature Trisomy 18 female. Autops Case Rep [Internet]. 2017;7(4):26-29. http://dx. ABSTRACT Pancreatic hamartomas are extremely rare tumors in adults and even more so in children. They are lesions characterized by acinar, islet and ductal components found in varying proportions and in a disorganized pattern. We report a case of a premature female with trisomy 18 diagnosed by amniocentesis. The newborn was delivered
more » ... y cesarean section at thirty-three weeks of gestation and expired within one hour of birth. Postmortem examination exhibited numerous features associated with Trisomy 18 including lanugo on the torso and arms, micrognathia, microstomia, left low-set ear with small flat pinna, closed ear canal, clenched fists with overlapping fingers, rocker-bottom feet, narrow pelvis, large right diaphragmatic hernia and left pulmonary hypoplasia. Microscopic examination of the pancreas revealed an area, 1.2 cm in greatest dimension, with branching ducts and cysts lined by cuboidal epithelium intermingled within primitive mesenchymal proliferation and exocrine glands. The cysts measured up to 0.2 cm and were surrounded by a collarette of proliferating spindle cells as highlighted by Masson's trichrome stain. A diagnosis of pancreatic hamartoma was rendered. A total of thirty-four cases of pancreatic hamartomas have been reported in the literature including twenty-seven in adults, five in children and two in newborns. Our case may be the third pancreatic hamartoma reported in association with Trisomy 18. We recommend that careful examination of the pancreas be performed in individuals with Trisomy 18 to further characterize this lesion as one of the possible abnormal findings associated with this syndrome. CASE REPORT The premature female was born at 33 weeks of gestation to a 25 years old mother, gravida 3 para 2, with a history of pre-eclampsia in the previous pregnancy. In this pregnancy, the fetus was found to have Trisomy 18 karyotype by amniocentesis. Fetal ultrasound at 21 weeks gestational age showed left displacement of the heart and absent nasal bone. The mother presented to at 33 weeks of gestation for premature rupture of membranes and contractions. Fetal ultrasound performed at that time showed normal amniotic fluid volume, intrauterine growth restriction with estimated fetal weight of 1519 grams (less than the 10th percentile for gestational age), right side congenital diaphragmatic hernia with liver herniating into the thorax, left mediastinal shift of the heart, hypoplastic left lung, and clenched hands with
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