- Pancreatoblastoma is a rare pancreatic tumor that is most commonly encountered in infants and young children. This report describes an unusual presentation of a large pancreatic body pancreatoblastoma presenting with intraabdominal bleeding due to spontaneous rupture of the tumor in a 5-year-old boy. Subsequent molecular analysis from the resected specimen identified a mutation in CTNNB1 and aberrant methylation of the tumor suppressor RASSF1A.
- Meconium peritonitis is a sterile chemical peritonitis caused by bowel perforation with intraperitoneal extravasation of the meconium in utero. When the inflamed intestinal loops become fixed, meconium peritonitis leads to a cystic cavity with a fibrous wall, and the result is termed cystic-type meconium peritonitis. On the contrary, a meconium pseudocyst has a muscle layer continuous with the normal intestine and is distinguished from cystic-type meconium peritonitis based on the histopathologic findings.
- We report a case of papillary carcinoma (PC) with extensive squamous metaplasia arising from a thyroglossal duct cyst (TDC) that required differential diagnosis from squamous cell carcinoma (SCC). An 11-year-old Japanese girl presented with a 9-month history of an anterior-midline neck mass that was clinically diagnosed as TDC. Open neck biopsy revealed nested proliferation of atypical squamous cells within the cystic structures, and SCC arising from TDC was initially suspected. Further examination, however, including immunohistochemistry, revealed the tumor to be of thyroid cell origin.
- Ingestion of a button battery has been considered a serious problem, causing necrosis and perforation, when impacted in the esophagus. However, such batteries in the stomach rarely cause any harm to the gastric wall, which is regarded as evidence supporting the use of conservative treatment. We present the rare case of a 3-month-old infant with severe gastric wall injury caused by a button battery lodged in the stomach. The present case suggests that button batteries located in the stomach should be removed as soon as possible, especially in infants.
- This report presents a case of a surviving monochorionic twin with multiple intestinal atresia and congenital bilateral perisylvian syndrome, which developed after the intrauterine death of the cotwin. The pathology of the placenta demonstrated vein-to-vein communication between the twins and multiple intravascular thrombi in the dead cotwin.
- Localized myopathy of the muscular layers may be an important factor contributing to segmental dilatation of the intestine (SDI). Only one report has described SDI of the jejunum in a neonate showing no abnormality of the interstitial cells of Cajal (ICC). The present report describes the very rare case of a neonatal girl with segmental dilatation of the distal duodenum and proximal jejunum with irregular arrangements of Auerbach's plexus and ICC and the successful surgical treatment of SDI. We review the literature on this type of relationship between abnormality of ICC and SDI and discuss the clinical features of this complication.
- Hepatic alveolar echinococcosis (AE) is an endemic disease in certain parts of the world and relatively rare in children. This report describes a 9-year-old girl with hepatic AE invading the diaphragm and directly spreading to the chest and abdominal wall. She was treated by surgical extirpation and albendazole therapy. In advanced hepatic AE, combined therapy of surgical and medical intervention is thought to improve the prognosis.