Intraductal papillary mucinous neoplasm (IPMN) is a tumor originating from pancreatic ductal epithelial cells, leading to excessive mucus secretion and dilation of the pancreatic duct. Pathologically, IPMNs display a wide range of dysplasia, ranging from low-grade dysplasia to invasive carcinoma. Invasion into surrounding organs, especially into spleen, is rare and has not been reported in Korean journals. Worldwide, only two cases have been reported. Here, the authors report their experience with a rare case of IPMN in the pancreas that invaded the spleen.

Intraductal papillary mucinous neoplasm (IPMN) is a tumor originating from pancreatic ductal epithelial cells, leading to excessive mucus secretion and dilation of the pancreatic duct. Pathologically, IPMNs display a wide range of dysplasia, ranging from low-grade dysplasia to invasive carcinoma. Invasion into surrounding organs, especially into spleen, is rare and has not been reported in Korean journals. Worldwide, only two cases have been reported. Here, the authors report their experience with a rare case of IPMN in the pancreas that invaded the spleen.

Intraductal papillary mucinous neoplasm (IPMN) is a tumor originating from pancreatic ductal epithelial cells, leading to excessive mucus secretion and dilation of the pancreatic duct. Pathologically, IPMNs display a wide range of dysplasia, ranging from low-grade dysplasia to invasive carcinoma. Invasion into surrounding organs, especially into spleen, is rare and has not been reported in Korean journals. Worldwide, only two cases have been reported. Here, the authors report their experience with a rare case of IPMN in the pancreas that invaded the spleen.

Infantile cortical hyperostosis, or Caffey's disease, usually presents with typical radiological features of soft tissue swelling and cortical thickening of the underlying bone. The disease can be fatal when it presents antenatally, especially before a gestational age of 35 weeks. This fatal, premature form of the disease is known to occur in various ethnic groups around the globe, and approximately 30 cases have been reported in English literature. This paper is unique in that it is the first paper to report a lethal form of prenatal-type infantile cortical hyperostosis diagnosed in South Korea. Born at gestational age of 27 weeks and 4 days, the patient had typical features of polyhydramnios, anasarca, hyperostosis of multiple bones, micrognathia, pulmonary hypoplasia, and hepatomegaly. The patient was hypotonic, and due to pulmonary hypoplasia and persistent pulmonary hypertension, had to be supported with high frequency ventilation throughout the entire hospital course. Due to the disease entity itself, as well as prolonged parenteral nutrition, liver failure progressed, and the patient expired on day 38 when uncontrolled septic shock was superimposed. The chromosome karyotype of the patient was normal, 46, XX, and COL1A1 gene mutation was not detected.
Edema ; Ethnic Groups ; Gestational Age ; Hepatomegaly ; High-Frequency Ventilation ; Humans ; Hyperostosis ; Hyperostosis, Cortical, Congenital ; Hypertension, Pulmonary ; Infant, Newborn ; Infant, Premature ; Karyotype ; Korea ; Liver Failure ; Micrognathism ; Parenteral Nutrition ; Polyhydramnios ; Shock, Septic