A Case of Isolated Ileal Ganglioneuroma

Ganglioneuromas of the gastrointestinal tract are rare, but have an established association with genetic disorders, such as the multiple endocrine neoplasia (MEN) syndrome (type 2b) and neurofibromatosis (type 1). However, solitary ganglioneuromas are not associated with an increased risk for MEN 2b, neurofibromatosis type 1, or any other systemic conditions. Ganglioneuromas of the gastrointestinal tract have been reported to predominantly involve the colon and rectum, and are thereby occasionally detected during colonoscopy or surgery. Although there are no characteristic symptoms of solitary ganglioneuromas, symptoms can be induced by solitary ganglioneuromas, such as abdominal pain, bleeding, or obstruction, depending on the location and size. Herein we report a case of a solitary ganglioneuroma of the ileum. A 34-year-old man sought evaluation at our hospital for anemia. The medical and family histories were benign and there was no history of genetic disorders. The evaluation for anemia revealed iron-deficiency anemia and CT enterography revealed a single mass in the ileum. Laparoscopic resection of the lesion was performed and the pathologic examination confirmed an ileal ganglioneuroma.

A Case of Isolated Ileal Ganglioneuroma

Ganglioneuromas of the gastrointestinal tract are rare, but have an established association with genetic disorders, such as the multiple endocrine neoplasia (MEN) syndrome (type 2b) and neurofibromatosis (type 1). However, solitary ganglioneuromas are not associated with an increased risk for MEN 2b, neurofibromatosis type 1, or any other systemic conditions. Ganglioneuromas of the gastrointestinal tract have been reported to predominantly involve the colon and rectum, and are thereby occasionally detected during colonoscopy or surgery. Although there are no characteristic symptoms of solitary ganglioneuromas, symptoms can be induced by solitary ganglioneuromas, such as abdominal pain, bleeding, or obstruction, depending on the location and size. Herein we report a case of a solitary ganglioneuroma of the ileum. A 34-year-old man sought evaluation at our hospital for anemia. The medical and family histories were benign and there was no history of genetic disorders. The evaluation for anemia revealed iron-deficiency anemia and CT enterography revealed a single mass in the ileum. Laparoscopic resection of the lesion was performed and the pathologic examination confirmed an ileal ganglioneuroma.

A Case of Diffuse Large B-cell Lymphoma in a Kidney Transplant Recipient / 대한신장학회잡지

Posttransplant lymphoproliferative disorders (PTLD) is an infrequent but serious complication of transplantation. Previous studies have suggested the terms of reference, "early PTLD" (referring to PTLD that occurs within 1 year of transplantation) and "late PTLD" (PTLD that occurs after 1 year). Early PTLD generally involves a single organ or nodal region and often responds favorably to a decrease in immunosuppression. Late PTLD tends to be disseminated, responds less frequently to a decrease in immunosuppression, and has a dismal prognosis. We encountered a diffuse large B-cell lymphoma in a 44-year-old man who underwent kidney transplantation over 10 years ago, in 1995. In situ hybridization for Epstein-Barr virus showed positive results in tumor cell. With decreased immunosuppressants and chemotheraphy, he is currently in complete remission.

A Case of Disseminated Scedosporium apiospermum Infection in a Liver Transplant Patient

Sedosporium apiospermum is a saprophytic fungus commonly found in soil and polluted water. This organism is known as a cause of mycetoma, which may occur in immunocompetent hosts following trauma. However, in immunocompromised patients, S. apiospermum can also cause life-threatening invasive disease, including central nervous system infection or disseminated infection. We report a fatal case of disseminated S. apiospermum infection in a 46-year-old woman after liver transplantation. Eight days postoperatively, she developed pneumonia, followed by altered mentality in the 15 days. A head CT demonstrated multiple brain abscesses. Sputum and stereotactic-aspirated brain abscess culture yielded S. apiospermum. Despite treatment with voriconazole, the patient died of intracranial hemorrhage.

A Case of Disseminated Scedosporium apiospermum Infection in a Liver Transplant Patient

Sedosporium apiospermum is a saprophytic fungus commonly found in soil and polluted water. This organism is known as a cause of mycetoma, which may occur in immunocompetent hosts following trauma. However, in immunocompromised patients, S. apiospermum can also cause life-threatening invasive disease, including central nervous system infection or disseminated infection. We report a fatal case of disseminated S. apiospermum infection in a 46-year-old woman after liver transplantation. Eight days postoperatively, she developed pneumonia, followed by altered mentality in the 15 days. A head CT demonstrated multiple brain abscesses. Sputum and stereotactic-aspirated brain abscess culture yielded S. apiospermum. Despite treatment with voriconazole, the patient died of intracranial hemorrhage.

Comparison of Clinical Findings between Autoimmune Pancreatitis with Bile Duct Involvement and Primary Sclerosing Cholangitis / 대한소화기학회지

BACKGOUND/AIMS: The involvement of bile ducts is frequently reported in autoimmune pancreatitis (AIP), which seem to have similar features to primary sclerosing cholangitis (PSC). Recent systematic comparative studies about these diseases are rare in Korea. METHODS: We retrospectively analyzed 26 patients with AIP with bile duct involvement and 30 patients with classic PSC who were diagnosed during the last decade. RESULTS: The mean age of patients was significantly higher in AIP than PSC at the time of diagnosis. There was a preponderance of men in both group, which was more prominent in AIP. The most common symptom in patients with AIP was jaundice, but PSC patients usually visited hospitals due to incidentally detected abnormal liver function tests. Most (26/31) of AIP had bile duct involvement. All of these patients showed narrowing of intrapancreatic common bile ducts and one patient exhibited hilar involvement as well. About 80% of PSC had both intra- and extrahepatic ducts involvement, and the characteristic features involve multifocal strictures. AIP patients showed improvement with steroid treatment, however, most PSC patients showed clinical deterioration. CONCLUSIONS: The clinical and cholangiographic findings of patients with AIP and PSC have many different characteristics. Therefore, further study of two diseases is required for the proper diagnosis and management.

Thirteen Cases of Intrahepatic Biliary Cystadenoma and Cystadenocarcinoma: A Single Center Experience / 대한소화기학회지

BACKGROUND/AIMS: Biliary cystadenoma (BCA) and biliary cystadenocarcinoma (BCACa) are rare cystic neoplasms that usually arise from the liver. We reviewed the clinicopathologic and radiologic findings of 13 cases of intrahepatic biliary cystic neoplasms. METHODS: Seven patients with BCA and 6 patients with BCACa which were pathologically proven within past 10 years were included in this retrospective study. RESULTS: BCA (7 of 7) was more common in female compared to BCACa (4 of 6). Mean age at diagnosis was 53.4 years (BCA) and 58.5 years (BCACa). Abdominal pain (54%) was the most common presenting symptom. Eleven patients (61.5%) exhibited normal liver function profiles and 5 patients (38%) showed elevated levels of serum CA19-9 levels (mean 894.2 U/mL, range: 78.7-2,080). Mean size of tumor was 11.7 cm (range: 5-15). Most frequent radiologic finding was a single cystic mass with septation. BCACa tended to have intracystic solid portion. The cut surface revealed a unilocular or multilocular cystic mass with mucinous contents. Complete surgical excision was done in 12 patients. After the complete resection, recurrence was observed in 1 case of BCACa. CONCLUSIONS: The possibility of biliary cystic neoplasm should be suspected when an intrahepatic cystic lesion with multiseptation or solid portion is noted on imaging study. In addition, complete excision for definite diagnosis and treatment need to be performed.

Outcome of Intensive Medical Treatments in Patients with Infected Severe Necrotizing Pancreatitis / 대한소화기학회지

BACKGROUND/AIMS: Infection of pancreatic necrosis is one of the leading cause of death in patients with severe necrotizing pancreatits. Because of high mortality rate up to 50%, immediate surgical debridement including pancreatectomy is recommended. However, early surgical treatment still showed high mortality rate and better treatment strategy is required. This study was conducted to evaluate the outcomes of early intensive non-surgical treatments in patients with infected necrotizing pancreatitis. METHODS: This study was based on retrospective analysis of 71 patients with acute severe necrotizing pancreatitis (APACHE II score>or=8, or Ranson's score>or=3, and pancreatic necrosis on CT scan), who were admitted to medical center during past 16 years. Infection of pancreatic necrosis was confirmed by fine needle aspiration, and early intensive medical treatments comprised of prophylactic antibiotics coverage, fluid resuscitation, organ preserving supportive measures, and percutaneous catheter drainage were carried out. RESULTS: Among the enrolled patients, infections were suspected in 46 patients, but fine needle aspirations were done only in 32 patients. In 21 patients, infections of necrotic tissue were confirmed by bacteriology, while other 11 patients showed no evidence of bacterial growth. Of 21 patients with infected necrosis, initial surgical interventions were performed in 2 patients, while initial medical treatments were performed in 19 patients. The success rate of medical treatment group in infected necrotizing pancreatitis was 79% (15/19). The mortality rate of medical treatment group and surgical treatment group was 5% (1/19) and 50% (1/2). CONCLUSIONS: Early intensive medical treatment seems to be a good therapeutic strategy, even if the infection has developed in pancreatic necrosis. Further prospective randomized studies are required to confirm this finding.