Toru Ichikawa

Adenocarcinoma arising from a gastric duplication cyst with invasion to the stomach: a case report with literature review

This report describes a rare case of adenocarcinoma arising from a gastric duplication cyst, with invasion to the stomach wall, in a 40 year old Japanese man. A cystic lesion was found between the stomach and the spleen. The cyst had a well circumscribed smooth muscle layer, corresponding to the muscularis propria of the stomach and the mucosa of the alimentary tract. A well differentiated adenocarcinoma was found within the duplication cyst, invading its serosa. Well differentiated adenocarcinoma was independently found in the fundus of the stomach; the tumour of the cyst was connected by fibrous tissue. Microscopically, there was neither adenocarcinoma in situ nor precancerous lesions, such as epithelial dysplasia, suggesting that the carcinoma derived from a gastric duplication cyst that invaded the stomach. Duplication cysts should be included in the differential diagnosis of cystic masses of the gastrointestinal tract, and the possibility of malignancy within these cysts should be considered.

Telomerase activity in neuroblastoma: is it a prognostic indicator of clinical behaviour?

Neuroblastomas show remarkable biological heterogeneity, resulting in favourable prognosis or unfavourable prognosis due to aggressive growth despite multimodal therapy. Recently, we proposed that aggressive tumours express telomerase at a high level while the favourable tumours lack or have low telomerase expression. To evaluate the correlation between telomerase activity and other biological characteristics reported as prognostic markers (MYCN gene amplification, loss of heterogeneity (LOH) in the short arm of chromosome 1, trk-A expression, Ha-ras p21 expression, and DNA ploidy), we investigated these biological features in 105 untreated neuroblastomas. In these cases, 23 showed high telomerase activity, 78 showed low activity, and telomerase activity was undetectable in 4 cases. Most tumours with genetic alterations (MYCN amplification or 1p32 LOH) showed high telomerase activity. Most tumours with low or undetectable activity were aneuploid, and showed trk-A and Ha-ras expression. Three of the four tumours with undetectable telomerase activity regressed. In 2 of the tumours with low telomerase activity, the residual tumours maturated and showed repression of telomerase activity. Thus, the level of telomerase activity correlated with other genetic alterations and/or gene expression and may be a useful prognostic indicator in neuroblastoma.

Surgical management of tracheal agenesis

Tracheal agenesis is a rare congenital malformation that usually is fatal. This report describes our experience in two such cases. In both cases, the diagnosis was suspected at birth, because the patients had respiratory distress without an audible cry and were difficult to intubate. A gastrostomy and banding of the abdominal esophagus provided effective initial stabilization, in conjugation with respiratory management. The first patient also had complex cardiac malformations, and the infant died of cardiac failure 1 week after birth. The second infant, who had tracheal agenesis with a proximal tracheoesophageal fistula and a bronchoesophageal fistula, was managed successfully. At 9 months of age, a tracheotomy was performed, a long T tube was inserted to maintain the airway patency beyond the proximal tracheoesophageal fistula, and the patient was discharged. At 3 years of age, esophageal reconstruction was performed with a colonic interposition graft. The patient is thriving and developing normally at 4 years of age. Diagnosis at birth and maintenance of airway patency are essential for successful management of tracheal agenesis. Initial surgical interventions are palliative but lifesaving. Subsequent management focuses on improving the quality of life and allowing swallowing and speech.

An evaluation of laparoscopic cholecystectomy after selective percutaneous transhepatic gallbladder drainage for acute cholecystitis.

BACKGROUND The aim of this study was to evaluate the safety and usefulness of laparoscopic cholecystectomy after selective percutaneous transhepatic gallbladder drainage in patients with severe acute cholecystitis and patients with acute cholecystitis and severe comorbid disease. METHODS According to whether percutaneous transhepatic gallbladder drainage was performed before surgery, 133 patients with acute cholecystitis were divided into a percutaneous transhepatic gallbladder drainage group (n=60) and non-percutaneous-transhepatic-gallbladder-drainage group (n=73). Background factors, safety, and postoperative course were retrospectively evaluated and compared between these two groups. RESULTS Compared with the non-percutaneous-transhepatic-gallbladder-drainage group, the percutaneous transhepatic gallbladder drainage group was significantly older (p=0.0009), had a higher frequency of comorbid disease (p=0.0252), and a worse American Society of Anesthesiology classification (p=0.0021). In individual statistical tests, body temperature (p=0.0288), white blood cell count (p=0.0175), and C-reactive protein value (p=0.0022) were significantly elevated in the percutaneous transhepatic gallbladder drainage group; however, for frequency of comorbid disease, body temperature, and white blood cell count, significance was removed by correction for multiple testing of data. There was no significant difference in gender distribution, history of upper abdominal surgery, or body mass index between the two groups. The duration of surgery was marginally but significantly longer in the percutaneous transhepatic gallbladder drainage group (p=0.0414; in a single statistical test; however, that significance was removed by correction for the multiple testing of data). Between the two groups, there was no significant difference in blood loss at surgery, frequency of postoperative complications, rate of conversion to open laparotomy, interval until oral feeding was resumed, and length of postoperative hospital stay. CONCLUSIONS These data suggest that satisfactory outcomes can be achieved with selective pre-operative gallbladder drainage in older and sicker patients with acute cholecystitis.

Patient with perforation caused by emphysematous cholecystitis who showed flare on the skin of the right dorsal lumbar region and intraperitoneal free gas

We report an 84-year-old man with perforation caused by emphysematous cholecystitis who showed flare on the skin of the right dorsal lumbar region and intraperitoneal free gas. The patient was admitted for abdominal pain, abdominal swelling, and consciousness disorder 18 days after the onset. Abdominal computed tomography (CT) revealed emphysema in the gallbladder and a small amount of intraperitoneal free gas. Intraoperative findings suggested gangrenous cholecystitis. The gallbladder wall was perforated, and an abscess involving the right subphrenic region, the periphery of the liver and gallbladder, and the right paracolonic groove, was detected. The flare on the body surface may have reflected abscess formation in the right abdominal cavity. Emphysematous cholecystitis induces necrosis and perforation in many patients, and immediate strategies such as emergency surgery are important.

CHARGE association with neuroblastoma

The CHARGE association, choanal arresia or coloboma with multiple anomalies, is rare. A newborn boy with CHARGE association was referred to our hospital because of bilateral choanal atresia. Additionally, he had left renal aplasia, one of the anomalies associated with this syndrome. A right suprarenal neuroblastoma (stage IV) was diagnosed when the patient was 5 months old. At operation, great care was taken not to injure the right kidney, and resection of the primary tumor as well as radical dissection of the right renal hilar and para-aortic lymph nodes were performed without difficulty. The patient received postoperative chemotherapy with vincristine, cyclophosphamide, and adriamycin for 2 years and is alive without recurrence 5 years after the operation. No report of the CHARGE association with a malignancy has appeared in the literature. The relationship between the CHARGE association and malignant tumors is still obscure, so that further study is necessary to define it.