Iwao Yamagiwa

Severe hypertension and cardiac failure associated with neuroblastoma: A case report

The authors report on 3-year-old-girl with neuroblastoma complicated by severe hypertension and cardiac failure. She had cardiomegaly and pleural and pericardial effusions. Echocardiogram showed left ventricular hypertrophy and decrease of the left ventricular ejection fraction to 0.36 (normal > .40). Abdominal computed tomographic scan indicated a 7 x 7-cm tumor in the left suprarenal area. There was a marked increase in catecholamines and metabolites in her body fluids. After hypertension was controlled with doxazosin (a long-acting alpha 1 adrenergic blocker), her cardiac function gradually improved. A tumor was surgically removed and diagnosed as a poorly differentiated ganglioneuroblastoma. Preoperative differentiation between neuroblastoma and pheochromocytoma was not possible on the basis of catecholamine analysis or imaging studies including computed tomography scan and magnetic resonance imaging. It is important to control hypertension quickly in the patients with catecholamine-induced cardiomyopathy to facilitate surgical intervention for diagnosis and treatment.

Heterotopic Pancreas of the Esophagus Associated With a Rare Type of Esophageal Atresia

An infant with a rare type of esophageal/tracheal anomaly associated with heterotopic pancreas of the esophagus is herein reported. The upper pouch containing heterotopic pancreas reached 1.5 cm below the tracheal carina, and the distal esophagus connected to the trachea 2 cm above the tracheal carina and thus formed a partial duplication of the esophagus. Heterotopic pancreas of the esophagus is extremely rare, with only 7 cases previously reported. Here we report the combination of heterotopic pancreas and esophageal atresia with tracheoesophageal fistula.

Prenatally detected cystic neuroblastoma

Abstract A case of adrenal cystic neuroblastoma (NB) detected by prenatal ultrasonography (US) is presented. The suprarenal mass initially showed pure cystic features on a variety of imaging studies such as US, computed tomography, and magnetic resonance imaging. Tumor markers were negative. The mass was suspected to be an adrenal hemorrhage rather than a NB. Three months later, although the diameter was unchanged, the thickness of the cyst wall seemed to have slightly increased. Surgical exploration revealed an adrenal cystic tumor and histology showed a NB in situ. Forty-five infants with prenatally detected NB were found in the English literature; about one-half of them were cystic NBs, and most had a favorable outcome.

Novel breakpoints of the EWS gene and the WT1 gene in a desmoplastic small round cell tumor.

We report here a 15-year-old boy with an intraabdominal desmoplastic small round cell tumor (DSRCT). Cytogenetic analysis of the tumor cells showed the chromosomal translocation (11;22). Reverse-transcriptase polymerase chain reaction and sequencing analysis revealed a chimeric transcriptional message of the EWS gene exon 10 fused to the WT1 gene exon 8. The typical chimeric transcript seen in DSRCT is an in-frame fusion of EWS exon 7 to WT1 exon 8. The tumor in this case had a novel and longer chimeric transcript, which should be a potent transcription factor. Genetic analysis is a very powerful and specific aid in the differential diagnosis of small round cell tumors.

Chromosomal aberration in lipoblastoma: A case with 46,XX,ins(8;6)(q11.2;q13q27)

Chromosomal aberrations involving 8q11.2 have been reported in lipoblastoma. We report here a case of lipoblastoma with new chromosomal aberration. 46,XX,ins(8;6)(q11.2;q13q27). Cytogenetic analysis would facilitate the clinical differentiation between myxoid liposarcoma and the pathologically similar lipoblastoma and the identification of genetic loci related to cellular growth.

Choledochal cyst and duodenal atresia: a rare combination of malformations

Abstract. A rare case of congenital duodenal atresia (DA) associated with a choledochal cyst (CC) is reported. At 38 weeks of gestation, a 1,610-g girl was born by cesarean section with a prenatal diagnosis of congenital DA. After the disease was confirmed by radiographs, she underwent a duodenoduodenostomy for complete separation of the duodenum with an annular pancreas. Thirty-two months after the initial operation, she developed upper abdominal pain and acholic stools. Abdominal ultrasonography demonstrated a CC and dilated intrahepatic bile ducts. Magnetic resonane cholangiopancreatography showed an anomalous arrangement of the choledochus and the main pancreatic duct. A diffusely dilated extrahepatic bile duct was resected, and a hepaticoduodenostomy was performed after a cholecystectomy. The patient was discharged without complications. We could not find a similar case report in the English literature. Although it is not reported that there is a close relation of DA and CC in embryologic development, the presence of this combination should be considered.

Choledochal cyst and duodenal atresia: a rare combination

Abstract A rare case of congenital duodenal atresia (DA) associated with a choledochal cyst (CC) is reported.At 38 weeks of gestation, a 1,610-g girl was born by cesarean section with a prenatal diagnosis of DA. After the disorder was confirmed by X-ray, she underwent a duodenoduodenostomy for a complete separation of the duodenum with an annular pancreas. Thirty-two months after the initial operation, she developed upper abdominal pain and acholic stools. Abdominal ultrasonography demonstrated a CC and dilatated intrahepatic bile ducts. Magnetic resonance cholangiopancreatography showed an anomalous arrangement of the choledochus and main pancreatic duct. A diffusely dilatated extrahepatic bile duct was resected, and a hepaticoduodenostomy was performed after cholecystectomy. The patient was discharged without complications. We could not find a similar case report in the English literature. Although it is not reported that there is a close relationship in embryologic development of DA and CC, one should be aware of the possibility of this combination.

Intestinal pseudoobstruction in a neonate caused by idiopathic muscular hypertrophy of the entire small intestine

We report a case of intestinal obstruction in a neonate caused by massive muscular hypertrophy of the entire small intestine. Furthermore, the muscularis propria consisted of three layers, in most part. The pathogenesis of this apparently unique lesion is obscure.

Clinico-pathological studies on a transitional type between extrahepatic biliary atresia and paucity of the interlobular bile ducts

Among the neonatal and infantile cases of obstructive jaundice seen at Niigata and Yamagata University Hospitals between 1976 and 1990, extrahepatic bile ducts were visualized in 19 cases by either preoperative endoscopic retrograde cholangiopancreaticography (ERCP) or intraoperative cholangiography. Neonatal hepatitis was diagnosed in 3 of these cases by clear images of the bile duct system extending from the common bile duct to the intrahepatic bile duct. In 7 cases, the common bile duct was able to be seen, while the common hepatic duct was only slightly visualized. Four of these 7 cases were consistent with paucity of the interlobular bile ducts (PILBD) based on hepatic histology, while the remaining 3 showed fibrosis, bile ductular proliferation, and many bile plugs in the bile ductuli of the portal areas, concurrent with histological changes in extrahepatic biliary atresia (EHBA), not PILBD. In 9 cases, only the common bile duct was visualized while the common hepatic duct was not seen, 7 of these 9 cases being consistent with type III-al EHBA. In 2 cases, neither fibrosis nor proliferation of the bile ductuli was observed in the portal areas, and portal areas without any bile ductuli were also seen, in accordance with findings for PILBD. Three cases which showed similar hepatic histological findings to EHBA despite the presence of patent extrahepatic bile ducts, and 2 cases which had obstructed extrahepatic bile ducts and hepatic histological findings similar to PILBD, were thought to be of a transitional type between EHBA and PILBD. Thus, it is postulated that the entire bile duct system covering hepatocytes to extrahepatic bile ducts is affected by a certain factor, but the expression of individual disease types may be dependent upon the location of the site most seriously affected.

Renal atrophy following removal of a neuroblastoma

With the increased survival of neuroblastoma patients, there have been several reports of acute renal failure, atrophy, or disappearance of the unresected kidney after tumor resection in relation to the surgical procedures employed. Between February 1987 and February 1993, we experienced three cases of renal atrophy or disappearance folowing neuroblastoma removal that were unrelated to chemotherapy or irradiation. Two of them were caused by renal infarction due to disturbed renal blood flow during and after the surgery. Traction on the renal artery may cause renal artery spasm and endothelial damage, which may lead to stagnation of renal blood flow and finally to renal artery thrombosis. To prevent the occurrence of this complication, the renal artery should be handled gently to avoid traction, with no tape placed around it during excision of the tumor and para-aortic lymph nodes. The third case was caused by ureteric obstruction due to fibrosis around the silver clip placed at surgery. This complication may obviously be prevented by not using such clips.