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This report concerns two patients (female, 9 and 6 years) who were diagnosed with megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS). Although they exceeded the usual life expectancy of patients diagnosed with MMIHS because of total parenteral nutrition (TPN), they demonstrated progressive neurological deficits and showed histopathological features in the brain. Both patients were diagnosed with MMIHS in the neonatal period and were fed by TPN. The first patient developed visual and gait disturbances at the age of 7 years. Two months later, she developed dysarthria and muscular weakness, and could not maintain her posture. The level of serum selenium was extremely low. The second patient developed flexion and spasticity of the extremities followed by decorticate posture at the age of 3 years. Both patients died of sepsis. The brain weights of the two cases were 880 g and 715 g. In both cases, severe neuronal loss and gliosis were present in the medial convolutions of the occipital lobe, including the visual cortex. The postcentral gyrus and temporal transverse gyrus were also involved. In addition, extensive loss of Purkinje cells and granular neurons, and gliosis were observed in the cerebellum. We measured the selenium content of the brains and livers using the graphite furnace atomic absorption spectrometry method. Selenium was not detected in either brain, although the livers of both cases contained a low level of selenium. On immunohistochemical examination of the anti-oxidative enzymes, histiocyte-macrophage lineage cells in MMIHS cases, including microglia and Kupffer cells, showed only a weak reaction for glutathione peroxidase, of which selenium is an essential component. However, the cells in the control cases were strongly positive. In cases of MMIHS and methylmercury intoxication, the brain features similar lesions, in both their topographical and histopathological aspects. We considered that the brain lesions of the MMIHS patients mainly resulted from oxidative damage of the brain related to the low levels of glutathione peroxidase and other selenoproteins due to selenium deficiency.

Background: Surgical treatment of neuroblastomas, both those detected by screening and those detected clinically, in infants less than 12 months of age, is controversial, because some tumors in this age group potentially have the ability to regress spontaneously. Methods: From January 1985 to March 1997, the authors treated 50 infants (under 1 year of age) with neuroblastoma: 23 boys and 27 girls. Forty-one cases were detected preclinically by screening when the patients were 6 to 11 months of age (median, 7 months), and nine patients were discovered to have clinical manifestations at the age of 1 to 10 months (median, 4 months). Results: The tumor was INSS stage 3 or 4 in 10 patients (24%) with screening-detected tumor and in five (56%) with clinically detected tumor, although the difference was not statistically significant. Four screening-positive patients had multifocal primary tumors, and three of them were synchronous bilateral adrenal neuroblastomas. There was no statistically significant difference between the screening-detected tumors and the clinically detected tumors in biological characteristics such as Shimada's histology, DNA ploidy, and N- myc amplification. Complete resection of the primary lesion was accomplished by either primary surgery or second look (delayed primary) surgery in 46 patients (92%), and the resection was incomplete in the remaining four. In patients with bilateral adrenal tumors, the larger one was primarily resected, and the smaller contralateral tumor was enucleated or resected by partial adrenalectomy. Surgical complications included postoperative adhesive ileus (n = 2), Horner's syndrome (n = 2), renal atrophy (n = 1), renal failure (n = 1), phrenic nerve injury (n = 1), chylous ascites (n = 1), chylothorax (n = 1) and intussusception (n = 1). One patient died of respiratory failure caused by a complication, but 49 patients (98%) were alive at the time of evaluation. Conclusion: When considering surgical treatment of infants with biologically favorable neuroblastoma, the risk involved in treatment should be weighed against the risk inherent in a tumor capable of spontaneous regression, and aggressive surgery is unacceptable.

To determine whether dilated bowel proximal to obstruction associated with duodenal atresia and stenosis is related to feeding problems after a surgical correction of obstruction, the authors reviewed retrospectively the degree of bowel dilatation and disturbed transit as well as other clinical features in 18 duodenal atresia and 12 duodenal stenosis patients. A multivariate analysis was conducted to determine the possible correlation among them. The authors also evaluated the physiological function of the dilated bowel in duodenal atresia and stenosis patients (n = 8) by manometry of dilated bowel. The results were as follows. (1) In multivariate analysis, using the degree of disturbed transit as a dependent variable and using other clinical features as independent variables, the presence of postoperative complication and the existence of bowel dilatation 2 weeks after the operation were risk factors for disturbed transit. (2) One or two episodes of phase 3 were found in six of eight measured patients during the recorded period. The most distinctive manometric finding was the low contraction amplitude of both phase 2 and phase 3. These results indicate that dilated bowel was related to disturbed transit during the postoperative period, and that the low contraction amplitude of the dilated bowel was the main pathophysiological feature. The tapering or plication of dilated bowel might be indicated in patients with a markedly dilated bowel.

Hepatoblastoma, a malignant hepatic tumor in children, is thought to be an embryonal tumor resulting from developmental disturbances during organogenesis. Although factors that might be involved in the tumorigenesis have been suggested, an association between hepatoblastoma and the patient's birth weight has not been reported. We have accessed the data in the Japan Children's Cancer Registry and have analyzed patients' diagnoses and birth weights. During the 9 years from 1985 to 1993, 38 (0.38%) patients with tumors who weighted less than 1500 gm at birth were identified among 9923 registered patients. Hepatoblastoma was diagnosed in 9 patients of very low birth weight, representing 3.9% of the 231 patients with hepatoblastoma registered. A significant linear trend toward an increase in the percentage of patients with a birth weight of less than 1500 gm was observed specifically in hepatoblastoma (p = 0.0047). The percentage rose from 0.7% (1/138) in the 5-year period of 1985 to 1989 to 8.6% (8/93) in the next 4-year period (1990 to 1993). This increase was attributed to the significant increase in the percentage of patients who weighed less than 1000 gm at birth (p = 0.0028). A separate peak in the number of patients in the birth weight range of less than 1000 gm suggests that the cause of hepatoblastoma related to very low birth weight may be different from that of other patients. Full analysis of the patients' data is an urgent matter.

In January 1985, a single protocol consisting of cyclophosphamide, vincristine, tetrahydropyranyl adriamycin, and cis-platinum for the treatment of advanced neuroblastoma was begun nationwide in Japan and was found to improve clinical results significantly in terms of 2- or 3-year survival rate. Between January 1985 and December 1988, 113 eligible patients (7 infants younger than 12 months of age with stage IVA disease and 106 patients aged 12 months or older with stage III or IV disease) were enrolled and followed up for 5 years or more after initiation of treatment, as of March 1994. In this study, the usefulness of the protocol for the treatment of advanced neuroblastoma was evaluated with survival rates in relation to age, tumor site, stage, and N-myc amplification for patients surviving more than 5 years after initiation of the protocol. Fifty of the 113 patients were alive 5 years or more after initiation of the treatment, 39 without any episodes of disease recurrence. Fourteen (70%) of 20 patients with stage III, 6 (50%) of 12 with stage IVB, and 24 (30%) of 81 with stage IVA disease were alive and disease-free 5 years after initiation of the protocol. Twenty (56%) of 36 patients without N-myc amplification were alive at 5 years after initiation of the protocol. Only one patient who was alive without evidence of the disease at 5 years had recurrence afterward.

To seek evidence that tumor regression in neuroblastoma might result from massive apoptosis, we investigated tumor cell death in 39 neuroblastomas. Characteristic histologic features of apoptosis, condensed nuclear fragments and eosinophilic cytoplasm, were observed in all specimens. A ladder of DNA fragments induced by apoptosis was demonstrated by means of DNA agarose gel electrophoresis in 18 of the 19 tumors examined. In situ DNA nick end labeling (TUNEL) stained the nuclei with DNA fragmentation in 16 of 39 neuroblastomas. The TUNEL -positive cells were distributed in a scattered fashion in 10 tumors. In the remaining six tumors, they were densely located around nonviable areas of calcifications, where karyorrhectic or pyknotic cells were frequently observed. Five of six patients with such tumors were under 12 months of age, but there was no significant difference between the two groups in the patient age, origin of the primary lesion, or tumor stage. Biological features, including histology. DNA ploidy, and N-myc amplification, were not significantly different . Double fluorescent staining for bcl-2 oncoprotein and TUNEL showed that bcl-2 oncoprotein was expressed in the cytoplasm of tumor cells that were negative for TUNEL staining. This accumulated evidence suggests that massive apoptosis of tumor cells occurs in some neuroblastomas and may be related to tumor regression, whereas inhibition of apoptosis by bcl-2 oncoprotein expression might be associated with the tumorigenesis of neuroblastomas, as reported in our previous study.

A 11-month-old girl was noted to have hepatomegaly, and abdominal echography, CT scan and MRI revealed a huge tumor in the medial segment of the left lobe, located close to the hepatic hilum and involving the anterior segment of the right lobe. It was made clear that tumor resection was impossible at the exploratory laparotomy. We started the chemotherapy using THP-Adriamycin and Cis-platinum according to J·PLT-91B2 protocol. After four courses of chemotherapy, the tumor had regressed markedly and was resected with medial segmentectomy at second look operation. Postoperatively, the patient was treated with two courses of chemotherapy. The serum level of AFP is not within normal limits, but she is doing well without any evidence of disease 6 months after hepatectomy.

Fourteen patients with hepatoblastoma were treated at Gunma University Hospital and Gunma Children's Hospital Medical Center between 1966 and December 1992. The ages of the patients, 13 boys and 1 girl, ranged from 2 months to 10 years (median 1 year). The tumor was stage I in 2 patients, II in 5, IIIA in 2, IIIB in 4, and IV in one patient. The histological type was well differentiated in 6 and poorly differentiated in 4 (unknown in 4). Macroscopically, the tumor was resected in 9 of 11 patients who underwent operation. Intraarterial infusion of adriamycin or mitomycin C was done in 5 patients and embolization of the hepatic artery was also performed in 3. Chemotherapy was used in 11 patients but radiation (16-40Gy) only in 3. At the time of evaluation, January 1993, 7 out of 9 patients whose tumor was resected survived without any evidence of disease. Six patients died of disease between 1 and 8 months after treatment. One patient died of recurrent disease 5 years after treatment. Postoperative complications included portal hypertension with esophageal varices. Hemihypertrophy was observed in 2 patients.

Thirteen patients with nephroblastoma, including one with unfavorable histology and one mesoblastic nephroma, were treated at Gunma University Hospital and Gunma Children's Hospital Medical Center between 1971 and December 1988. The ages of the patients, 6 boys and 7 girls, ranged from 3 months to 11 years (median 2 years). The stages were I in 4 patients, II in 2, III in 5, and IV and V in one patient each. Nephrectomy was done in 12 patients and rupture with tumor spillage was experienced in 3. Chemotherapy consisted of actinomycin D and vincristine before 1987. Radiation (16-40. 5Gy) was given in 11 patients. After 1987, postoperative therapy was performed according to the NWTS-2 regimen. At the time of evaluation, January 1992, 11 out of 13 patients survived without any evidence of disease. Complications of treatment included scoliosis of the spine, atrophy of the chest and abdominal wall and postoperative intestinal obstruction after nephrectomy. Recent advances in the understanding and management of nephroblastoma are discussed.

The authors report a peculiar form of intrahepatic multiple cysts of the biliary system in a patient with biliary atresia (BA). An 11-year-old girl was admitted to our institution to be investigated for repeated cholangitis occurring after the age of 10 years. She underwent a hepaticojejunostomy, caused by Type I (cyst-type) BA, in the neonatal period. The radiological examination results showed multiple cystic dilatation of the intrahepatic biliary system with a vascular structure protruding into the cyst lumen. Such cystic dilatation with a protruding vascular structure has been noted in patients with Caroli's disease and congenital hepatic fibrosis, and ductal plate malformation is shown to be responsible for the cyst formation. The authors postulate that such malformation of the intrahepatic biliary system is related to the cyst formation in our case.

To evaluate liver function in biliary atresia (BA) patients after the Kasai operation, magnetic resonance imaging (MRI) was carried out 28 times in 19 BA patients. Sixteen of these were divided into three groups on the basis of the serum level of total bilirubin (t-bil), glutamic pyruvic transaminase (GPT) and gamma-glutamyltransferase (gamma-GTP) in the postoperative follow-up period (2-11 years). In group 1 (n = 7) the t-bil was continuously kept under 1 mg/100 ml. In group 2 (n = 4) the t-bil was continuously kept under 1 mg/100 ml, but GPT and gamma-GTP remained high (GPT > 100 IU/liter, gamma-GTP > 200 IU/ liter) for more than 2 years. In group 3 (n = 5) an increase in the t-bil level reappeared (1-2 mg/100 ml; n = 4, > 2 mg/100 ml; n = 1). The differences between MRI finding in the 3 groups, and the correlation between MRI findings and laboratory data (t-bil, cholinesterase; ChE, GPT and gamma-GTP), which were taken around the time of MRI examinations, were studied. The results were as follows: (1) All 19 patients had normal or high signal areas of various sizes on T1 weighted images (WI). (2) Eighteen of 19 patients had high signal areas of various sizes on T2 WI in the portal system areas and/or liver parenchyma, and these areas were enhanced by gadolinium-DTPA in about half of the patients. (3) Ten of 16 patients had atrophic change over one liver lobe. (4) MRI findings for group 1 and the other groups were significantly different, and MRI findings, except for atrophic change, were correlated with the increase or decrease in laboratory data taken around the time of MRI examinations (p < 0.05). These results indicate that a normal or high signal area on T1 WI shows functional tissue, and that a high signal area on T2 WI shows tissue damaged by inflammation and/or progressive fibrosis. MRI is useful for evaluating liver function, especially in terms of morphological features, in BA patients after the Kasai operation, and will be one method for establishing their prognosis.

To investigate the possible clinical application of the hypothesis that insufficient induction of 3,4-dihydroxyphenylalanine decarboxylase (DDC) causes accumulation and secretion of 3,4-dihydroxyphenylalanine (DOPA) in unfavorable neuroblastomas, we measured plasma DOPA in 28 neuroblastoma patients. Abnormally high levels were demonstrated in patients with neuroblastoma, and the levels in patients with clinical manifestations (median, 44,800 pg/ml; range, 17,700 to 220,000 pg/ml; n = 6) were significantly higher than those in patients detected by screening (median, 5825 pg/ml; range 2890 to 33,300 pg/ml; n = 22) (P = 0.0004). The catecholamine secretion profiles of the two groups were different, and it was suggested that the relative deficiency of DDC caused DOPA secretion in patients in the former group, whose prognosis was unfavorable, except in one case. In both groups, serial determination of plasma DOPA was a good monitor of the disease course. The higher plasma DOPA level (9400 pg/ml) was significantly correlated with the patients' age (1 year old) (P = 0.019), tumor stage (III, IV) (P = 0.029), and DNA diploidy (P = 0.018). These results are consistent with previous studies that demonstrated plasma DOPA was a useful marker in the diagnosis and follow-up of neuroblastoma. The results also indicate that higher plasma DOPA levels are associated with the unfavorable characteristics of neuroblastoma, which seem to support the hypothesis on DDC deficiency in unfavorable neuroblastoma.

Small bowel obstruction, excluding postoperative adhesive ileus, in patients > 1 month old treated between June 1982 and May 1992 at Gunma Children's Hospital Medical Center is reviewed. There were 32 patients, 22 boys and 10 girls, whose ages ranged from 1 month to 6 years (median 9 months). Intussusception was the most frequent cause of obstruction and was seen in 17 patients (53.1%). Causative lesions were identified in five patients, and were ileal duplication cysts in four and Meckel's diverticulum in one. Incarcerated inguinal hernia and mesenteric cysts resulted in bowel obstruction in six and three patients, respectively. Other causes included mesodiverticular band, ileal volvulus without malrotation, abnormal adhesion of omentum, abnormal band, vitelline duct remnant and trapping in a mesenteric defect. As for the age distribution, there was no significant correlation between the causes of obstruction and the age of patients. Ultrasonography was useful in differential diagnosis, and this modality should therefore be used in every patient with signs of small bowel obstruction.

The role of surgery was evaluated in 19 stage III and 102 stage IV neuroblastoma patients, all of whom were treated with intensive induction chemotherapy by the Study Group of Japan between January 1985 and March 1990. For stage III neuroblastoma, surgical intervention at the primary site was performed in 18 of the 19 patients, 9 during and 9 after the first three cycles of A1 regimen, consisting of high-dose cyclophosphamide, vincristine, THP-adriamycin, and cis-platinum. Gross complete resection of primary tumor and regional lymph nodes was feasible in 17 of the 19 patients (89%), and the survival rate for the 17 patients were 79%, 70%, and 70% at 2 years, 3 years, and 4 years, respectively. For stage IV, surgical intervention at the primary site was performed in 92 of the 102 patients (90%): 30 cases during the first 3 cycles of A1 chemotherapy and 62 cases after that, with gross complete resection accomplished in 81 of the 102 patients (79%). The 81 patients with gross complete resection achieved had a better prognosis than those 11 patients with partial resection (P less than .05). Overall survival rate was 62% at 2 years for 27 patients who underwent complete resection after 3 cycles of A1 when resolution of all metastases was obtained, whereas the survival was 52% at 2 years for 31 patients who similarly underwent complete resection but when evidence of persistent metastases was present. Patients in whom the ipsilateral kidney was preserved at surgery had an outcome superior to that of those with associated nephrectomy (P less than .05).(ABSTRACT TRUNCATED AT 250 WORDS)

In 1971, the Japanese Society of Pediatric Surgeons' Committee on Malignancies proposed new criteria for neuroblastoma staging. It was fundamentally, based on the system of Evans et al. described in 1971. The main difference was the separation of stage IV disease into stages IV-A, with metastases to bone, orbita, distant lymph nodes and viscera other than liver, IV-B, the primary tumor extending over the midline and with metastases to bone marrow, liver and skin, and IV-S, which was the same as that of Evans et al. The new criteria did not include the resectability of the primary tumor, assessment of regional lymph node involvement or any other disease assessment resulting from therapeutic intervention. For the purpose of international usage, the Japanese system has been newly formulated and proposed as the Japanese Tumor Node Metastasis (TNM) Postsurgical Histopathological Classification for Neuroblastoma. In the present report, 495 neuroblastomas, registered between 1970 and 1985, were analyzed retrospectively according to the International Union Against Cancer (UICC) TNM classification and the proposed Japanese TNM system. The analyses suggested that the Japanese system reflected both the extent of tumor invasion and its biological neuroblastoma characteristics better than the UICC TNM classification based on statistical analysis.

Neuroblastoma (NB) is a tumor which arises from neural crest cells. In the developing neural crest cells, the induction of 3, 4-dihydroxyphenylalanine (DOPA) decarboxylase is more delayed than that of tyrosine hydroxylase and dopamine-β-hydroxylase. If NB cells are arrested in an early stage of neural crest development, the induction of DOPA decarboxylase is insufficient and the accumulation and secretion of DOPA can be caused. The biochemically immature phenotype is thought to represent the undifferentiated characteristics of the cells and might correlate with the grade of malignancy. To investigate whether the hypothesis is clinically applicable or not, we have measured plasma DOPA, dopamine and urinary catecholamine metabolites in NB patients. The levels of plasma DOPA, dopamine, urinary homovanillic acid (HVA) and vanillactic acid (VLA) were significantly higher in patients with unfavorable NBs and the higher plasma DOPA level was significantly associated with the patients' age (>1 year old), tumor stage (III, IV) and DNA diploidy. Serial determination of plasma DOPA was a good monitor of the disease course. These results are compatible with the hypothesis on DOPA decarboxylase deficiency and DOPA secretion in undifferentiated, unfavorable NBs. In conclusion, the plasma DOPA can be used to predict patients' prognosis as well as to follow up patients with NB. (Hypertens Res 1995; 18 Suppl. I: S209-S210)

A retrospective clinical analysis was carried out on 39 patients with neuroblastoma treated from 1960 to June, 1988. Survival rate was analyzed according to stage, primary site, histology and age. Two-year survival was as follows : stage I, II and IVS, 100% ; stage III, 53% ; stage IVB, 40% : and stage IVA, 0 %. For all stages, patients younger than 1 year of age survived longer than those older than 1 year of age (p

A case of perforation of the cecum in a two-day-old boy was presented. He started to vomit green bile from the night of the birth day. As his abdomen became distended on the second day of life, he was transfered to our institutions. A scout film of the abdomen on admission to the Tomioka-Kosei Hospital showed free intraperitoneal air with gas distension of stomach and upper intestine. There were a few scattered gas shadows in the right lower quadrant of the abdomen but no further gas shadow was visible, and the dilatation of a loop of jejunum in the left upper quadrant was noted. Under the preoperative diagnosis of a diastatic perforation of the small bowel due to congenital stenosis or atresia, the emergency celiotomy was carried out. On laparotomy, a large amount of purulent ascites with meconium gushed out. A segmental dilatation of the upper jejunum was found, but the small bowel tapered gradually toward the terminal ileum and neither atresia nor stenosis was found. By means of the systematic examination of the gastrointestinal tract, a perforation of the anterior wall of the cecum with contracted small caliber colon suggestive of an entire colon aganglionsis was found. The perforated cecum was exteriorized and the abdominal cavity was irrigated and drained. The appendix was removed for histological verification of the ganglion cells and it was reported as aganglionic. Postoperative course was uneventful, however, the postoperative barium enema revealed the small caliber colon, but the delayed film demonstrated almost normal evacuation of the contrast material contradicting to the aganglionosis. Thus, the etiology of this perforation of the cecum is unknown at present.

Meconium peritonitis is an aseptic chemical peritonitis caused by spill of aseptic meconium in the peritoneal cavity due to perforation of the gut during the last 6 months of the gestation and early postnatal period. It has grave prognosis and only 15 survivors had been reported in Japanese literatures prior to 1975.A case of fibroadhesive type of the meconium peritonitis in a five-day-old male infant was presented. He was born in June 2, 1974. No abnormalities were found until he was in the 6th day of life, when he began to vomit bile stained fluid. He became distended by the next day and was transfered to the Gunma University Hospital. The scout film on admission (Fig. 1) revealed, a round calcium fleck in the subhepatic area with distended small bowel gas shadows. At laparotomy performed on the day of admission, dense calcified fibrous adhesions were found throughout the abdomen. No free perforation could be found. The adhesion was dissected out in the right lower abdomen and a side-to-side anastomosis was carried out between the bulbous distended portion of the small bowel and the terminal ileum. Immediate postoperative course was uneventful and he was discharged on June 26. However, he was readmitted on 83rd p.o. day in another hospital with fever and jaundice and died of an acute yellow liver atrophy caused by post-transfusion hepatitis on the 88th p.o. day. Hepatitis B antigen was demonstrated by an orcein method in the liver of the autopsied material.

A case of atresia of the ascending colon in a 2-day-old male infant with recovery was presented. The patient was born on Aug. 26, 1964. He started to vomit bile-stained vomitus on the second day of life and had not passed meconium.On admission, he was dehydrated and distended. The right side of the abdomen was somewhat bulgy and a elliptical doughy mass was felt. A flat and upright film showed marked distention of multiple loops of intestine with air fluid levels. There was an oval shaped gigantic intestinal gas shadow in the right half of the abdomen. Barium enema revealed microcolon with the radio-opaque material terminating at the splenic flexure. Emergency laparotomy was performed with the presumptive diagnosis of colonic atresia. A type III atresia of the ascending colon was confirmed at surgery. The ascending colon, including 10cm. of terminal ileum was resected and ileosigmoidostomy was performed. His postoperative course was uneventful and he was well in seven years after surgery.Congenital atresia of the colon is rare cause of intestinal obstruction in the newborn infant. During the year 1958 to 1973 inclusive, 32 infants have been operated upon for intestinal atresia at the Department of Surgery of Gunma University Hospital, and only 2, or 6 per cent, was located in the colon.

A case of pneumatic rupture of the stomach in a nineteen-month-old boy was presented. The patient who was going to be operated upon the right inguinal hernia, suddenly developed marked abdominal distention during the induction of general anesthesia under G.O.F. with a mask. The distention was not benefited by passage of a nasogastric tube. X-ray in the supine position revealed massive pneumoperitoneum showing Miller's “foot ball” sign or “air dome” sign. The pneumoperitoneum was much more evident in the confirmatory upright film.Laparotomy was performed three and a half hours after the onset of abdominal distention. At laparotomy incision of the peritoneum released a large quantity of free air, however, no apparent perforation was found in either the stomach or the small intestine, and abdominal viscera were seemed to be intact except the small hemorrhagic and emphysematous area in the lesser omentum at the junction to the stomach near cardia. The lesser omentum was detached from the stomach and a 2.5cm. linear tear was discovered along the lesser curvature. The rent was sutured in two layers and peritoneal cavity was thoroughly irrigated with warmed sterile saline. No local antibiotics was instilled and on drainage was employed. Postoperative course of the patient was uneventful.

X-ray films of gastric rupture in the newborn infant were presented with a brief comment.Case 1 : 1-day-old girl (birth weight 2, 800g.). She developed marked abdominal distention and cyanosis immediately after birth. An up-right scout film of the abdomen revealed massive pneumoperitoneum (Fig. 1) and the absence of the intestinal gas. The diagnosis of the gastric perforation with intestinal atresia was made. A linear tear, 2 cm, was found along the greater curvature high in the fundus. Atresia of the intestine was not confirmed at surgery. The gastric rent was closed in two layers and abdominal cavity was drained. The patient died shortly after operation. Permission for autopsy was refused.Case 2 : 6-day-old boy (birth weight 3, 100 g.). There had been no difficulties with delivery and no gastric intubation or attempts at resuscitation. On the 6th day of life, he become dyspneic and developed abdominal distention. The plain up-right film of the abdomen revealed pneumoperitoneum. Laparotomy disclosed a 4 cm. linear tear along the greater curvature from the fundus to near the antrum. The rent was closed in two layers and abdominal cavity was drained. He expired on the 1st postoperative day. Postmortem examination revealed generalized peritonitis. The sutured areas in the stomach wall were intact.

The TNM classifications of neuroblastoma, nephroblastoma and soft tissue sarcoma were adopted at the International Conference for TNM Classification (UICC) held in May 1980. There is no TNM system under contemplation, however, for primary liver carcinoma in childhood. Accordingly, we have formulated the proposed Japanese TNM system for this carcinoma in children and examined its validity in 136 cases of hepatoblastoma seen in the listed 14 institutions. The basic policy of the Committee on the Japanese TNM Classification is not to include the resectability of the tumor and regional lymph nodes or any other status of the disease resulting from therapeutic intervention as a component of the pTNM system. This is a feature which makes our proposed system widely divergent from the accepted classification scheme for the three types of tumor cited above.

A case of gigantic liposarcoma of the breast in a 52-year-old woman was reported. She had a small indolent mass in the left breast for twenty years. The mass grew rapidly from the summer of 1979 and became gigantic and penetrated the skin over the mass. Her general condition on admission was poor but resection, of the tumor was carried out. She suddenly died of cardiac failure with electrolytes imbalance 13 days after the operation. The diagnoses of liposarcoma, predominantly myxoid, was made on the basis of light and electron microscopic findings. Forty cases of liposarcoma of the breast were collected from the literature. The possibility of malignant trans-formation of a benign tumor was suggested.

This is a case report of a cystic duplication of the cecum in a 6-month-old female infant, which was lined by both columnar and squamous epithelium. This squamous epithelium did not resemble esophageal epithelium but dermal epithelium. The so-called split notochord theory can explain the possibility of the coexistence of ectoderm and entoderm components. However, the present case had no vertebral abnormalities.

In the newborn, traumatic rupture of the spleen is rare. Only 24 survivors have been reported in the world literatures, including four cases which have appeared in the Japanese literature. All were treated by splenectomy.‘-3 In view of the danger of overwhelming infection following splenectomy in early childhood,4 preserving whatever splenic tissue remains vascularized is indicated. SeraS reported two infants in whom pneumococcal meningitis occurred after splenectomy in the neonatal period for splenic rupture. The first survivor of a ruptured spleen treated by repair of the organ is reviewed.