Hepatoblastoma--evolution of management and outcome and significance of histology of the resected tumor. A 31-year experience with 40 cases.

BACKGROUND/PURPOSE: The aim of this study was to retrospectively evaluate and compare the clinical features, treatment strategy, pathology, and outcome of all patients with hepatoblastoma treated at an African hospital over a 31-year period (1970 to 2001). METHODS: Forty patients with hepatoblastoma were divided into 3 groups according to the treatment given. Group I (1970 to 1983, 14 patients) had no protocol therapy; group II (1984 to 1988, 6 patients) received protocol treatment according to Children's Study Group (CCSG) guidelines; group III (1989 to 2001, 20 patients) received SIOPEL protocol therapy. All available clinical, surgical, radiologic, and pathologic data were reviewed and analyzed. RESULTS: Overall patient survival was as follows: group I, 14%; group II, 50%, and group III, 80%. Deaths in group II were caused by chemotherapy-induced immunosuppression only. Prognostic data for group III showed that all tumor-related deaths could be predicted by identifying multifocal disseminated growth patterns (P =.001) or vascular invasion (P =.001) in resected tumors. Of the 40 diagnostic tumor biopsies performed, 2 significant complications (1 death, 1 intraperitoneal tumor seeding) occurred. Histologic criteria evaluating these biopsies were not predictive of overall survival. CONCLUSIONS: The introduction of protocol therapy has resulted in a marked improvement in survival. Immunosuppression-related sepsis in our setting resulted in unacceptable mortality in patients treated according to CCSG guidelines. A diagnostic biopsy in hepatoblastoma is of value but not without complications. Preoperative chemotherapy followed by complete surgical excision according to International Society of Paediatric Oncology guidelines yields excellent results with a current survival rate of 80%.

Interdigitating dendritic cell sarcoma: a report of four paediatric cases and review of the literature.

AIMS: To report a series of four paediatric cases of interdigitating dendritic cell sarcoma (IDCS) and add to the known extranodal sites of occurrence for this tumour. Neoplasms derived from interdigitating dendritic cells are rare, with only 33 cases being reported in the literature (Medline search). These tumours usually occur in lymph nodes in the adult population. METHODS AND RESULTS: The patients were a 10-year-old girl with a large soft tissue mass bulging into the left chest, a 12-year-old girl with a right paraspinal mass, a 21-month-old boy with generalized lymphadenopathy and hepatosplenomegaly and a 6-year-old girl with a large bladder mass. Paraffin blocks and haematoxylin and eosin slides were available in all cases. In addition, immunohistochemistry and electron microscopy were performed. A diagnosis of IDCS was made in all cases. CONCLUSION: The diagnosis of IDCS can rarely be entertained on clinical information alone. Microscopically, there is a wide spectrum of features. Thus, immunohistochemistry and electron microscopy are crucial in making the diagnosis. The differential diagnosis includes inflammatory pseudotumour, follicular dendritic cell sarcoma, true histiocytic lymphoma, malignant Langerhans cell histiocytosis, anaplastic large-cell lymphoma, melanoma, and a range of sarcomas. IDCS displays aggressive behaviour and approximately half of the patients die of the disease.

Intrahepatic fetus-in-fetu: a case report and review of the literature.

The authors report the third case since 1964 of fetus-in-fetu presenting at Red Cross Children's Hospital. A fetal liver abnormality was detected during routine antenatal ultrasound scan, and the postnatal diagnosis of an intrahepatic, multiple fetus-in-fetu was made with abdominal ultrasound scan, plain radiograph of the abdomen and computed tomography. The mass was removed surgically by hemihepatectomy when the patient was 4 months of age. Imaging and the macroscopic and microscopic appearances of the abnormality confirmed the diagnosis and amplified it to include an adjacent teratoma. Multiple small subcapsular and perimass vascular hamartomas were coincidentally discovered in the liver. Two of the 3 nodules within the amniotic-lined cavity of the intrahepatic mass individually comply with the accepted criteria for fetus-in-fetu, and the third nodule has the accepted features of a benign teratoma. The existence of multiple fetus-in-fetu and fetus-in-fetu combined with a teratoma, is recorded in the medical literature. The imaging as well as the macroscopic and microscopic features are illustrated.

Sacrococcygeal germ-cell tumours--the Red Cross War Memorial Children's Hospital experience, 1980-1996.

OBJECTIVE: To document the experience of Red Cross War Memorial Children's Hospital in the treatment of sacrococcygeal germ-cell tumours. PATIENTS: Twenty-seven patients with sacrococcygeal germ-cell tumours were treated in our hospital from 1980 to 1996. DESIGN: A retrospective review of these patients' records was undertaken. RESULTS: There were 19 female and 8 male patients. Seventeen (63%) presented in the neonatal period, 13 on the first day of life. Complete surgical resection of the tumour was achieved in all patients with mature or immature teratomas (20 patients) and in 2 neonates with malignant tumours. The first of these 2 neonates, with a malignant teratoma, was not given chemotherapy and remains well 10 years later. The second, with a yolk-sac tumour, also received no initial chemotherapy. He relapsed at the age of 9 months and was successfully treated with repeat excision and chemotherapy. All 5 patients first diagnosed after the age of 1 year had malignant tumours. These patients had incomplete surgical resection (3) or biopsy only (2), and 3 were successfully treated with chemotherapy. One patient relapsed with yolksac tumour after initial complete resection of a mature teratoma. She was successfully treated with repeat surgery and chemotherapy.

Congenital stenotic arteriopathy with medial dysplasia of aorta, pulmonary artery, and their major branches.

This report describes the morphological findings in a young child with congenital stenotic arteriopathy who died suddenly following arteriography. Hyperplasia of all of the medial components had produced severe thickening of the wall of the aorta (mean number of lamellar units = 133 in the thoracic aorta and 125 in the abdominal aorta), the pulmonary artery, and their major proximal branches, resulting in significant luminal narrowing. Bilateral renal artery stenosis, attributable mainly to intimal longitudinal smooth muscle hyperplasia associated with fibroelastosis, was the cause of her systemic hypertension. The left ventricle showed healed subendocardial infarction.

Idiopathic annular submitral aneurysm associated with Takayasu's aortitis. A report of two cases.

The pathologic findings in two patients with idiopathic annular submitral left ventricular aneurysms and coexistent Takayasu's aortitis are documented. The evidence of chronic persistent myocarditis in one patient and marked myocardial fibrosis in the other supports two theoretical possibilities: first, the aneurysms may have an inflammatory etiology and, second, a common inflammatory process may have accounted for both the myocardial and the aortic lesions.

Morphologic features of the myopathy associated with chronic renal failure.

Our previous studies indicate that impaired function of skeletal muscle limits the exercise tolerance of patients with end-stage renal failure who are either maintained on dialysis or undergo renal transplantation. To study the morphology of the condition, muscle biopsies were performed on eight patients with renal failure-associated myopathy. Control samples were taken from seven healthy athletes undergoing knee surgery and from five otherwise healthy but untrained subjects. Tissues were examined by routine light and transmission electron microscopy. Histochemical staining of frozen sections for myosin adenosine triphosphatase and quantitative computer-assisted morphometry of the fiber type and size was performed. The mean (+/- SD) size for type I fibers in patients was 61.2 +/- 11.8 microns, while type II fibers measured 46.7 +/- 11.4 microns. The mean percentage of type II fibers was 67% +/- 12%. These values are within the normal population range and were not different from controls. Significant changes were found on light microscopy of patient samples. These included fiber splitting, internalized nuclei, nuclear knots, moth-eaten fibers, fiber degeneration and regeneration, increased content of lipid droplets, and fiber-type grouping. Electron microscopy showed a large variety of nonspecific abnormalities, including mitochondrial changes, Z-band degeneration, myofilament loss, and accumulation of intracellular glycogen. Ten of 12 control subjects showed no such changes; minor abnormalities were noted on both light and electron microscopy in the remaining two subjects. Muscle oxidative capacity (19.5 +/- 5.1 microL O2/min) for patients with end-stage renal failure was not different from values for those who had undergone renal transplantation, but was lower than values found in trained athletes.(ABSTRACT TRUNCATED AT 250 WORDS)

The role of chorioamnionitis and prostaglandins in preterm labor.

Spontaneous preterm labor remains a significant cause of high morbidity and mortality in the newborn. Chorioamnionitis with an associated rise in prostaglandins (PGs) is thought to be one of the factors responsible for the onset of preterm labor. In this study, 52 placentas and membranes from gestations of 35 weeks' or fewer were collected. Tissue samples of membranes and placentas were incubated in pseudoamniotic fluid, and the levels of PGs and leukotriene B4 were assayed. Swallowed amniotic fluid aspirated from the infants' stomachs was analyzed for PGs and examined microscopically for the presence of degenerate neutrophilic polymorphonuclear leukocytes (pus cells) and bacterial organisms. Prostaglandins E and F and leukotriene B4 production were significantly higher in the membranes and placentas with chorioamnionitis than in those without. Although the comparison of PG levels in the gastric fluid of infants with and without chorioamnionitis did not reach statistical significance, there was a trend toward higher levels of PGs with chorioamnionitis. Prostaglandin E levels from membranes and placentas and PGF from placentas were significantly elevated in the gastric fluid of infants with pus cells and organisms. These findings support the hypothesis that chorioamnionitis may initiate preterm labor.

Placentae of light for dates infants born to underweight mothers at term: a morphometric study.

Fifteen light for dates infants and their placentae were compared to 15 well-grown infants and their placentae. The former were born to thin, underweight women while the latter were born to women of normal weight. The light for dates infants were symmetrically growth retarded but not wasted at delivery and their placentae had a reduced weight, volume, chorionic surface area, percentage parenchyma and total villous surface area. The peripheral villous surface area and volume of peripheral villous trophoblast, fetal capillaries and connective tissue was also reduced in the placentae of light for dates infants, suggesting retarded placental growth in the latter half pregnancy. In contrast, the stem villous surface area and volume of stem villous trophoblast, fetal capillaries and connective tissue was similar in both groups of placentae, suggesting the same rate of growth in early pregnancy. There were no differences in the volume of fibrin or infarcts. The ratio of total villous surface area to infant weight, length and head circumference was reduced in the light for dates infants. This may restrict the materno-fetal oxygen exchange, and thereby increase the risk of fetal hypoxia during labour. It is concluded that the placentae of light for dates infants born at term to underweight women are both absolutely and relatively small with a reduced villous surface area.

Phakomatous choristoma of the lower eyelid. A light and ultrastructural study.

This article documents the eighth reported case (to our knowledge) of a phakomatous choristoma, a rare congenital tumor of the lower eyelid of infants. Among the eight cases, there was no instance of tumor recurrence recorded nor was there any detectable postoperative eye defect, despite incomplete resection of the tumor in two patients. While usually clinically diagnosed as a dermoid cyst, the highly characteristic histologic features of this entity should allow more frequent recognition. Ultrastructural examination in our case provided additional evidence of its lenticular origin.

Chorio-amnionitis in relation to mode of delivery at term.

The incidence of inflammatory changes on histological examination in the placenta, membranes and umbilical cord of 50 infants born by spontaneous vaginal delivery were compared with those of 50 infants born by elective caesarean section before the onset of labour at term. Inflammation was significantly more frequent after vaginal delivery (28%) than after caesarean section (6%). This suggests that intra-uterine bacterial colonisation is uncommon before the onset of labour and it is argued that chorio-amnionitis in the vaginally delivered placentas occurred during labour.

Intracisternal paracrystalline serpigenous inclusions: a marker of disordered fibroblastic proliferation.

Three young children with characteristic fibroblastic tumors are presented. These comprised one case each of congenital fibrosarcoma, dermatofibrosarcoma protuberans, and fibrous hamartoma of infancy. Ultrastructural examination in each tumor revealed the presence of a peculiar intracisternal paracrystalline material that exhibited transverse periodicity between 18 and 22 nm. Elongated profiles lying within rough endoplasmic reticulum had a serpigenous outline. It is thought that this material is related to procollagen or its precursors and its presence may signify disorders of fibroblastic proliferation that may occur in fibrous tumors.

Amniotic fluid infection syndrome in private and non-private patients.

The incidence of placental histological evidence of amniotic fluid infection syndrome (AFIS) was studied in two groups of patients delivered at term. One group received private medical care during pregnancy, while the other patients were delivered at a midwife obstetric unit. There was no significant difference in histological evidence of AFIS in the private patient (22%) and non-private patient (28%) groups.

Pathogenesis of adenocarcinoma complicating ureterosigmoidostomy. Experimental observations.

Columnar metaplasia, dysplasia, and adenocarcinoma occurred in patches of bladder epithelium exposed to the fecal stream in rats. This suggests that such tumors complicating ureterosigmoidostomy may arise from ureteric epithelium, and that urine is not essential for their development.

Amniotic fluid infection syndrome and abruptio placentae.

The incidence of amniotic fluid infection syndrome, as assessed by the presence of an acute inflammatory infiltrate of the placenta, umbilical cord, and membranes at birth, was 23.3% in pregnancies complicated by severe abruptio placentae. This was not significantly higher than that noted in uncomplicated pregnancies. These data therefore fail to support the proposal that amniotic fluid infection predisposes to placental abruption.

Dysplasia of ureteric epithelium: a source of adenocarcinoma in ureterosigmoidostomy?

Dysplasia was found in the terminal portion of the ureters in two children with ureterosigmoidostomy followed by routine colonoscopy. These observations provide further evidence that adenocarcinomas complicating this type of diversion may be derived from ureteric epithelium and that urine is not essential for their development.

Secretory IgA synthesis in Kwashiorkor.

The synthesis of intestinal secretory IgA was studied in in vitro cultures of duodenal mucosal biopsies from children with Kwashiorkor. Production of secretory IgA was measured by the incorporation of radioactive label and visualized following PAGE and autoradiography. Results obtained before and after nutritional rehabilitation demonstrate an enhanced synthesis of sIgA in children with acute Kwashiorkor. Histological examination of plasma cells in the biopsy tissue confirms a twofold increase in IgA staining plasma cells in acute Kwashiorkor. Peripheral blood B lymphocytes in acute Kwashiorkor however, showed a reduction in IgA synthesis in the acute stage. These results suggest an effective mucosal sIgA response to the increased intestinal antigen load in Kwashiorkor.

Complement activity in the cord blood of term neonates with the amniotic fluid infection syndrome.

Cord blood samples from 11 term neonates whose placentas showed histological changes typical of the amniotic fluid infection syndrome were analysed in order to determine haemolytic activity of the classic and alternative complement pathways and serum levels of complement proteins and immunoglobulins. Although the mean values of all these parameters were higher in this group than in an age-related control group, only classic haemolytic pathway activity was significantly elevated (P less than 0,025).