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Twenty-two patients with metaphyseal primary malignant bone tumors (17 osteosarcomas, 5 Ewing's tumors) occurring before closure of the growth plate were examined with plain radiographs and MRI in order to determine the physeal or epiphyseal extent of the tumor. Results were correlated with the pathologic examination. Transphyseal spread was pathologically proven in 13 cases (59%): 12 cases of osteosarcoma and 1 case of Ewing's tumor (70% and 20%, respectively). There was no significant relation between epiphyseal invasion, age of patient, length of tumor or, in the cases of osteosarcoma, response to chemotherapy. Plain radiographs showed epiphyseal involvement in 4 cases and there were 10 false negatives. MRI revealed epiphyseal involvement in all cases; there were no false positives or false negatives. T1-weighted images in coronal or sagittal planes appeared to be sufficient. These findings are very useful in planning surgical limb salvage procedures and stress the ineffectiveness of the "barrier effect" of the growth plate against tumor spread.

Splenic hemangioma is a very rare neoplasm in children. We report three cases of splenic hemangioma, two of solitary hemangioma and one of hemangiomatosis. Two patients had hematologic disorders related to hemangioma. Imaging findings of this entity are presented and discussed with emphasis on pathologic correlation.

Three children displaying hypotonia, cardiac involvement and defects of the mitochondrial respiratory chain complexes are reported. The first case showed severe neonatal hypotonia, failure to thrive, hepatomegaly, dilation of the right cardiac cavities, profound lactic acidosis and amino aciduria. The boy died at the age of 7 weeks. In the second case hypotonia, severe cardiomyopathy, cyclic neutropenia, lactic acidosis and 3-methylglutaconic aciduria occurred. The boy died at the age of 27 months. The third case presented at the age of 16 months as an acute hypokinetic hypertrophic cardiomyopathy with transient hypotonia and mild lactic acidosis. Spontaneous clinical remission occurred. In all cases muscle biopsy was performed. Morphological studies failed to show ragged-red fibers but there was lipid storage myopathy and decreased cytochrome c oxidase activity. Biochemical studies confirmed the cytochrome c oxidase deficiency in muscle in all cases. It was associated with complex I III deficiency in case 1 and with severe deficits of all respiratory chain complexes in case 2. Post-mortem studies in case 1 indicated that complex IV was reduced in the liver but not in the heart and quantitative analysis of mtDNA revealed a depletion in muscle. Cases 1 and 2 shared some clinical features with fatal infantile myopathy associated with cytochrome c oxidase deficiency, while case 3 displayed a very unusual clinical presentation. The histochemical enzyme reaction of cytochrome c oxidase is useful for the diagnosis of mitochondrial myopathy because ragged-red fibers may be lacking. Finally, biochemical measurement of the different mitochondrial respiratory chain complexes is required because multiple defects are frequent and occasionally related to mtDNA depletion.

Four siblings with hepatic fibrosis are described. The liver damage in these patients was secondary to chronic ingestion of massive doses of vitamin A for congenital ichthyosis. Although the extrahepatic manifestations were helpful in the diagnosis of hypervitaminosis A, the distinctive features of hepatic histopathology were confirmatory. The plasma concentrations of vitamin A and retinol-binding protein were misleading. The recovery from the liver damage in these patients was slow despite a complete withdrawal of the vitamin A intake. These cases show the importance of hepatic vitamin A assessment in the diagnosis of hepatic fibrosis.

We describe two cases of giant-cell fibroblastoma (GCF) with dermato fibro sarcoma protuberans (DFSP) component, occurring in two children in a chest wall localization. One case recurred 1 year later. The two patients were tumor-free 12 and 8 years later. GCF is a rare mesenchymal cutaneous and subcutaneous tumor reported mostly in the first two decades of life. Dermato fibro sarcoma protuberans, occurring preferentially in adults, is a rare skin tumor with a pronounced tendency to local recurrence. Some cases of association of recurrence of GFC under the form of DFSP have been reported, raising the question of a continuum between the two tumors. The treatment of choice of the two tumors is a wide local excision.

Marantic or non-bacterial thrombotic endocarditis is a common complication of terminal malignancy, usually discovered at autopsy. The authors report a case of marantic endocarditis as a presenting sign of pancreatic carcinoma in which the diagnosis was made ante-mortem by transoesophageal echocardiography.

• Background: The fetal face is clearly seen by ultrasonography: we considered measurement of certain orbitofacial parameters of interest in the human fetus in order to establish norms for facial development. • Methods: We included 108 “normal” fetuses ranging in age from 16.5 to 41 weeks of amenorrhea. The orbitofacial parameters studied were outer canthal distance, inner canthal distance, oropalpebral distance right side and left side, and palpebral fissure length rside and left side. The ocular parameters studied were corneal horizontal diameter and axial length. The traditional anthropometric parameters of the fetus were determined by pathological examination: age, weight, head circumference and height. A statistical study analyzed the different correlations and established linear regression equations for orbitofacial parameters as a function of age. Polynomial regression models were tested to the third degree as a function of age and the head circumference/17 ratio. • Results: Results are given in six different age groups. We find excellent correlation between the different parameters. Statistically valid linear regression equations were established for orbitofacial parameters. Polynomial regression equations were compared to linear equations their correlation coefficient and standard error, but showed no greater validity. Skull growth is more rapid than facial growth, which itself is more rapid vertically than horizontally. • Conclusion: This study establishes norms for the different orbitofacial parameters, in particular the oropalpebral distance, for which we found no bibliographic references. The general interest of these measures lies in the description of malformation syndromes.

Calcifications in lymphoma are rare findings prior to therapy. We report our experience with a 13-year-old boy who presented with a primary calcified duodenal Burkitt's lymphoma. US and CT findings are described. Two differential diagnoses are discussed.

A prospective registration of incident cancers in childhood in two south-east regions of France since 1 January 1984 allows us to collect pertinent data on 875 cases throughout a period of 8 years. World age-standardised overall incidence rate is 137.63 cases/million/year. It is close to that reported in other white European. North American and Oceanian populations. The age-adjusted (age-standardised) relative frequency of each pathological group is: leukaemias 29.71%; central nervous system tumours 20.61%; lymphomas 12.75%; sympathetic tumours 9.03%; soft tissues tumours 7.37%; bone tumours 5.89%; kidney tumours 4.82%; epithelial tumours 3.83%; germinal and gonadal tumours 3.24%; retinoblastomas 2.11%; liver tumours 0.45% and others 0.14%. The comparison of these results with international available data shows that we record the world highest adjusted incidence rates for neuroblastomas (15.46) and rhabdomyosarcomas (7.04) and a high rate for Ewing's sarcomas (3.30); this fact will need to be confirmed by a longer period of observation, but even now the total number of cases (particularly for neuroblastoma) is high when compared with the data of other children registries which give rates for longer periods and for similar or larger populations.

The authors have analysed a series of aneurysmal bone cysts (A.B.C.) in children and adolescents which were reviewed at an average follow-up of 4 years 2 months (6 months-15 years). The average age at diagnosis was 8 years (3-19 years). Pathological fractures were the main reason for consultation, probably due to the predominance of central lesions. If conventional radiology remains indispensable to diagnosis, magnetic resonance imaging is nevertheless the most important examination in determining the extent of the affection. The diagnosis remains anatomopathological, even if this may be difficult due to associated lesions (A.B.C. illness and A.B.C. symptoms). In long bones lesions, we observed recurrence after curettage in 3 out of 7 cases. For this reason the recommend simple resection or reconstructive resection rather than curettage whenever possible. 5 lesions in contact with growth plate were observed. In such cases we recommend its conservation by careful curettage, especially in young children. We feel that subsequent recurrence is easier to treat than an epiphysiodesis bridge. The surgical techniques employed to conserve the plate are described along with methods of bone reconstruction after surgery.

A case of acute eosinophilic myocarditis without hypereosinophilia, presenting as hypokinetic dilated cardiomyopathy in a 24-year old man is reported. Sudden worsening of subacute cardiac failure required heart transplantation 3 months after the onset of the disease. Only pathological examination provided the diagnosis of acute necrotizing eosinophilic myocarditis of undetermined origin. Two years after transplantation, the patient had no clinical or histological sign of recurrence. Seldom described in the literature, acute eosinophilic myocarditis is a dangerous form of eosinophilic heart disease which often follows a fulminant course beyond all therapeutic resources. This case, which is particular in its clinical presentation, in the lack of hypereosinophilia and above all in its cure after heart transplantation, enables the authors to discuss the mechanisms and various manifestations of the cardiotoxicity of eosinophils.

The authors report one case of Drash syndrome: association of Wilms' tumor, nephropathy and genital abnormalities. The definition, prognosis and origin of this triad are discussed.

Three pediatric cases of malignant rhabdoid tumor of the neck are described. Clinical data and imaging findings (US, CT and MRI) are stressed. The mass was well defined, containing punctate calcifications in two cases and encasing the vessels in two other cases. Two patients were treated with a chemotherapy regimen according to MMT 89 SIOP protocol, one had chemotherapy and radiotherapy. Two children died of progressive disease; the remaining child who had complete surgical removal of the tumor is on remission 17 months after diagnosis.

Chromosome analysis was performed in two cases of rhabdomyosarcoma (RMS): one embryonal RMS and one alveolar RMS. Analysis showed number and structural abnormalities of chromosome 2. The relationship between these findings and published reports of karyotypes from RMS is discussed.

A patient with spreading pseudomyxoma peritonei probably caused by a tumor of the appendix showing suggestive clinical features is presented. The long evolution was marked by the discovery of gelatinous substance in the stools and in the lumen of the gut, and the appearance of distant metastasis in the lungs, adrenal glands and lymph nodes. Histochemical studies showed that periodic Shiff acid and Alcian blue were fixed by the mucoid substance. The use of toluidin blue (pH 1) revealed metachromosia. Electron microscopy ascertained the presence of enterocytes and mucous cells and revealed a more obvious nuclear disorganisation than by histological microscopy. Biochemical study showed that the mucoid substance extracted from the pseudomyxoma was rich in carbohydrates and could be identified as a mucin on the basis of the composition of its monosaccarides and of the alkali-lability of its glycanprotein linkages. The mucin was weakly hydrolysed by trypsin in vitro but was deeply attacked by the same enzyme after desialylation. These results: a) confirm the possibility of observing spreading distant metastasis in the pseudomyxoma peritonei; b) underline the potential interest of electron microscopic study in case of wavering diagnosis; c) show that the mucoid substance belongs to the group of neutral sulfomucins; d) legitimate, in the future, a trial of palliative treatment by neuraminidase in such cases.

An apparently anaplastic retroperitoneal tumour was studied by a cell culture method, The patient was a young man. The method used revealed the morphological features and secretory potential of the lesion, which could thus be diagnosed as an immature neuroblastoma.

Epidemiology is a promising clinical way for research on cancer. A population based pediatric cancer registry was opened since January 1984 in 2 regions of south-eastern France. Its purpose is to analyse childhood cancer incidence and possible variations. A case-control study on risk factors was set up at the same time. Registration procedures and 1984 data are reported. The observed incidence is 11.86 new cancer cases/10(5) children/year.

Hb F values were estimated in 336 adults on 525 occasions. A variable elevation appeared not only in patients with a variety of haematological diseases, but also in conjunction with malignant neoplasms, liver disease etc. The highest values were encountered in cases of pernicious anaemia, haemolytic anaemia, panmyelopathy and plasmocytoma. The determination of Hb F in peripheral blood smears by means of this extremely simple elution method (Betke and Kleihauer) represents a useful tool in the diagnosis of haematological disorders in adults, especially the haemoglobinopathies. The possible causes of this biochemical redifferentiation of erythropoiesis are discussed.

In a prospective study of 23 patients who had undergone orthotopic heart transplantation we tried to assess the value of doppler-echocardiography in the detection of acute graft rejection. For this purpose, 220 echocardiographic records were compared with the results of endomyocardial biopsies performed at an interval of less than 12 hours. The parameters investigated by TM and two-dimensional echocardiography were: morphological parameters (including septal echodensity), left ventricular mass and systolic function parameters. Diastolic parameters (isovolumetric relaxation time [IVR], transmitral gradient half-decrease time [T 1/2] and proto-end-diastolic mitral velocity ratio [E/A] were measured by TM echocardiography and pulsed doppler velocimetry. The best doppler-echocardiographic criteria for graft rejection were a more than 15 ms reduction of IVR, a more than 15 p. 100 increase of myocardial mass, and a more than 30 p. 100 increase of teh E/A ratio, the corresponding sensitivities for histological rejection being 82, 76 and 74 p. 100 respectively. In contrast, T 1/2 and systolic function studies seemed to be disappointing. Finally, the increase of septal echodensity enabled rejection to be diagnosed with an excellent (92 p. 100) specificity but an insufficient sensitivity. Thus, none of the parameters measured were sensitive enough, taken separetely, to replace endomyocardial biopsy. However, the combined use of the most sensitive of them should make it possible to reduce the frequency of systematic biopsies.