Cell differentiation in Wilms' tumor (nephroblastoma): an immunohistochemical study.

Using an indirect immunoperoxidase technique, we tested frozen specimens from 12 Wilms' tumors with monoclonal antibodies (MoAbs) reacting against a large panel of molecules including laminin, fibronectin, cytokeratin, vimentin, villin, CD24, CALLA/CD10, CR1, CD26, class I and class II major histocompatibility complex (MHC) molecules, and endothelium factor VIII. These molecules were chosen because they are markers of specific segments of the mature kidney and because their loss or acquisition is indicative of different steps of human nephrogenesis. KI67 MoAb was used to evaluate the proliferating activity of the cells. The blastemal component (cell compact areas) of Wilms' tumors consisted of vimentin-positive cells with a fibronectin network. However, signs of epithelial maturation were present in compact areas where cytokeratin-positive cells producing laminin were observed. The cells exhibited a high degree of proliferating activity. The tubule formations consisted of cytokeratin-positive cells and had a defined laminin border. All the cells, whether in compact areas or in tubules, were strongly CD24-positive. Some tubular formations showed signs of proximal maturation with the presence of CALLA, CD26, and even villin. In four cases class I-MHC molecules were expressed by some tubular cells. Large cystic cavities present in five cases were edged by cytokeratin, CD24-positive cells, or by vimentin, CALLA, CR1-positive cells. Some glomeruloid bodies, present in two cases, were also composed of vimentin, CALLA, and CR1-positive cells which correspond to the mature podocyte phenotype. The interstitial tissue contained mainly laminin and fibronectin network with macrophages and few CD3 lymphocytes. The presence of large cells with muscular differentiation was noted; round vimentin and CD26-positive cells were also seen. The endothelial cells of the vessels exhibited vimentin, factor VIII, and class I and class II MHC molecules as do mature cells, but in some cases the endothelial cells lacked class II molecule expression and were CALLA-positive. These results which confirmed and extended those previously described show that cell differentiation in Wilms' tumor mimics that observed during metanephros development. Moreover, this study shows that tumoral cells in nephroblastoma share several antigens with cells from lymphoid lineage (CD24, CALLA, and CD26) as do developing and mature kidney cells. Such cell phenotype dissection provides a useful and reliable tool for testing the influence of various factors on the development of hetero-transplanted or cultured Wilms' tumors.

[Parotid carcinoma in children. Apropos of 3 cases]. / Carcinome de la parotide chez l'enfant. A propos de 3 cas.

Three pediatric cases of parotid carcinoma are reported two are acinic cell carcinoma of relatively good prognosis. The third case is a cystic variety of salivary duct carcinoma, more exceptional, which prognosis remains uncertain. A review of the parotid tumors of this age range is done.

[Cystic teratoma of the diaphragm. Apropos of a case, review of the literature]. / Tératome kystique du diaphragme. A propos d'un cas, revue de la littérature.

Benign tumors of the diaphragm are rare lesions. The cystic teratoma of this muscle is an internal axial tumor, a benign dysembryoma, and only 4 cases could be found in the published literature. The cystic teratoma reported was discovered fortuitously during investigation of hypertension. It was located in the diaphragm above the psoas arch.

[Appendical innervation in the long types of Hirschsprung's disease. Histological and histoenzymatic study]. / L'innervation appendiculaire dans les formes "longues" de maladie de Hirschsprung. Etude histologique et histoenzymatique

On the basis of the fact that the autonomic innervation of the appendix reflects that of the caecum, that appendicectomy is easier and less dangerous than multiple biopsies of the wall of the colon and that frozen section and later study, with the aid of a rapid histoenzymatic technique, is more convenient than that of small fragments which are always difficult to orientate, the authors recommend this investigation in cases in which a syndrome of obstruction in the neonatal or postnatal period poses the problem of the diagnosis and emergency treatment for total absence of colonic ganglia.

Pulmonary infiltrate with eosinophilia in a 14-year-old child.

A prolonged case of pulmonary infiltrates with eosinophilia of the lung was submitted to a large immunological enquiry. The biological results, added to the immunofluorescence study of a lung biopsy, militated for a complex underlying immune mechanism with, on one hand IgE, eosinophils and mast cells, and on the other hand plasma cells and IgG.

Severe combined immunodeficiency disease: a pathological analysis of 26 cases.

Autopsy material and clinical information were analyzed in 25 cases of untreated or unsuccessfully treated severe combined immunodeficiency disease and one case successfully treated by bone marrow grafting. Two cases were adenosine deaminase deficient and one was nucleoside phosphorylase deficient. The histological appearance of the thymus fell into four clearly recognizable patterns: simple dysplasia, dysplasia with corticomedullary differentiation, dysplasia with pseudoglandular appearance, and atrophic pattern. Three cases lacked lymph nodes and belonged to the category of thymic dysplasia with pseudoglandular appearance. From the data, the following conclusions can be made: (i) The thymic atrophic pattern is a phase in a dynamic process of which the end result is simple dysplasia or dysplasia with corticomedullary differentiation. (ii) The pseudoglandular pattern represents a disease process of early intrauterine onset. (iii) At least a proportion of the cases represent a T-cell defect rather than a lymphoid stem-cell defect. (iv) The lymphoid germinal centers are not the source of plasma cells. (v) The graft-versus-host reaction probably causes lymphoid cells depletion in lymph nodes and spleen.

Ig surface receptors and erythrophagocytic activity of histiocytosis X cells in vitro.

Membrane receptors for Ig and IgG and receptors for immune complexes (C3) have been identified on the surface of histiocytosis X cells by direct immunofluorescence and by rosette formation. Histiocytosis X cells also avidly phagocytose human RBC and latex particles. The cells studied originated from three eosinophilic granulomas of bone and were maintained in culture for 12 days. These immunological characteristics suggest that histiocytosis X cells represent a neoplastic variant of a cellular subpopulation (Langerhans lineage) which probably belongs to the mononuclear phagocyte system.

Deduction from Wilms' tumour that glomerular podocytes produce the basement membrane material bearing Goodpasture determinants.

Using an indirect immunoperoxidase technique, we tested frozen specimens from one Wilms' tumour composed of numerous glomeruloid bodies devoid of blood vessels, with monoclonal antibodies directed against vimentin, cytokeratin, CALLA/CD10, CD24, CR1/CD35, endothelium factor VIII, class I and II MHC molecules, laminin, fibronectin, and non-collagenic domain NC1 of type IV collagen. Two reagents against Goodpasture determinants were used: P1 monoclonal antibody and serum IgG (GP antibodies) from a biopsy-proven Goodpasture patient. Glomeruloid bodies comprised two cell types: a peripheral layer of parietal epithelial cells (cytokeratin and CD24-positive) and central cell clumps of podocytes (vimentin and CALLA-positive). The basal lamina surrounding the glomeruloid bodies contained laminin and NC1 domain of type IV collagen, while that present between the podocytes reacted strongly with laminin, and P1 and GP antibodies. Endothelium factor VIII was not detected within the glomeruloid bodies and CR1 molecules bound to the basement membrane material within them. These data favour the hypothesis that podocytes produce the basement membrane material which bears Goodpasture determinants recently identified as a novel chain, named the alpha 3 chain, of type IV collagen.

[Diffuse interstitial pulmonary amyloidosis (author's transl)]. / Amylose pulmonaire diffuse interstitielle.

A 56-year-old woman developed diffuse interstitial pulmonary amyloidosis and died two years after the onset of dyspneic symptoms. Lung biopsy confirmed the amyloid nature of the reticulonodular lesions, which were at the origin of the mixed restrictive and obstructive ventilatory deficiency. Investigations showed an obvious hypogamma globulinemia but the etiology of the affection remained unknown. Pathological examination revealed massive infiltration of amyloid material in the lungs, no involvement of the heart, what was unexpected, and minimal deposits in the mediastinal lymph nodes kidneys, and spleen. An immunofluorescence study of the pulmonary amyloid deposits was performed. The authors emphasize the rarity of such diffuse interstitial pulmonary amyloid lesions which differ greatly from nodular amyloidosis and upper respiratory tract amyloid infections.

Aniridia, male pseudohermaphroditism, gonadoblastoma, mental retardation, and del 11p13.

A 20-month-old male patient was referred because of severe growth and mental retardation, bilateral glaucoma, hypospadias, and cryptorchidism. Karyotyping revealed a de novo complex three-chromosome rearrangement as well as deletion of band 11p13:46,XY,t(4;7;15)(q212;p14;q26)del(11)(p13p14). Trabeculectomia revealed bilateral aniridia. Surgery on the genitalia revealed male pseudohermaphroditism and bilateral gonadoblastoma. The kidneys were normal. A deficiency in catalase (CAT) activity allowed the regional assignment of the CAT gene to band 11p13.

[Evaluation of 17-beta-hydroxysteroid dehydrogenase activity as a marker of the hormone dependence of breast cancers]. / Evaluation de l'activité enzymatique 17 beta-hydroxystéroïde deshydrogénase comme marqueur de l'hormonodépendance des cancers du sein.

Intratumoral activity of the enzyme 17 beta-hydroxysteroid dehydrogenase (17 beta-HSD) was measured in 55 patients with breast cancer (17 pre- and 38 post-menopausal) before and/or after 8 days of a progestin treatment (lynestrenol 10 mg/day). In 12 patients the 17 beta-HSD ability to be stimulated was compared to estradiol and progesterone receptor (ER and PR) levels. In premenopausal patients 17 beta-HSD was higher when tumorectomy was performed in the luteal phase than in the follicular phase. In post-menopausal patients, 17 beta-HSD is higher after progestin treatment. However 17 beta-HSD stimulation by lynestrenol depends on receptor levels. It is most after markedly stimulated in ER+ PR+ tumors. It remains low in ER- PR- tumors. In conclusion, intratumoral measurement of the progesterone dependent enzyme (17 beta-HSD) in breast cancer after progestin treatment provides a fine and reliable index of the presence and functional character of PR and hormone dependence of the tumor.