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Biomedical subjects

E Justrabo

Publications and source records attributed to E Justrabo.

At least 37 records · Page 2Linked to original sources

Metanephric adenoma of the kidney. A clinicopathologic, immunohistochemical and electron microscopic study of two cases.

We report two cases of metanephric adenoma in 40 and 48 year-old women. These rare kidney tumors were composed of cuboidal epithelial cells forming tubules, glomeruloid structures and sheets. Ultrastructural and immunohistochemical studies revealed that the tumor cells are similar to epithelial cells of developing nephrons. These features differentiate the metanephric adenoma from tubulo-papillary renal carcinoma, nephroblastoma, and cortical adenoma. According to its invariably benign course, the metanephric adenoma treatment could be restricted to a simple tumorectomy.

Adenoma↗

[Isolated leiomyomatosis of the right renal vein associated with hepatic myelolipoma: diagnostic and therapeutic problems].

The authors report a case of isolated intravenous leiomyomatosis involving a vein of the right renal hilum associated with operated hepatic myelolipoma with a diagnosis of metastatic renal cancer. These tumours are very rare and are usually diagnosed on histological examination of the resection specimen. Although they have a benign histology, they have a very different prognosis and therapeutic management. Although the association of these two exceptional tumours may appear to be purely incidental, a common aetiological factor may nevertheless exist.

Angiography↗

[Bilateral scrotal panniculitis in a prepubescent child].

Scrotal panniculitis or scrotal fat necrosis is an uncommon acute scrotal pathology. We report a case of scrotal fat necrosis in a 9 1/2 year-old over-weight boy with bilateral tender scrotal masses, located beneath the tests. Pathologic findings were those of subcutaneous fatty indurated masses with lipogranulomatous foci. The etiology of scrotal fat necrosis is unknown. A similar condition has been described in young children exposed to cold. In obese prepubescent boys, a greater sensitivity to cold and a higher saturated fatty acid concentration of the scrotal adipose tissue would induce fat necrosis. Our study of fatty acid composition by gas-liquid chromatography showed an elevation of stearic acid. The spontaneous resolution of scrotal fat necrosis is always the rule and allows symptomatic treatment without surgical investigation.

Adipose Tissue↗

[Hepatic mesenchymal hamartoma in children. Immunohistochemical, ultrastructural and flow cytometric case study].

Mesenchymal hamartoma is a rare liver lesion. This lesion was found in a 7-month-old girl with high serum alphafaetoprotein serum levels and was composed of loose connective tissue containing a certain number of epithelial cells of biliary or hepatic origin. Immunohistochemical studies showed that cytokeratins 7 and 19 were localized in bile duct epithelium. The ultrastructural study showed that the hamartoma was composed of well differentiated ductal structures surrounded by a myxoid mesenchyma with cysts formed either from degenerative mesenchymal areas or from dilated ducts. Flow cytometric analysis of nuclei from frozen tissue revealed that the lesion was DNA aneuploid, with a DNA index of 1.28.

Aneuploidy↗

Renal amyloidosis with a frame shift mutation in fibrinogen aalpha-chain gene producing a novel amyloid protein.

A French kindred with autosomal dominant hereditary renal amyloidosis was found to have a novel mutation in the fibrinogen Aalpha-chain gene. In this kindred, renal disease appeared early in life and led to terminal renal failure at an early age. Renal transplantation resulted in rapid destruction of the allograft by amyloid deposition within 2 years. Amyloid fibril protein isolated from a transplanted kidney was found to contain a novel, hybrid peptide of 49 residues whose N-terminal 23 amino acids were identical to residues 499 to 521 of normal fibrinogen Aalpha-chain. The remainder of the peptide (26 residues) represented a completely new sequence for mammalian proteins. DNA sequencing documented that the new sequence was the result of a single nucleotide deletion at position 4897 of the fibrinogen Aalpha-chain gene that gives a frame-shift at codon 522 and premature termination at codon 548. The contributions toward fibrillogenesis of the two portions of the amyloid fibril protein, ie, N-terminal fibrinogen sequence and C-terminal novel sequence, are presently unknown. However, the early onset and rapid reoccurrence of amyloid in renal transplants is unlike the clinical course with other amyloid proteins having single amino acid substitutions that give hereditary renal amyloidosis. Liver transplantation to stop synthesis of this abnormal hepatic derived protein should be considered early in the course of the disease.

Adult↗

[Prognostic factors of survival in medullary cancers of the thyroid. Apropos of 51 cases. Clinical, biochemical and immunocytochemical study].

The aim of this study was to assess the main clinical biochemical and immunocytochemical factors influencing survival in 51 patients operated for medullary thyroid carcinoma (MTC). There were 55% of women and 37% of familial cases. Mean age was 43 +/- 3 years. The following survival variables were tested: sex, age, stage, familial disease, Serum Carcino Embryonic Antigen (CEA) and Calcitonin (CT) levels three months postoperatively, intensity of CEA and CT immunostaining, percentage of cells stained for CEA and CT. The actuarial survival rate, including postoperative mortality (N = 1), was studied by uni and multivariate analysis using a Cox model (N = 31). The 5-year survival was 69 +/- 7%. By univariate analysis, stages I or II (p < 0.0001), age of 45 years and less (p < 0.0001), normalized CEA levels (p < 0.006), percentage of CT stained cells greater than 80% (p < 0.04) and weak CT and CEA staining (p < 0.02) were significant predictors of increased survival rates. Age less than 45 and stages I or II were significant prognostic factors of goof survival on multivariate analysis (p < 0.001). We conclude that clinical criteria constitute good survival prognostic factors in patients operated for MTC. The better prognosis of familial cases was probably related to their earlier detection. The prognostic value of immunostaining remains controversial and requires further studies.

Actuarial Analysis↗

[Microsurgical sutures with non-transfixing staples. An experimental study of 15 rat aortas].

This study, conducted in rats, studied a new system of anastomosis, by nontransfixing clips, pinching each edge of the artery with minimal trauma. Histological examinations were performed at one week and one month in order to investigate the vascular wall in the line of anastomosis. Clinical application of this procedure was undertaken in view of the encouraging and satisfactory results obtained.

Animals↗

[Ovarian steroid cell tumors: a bilateral form].

The authors report a case of bilateral ovarian steroid cell tumour, not otherwise specified (as subclassified now by Scully). For two years a 46-year-old woman had androgenic manifestations. Only plasma testosterone level was increased. A hysterectomy with bilateral ovariectomy was performed. The first diagnosis was bilateral Leydig cell tumour, but no Reinke crystal was found. The final diagnosis was steroid cell tumour, not otherwise specified. No significant nuclear atypia or mitotic activity were found and two-year follow-up was favourable. The authors discuss the difficulty to class some ovarian tumours between Leydig cell tumour and steroid cell tumour, not otherwise specified as defined by Scully.

Diagnosis, Differential↗

[Lympho-epithelial lesions induced by plasma cells in a pulmonary MALT lymphoma].

One case of pulmonary lymphoma of MALT origin is reported. The diagnosis was made by histological and immunohistological study of surgical specimen. Cytologically, the tumoral proliferation was made by an admixture of centrocyte-like cells, small lymphocytes and vacuolized plasma cells. Lympho-epithelial lesions were particular because induced by tumoral plasma cells. From this case, problems of diagnosis and physiopathology of pulmonary MALT lymphomas are discussed.

Cell Division↗

Piridoxilate-induced oxalate nephropathy can lead to end-stage renal failure.

A 71-year-old woman was admitted with end-stage renal failure and histological evidence of oxalosis. This case of diffuse renal tubular crystal calcium oxalate deposits seems to be induced by long-term piridoxilate therapy (10 years) or simultaneous intake of both piridoxilate and vitamin C (500 mg/day for 6 months), since no other cause of secondary oxalosis could be found. So, it seems necessary to monitor the serum creatinine level, especially in the elderly, during piridoxilate therapy and to avoid high vitamin C intakes in patients under such treatment to prevent development of renal insufficiency.

Aged↗

Statistical analysis of histomorphological findings in medullary thyroid carcinoma: distinction between the different familial forms of the disease. G.E.T.C. Groupe d'Etude des Tumeurs a Calcitonine.

A multifactorial analysis of morphological findings was performed on 153 cases of medullary thyroid carcinoma (MTC). The aim of the study was to utilize histological criteria to discriminate between MTC associated with multiple endocrine neoplasia type 2A (MEN 2A) and that associated with the inherited MTC only syndrome. The presence of fusiform cells associated with several other markers seemed to be more predictive of MEN 2A. A comparison of inherited MTC only and sporadic MTC only showed fusiform cells to be significantly less common in inherited MTC only. These results suggest that the inherited MTC only syndrome is a distinct clinical and morphological entity. Further investigations are needed to confirm the findings and understand its implications.

Carcinoma↗

[Extracapillary glomerulonephritis].

Extracapillary glomerulonephritis is characterized by cell proliferation within the urinary space of 50% of the glomeruli, where it covers more than 50% of the filtration chamber, associated with acute or rapidly progressive renal failure. It is a model of curable human renal failure. Extracapillary cell proliferation is an elementary lesion which may complicate any glomerulopathy and many systemic diseases, or appear to be primary. Its clinical manifestations may be extremely marked in some systemic diseases, but they may be minimal and delay a diagnosis which rests entirely on renal biopsy. An early renal biopsy commands the prognosis which depends on the finding of young cellular crescents that respond to treatment before fibrous transformation sets in. Experiments in animals and man suggest that cell proliferation results from rupture of the capillary walls and from the production of polymerized fibrin in the urinary space. This is followed by a cascade of reactions, with increased synthesis of local mediators issued from resident and invasive glomerular cells. These data constitute the basis of modern therapies, such as emboli of methylprednisolone, plasma exchange and immunodepressive drugs, aimed not only at a possible aetiological treatment but also at the cell proliferation itself. The use of such treatments, whose risks must be carefully weighted, has transformed the prognosis of extracapillary glomerulonephritis, since almost 50% of the cases the kidneys survive at 5 years.

Glomerulonephritis, Membranoproliferative↗

[Invasive adenocarcinoma with epidermoid carcinoma on the site of bladder exstrophy. Histochemical and immunocytochemical study].

To date, one hundred and ten cases of malignant neoplasms arising from untreated bladder exstrophy have been reported. We describe another case of uncorrected bladder exstrophy with invasive adenocarcinoma and in situ squamous carcinoma discovered in a 51 year old woman. Postoperative radiotherapy was performed after cystectomy with nephroureterectomy and hysterectomy. The neoplastic samples were investigated by mucin histochemistry and immunocytochemistry. The epithelium lining the tumor villi and the surrounding mucosae was colonic with mucin histochemistry and reacted to anti KL1, EMA and ACE antibodies. These features were those of colonic and vesical adenocarcinomas. Although early vesical reconstruction is the best oncologic prevention, patients with bladder exstrophy run the risk of sigmoid adenocarcinoma after cystectomy and urinary diversion including ureterosigmoidostomy.

Adenocarcinoma↗

[Favorable outcome of Wegener's disease limited to the lungs. Apropos of a case].

A case of Wegener's granuloma limited to the lungs is reported in a 22 year old man. The pulmonary radiograph showed bilateral nodules. A lung biopsy of a nodule revealed a necrosing vasculitis involving the arteries and the veins and some areas of necrosis in geographical contours around these vessels. The renal function was normal and 4 1/2 years later the outcome remains favourable with the minimum of treatment, the exception being a course of antibiotics post operatively. Fifty seven cases of Wegener's granuloma limited to the lungs have been diagnosed after lung biopsy, transbronchial or bronchial material. The outcome was favourable in 72% of cases. This result was observed in the absence of any treatment in 6 cases (13%). In 2 cases the pulmonary nodules disappeared or developed cavities. In 4 cases the anatomical and/or radiological progress is unknown. Currently the factors leading to such an outcome have not yet been identified.

Adult↗

Immunologic characterization of Ewing's sarcoma using mesenchymal and neural markers.

The two most recent hypotheses about the histogenesis of Ewing's Sarcoma (ES) are that it has a mesenchymal or neuroectodermal origin. Immunologic markers specific to these two tissue origins were tested on cryostat sections from three primary tumors carrying the chromosomal translocation t(11;22)(q24;q12). Cell lines established in vitro from two of these three primary tumors were also analyzed. Using antibodies directed against neural components (neurone-specific-enolase [NSE], HNK-1, and neurofilament triplet proteins [NFTP]), positive reactions were observed in cells from two primary tumors and their corresponding cell lines. Results of electron microscopic examination of the primary tumors were compatible with the diagnosis of ES. When using antibodies directed against mesenchymal cell surface antigens (common leucocytes, Leu M1, Leu M2, and Leu M3), the weak positive reactions observed in the three primary tumors were attributed to lymphoid infiltrates within tumor cells. Six additional ES cell lines carrying the translocation t(11;22) were also analyzed by immunocytochemical and flow cytometry methods using antibodies directed against mesenchymal and neural components. Positive reactions were observed in all seven cell lines tested using antibodies directed against NSE, HNK-1, and 200 KD subunit of the NFTP, whereas negative reactions were obtained with Leu M2 antibody. These results are consistent with a neuroectodermal origin of ES cells.

Antibodies, Monoclonal↗

[Hemangiopericytoma of the thyroid gland].

Hemangiopericytoma is an uncommon tumour of the thyroid gland. We report the fourth case of thyroid hemangiopericytoma in a 77 year-old woman who presented a goiter known for 23 years. The tumour, 11.5 X 7 X 7 cm in diameter, was located in the left lobe. Its histological features were those of a benign hemangiopericytoma as in two cases previously reported. Light microscopy discovered capillaries surrounded by sheets of plump cells and a reticulin network encircling each cell. A few tumour cells showed slight immunoreactivity with antibody against actin. There was no immunoreactivity with antibodies against desmin, vimentin and myosin intermediate filaments. Today, the electron microscopic analysis which revealed that tumour cells looked like pericytes, remains the best investigation for hemangiopericytoma diagnosis.

Aged↗