[Changes in the incidence and mortality rates of malignant melanoma in the Seine-Maritime region over a 20-year period]. / Évolution de l'incidence et de la mortalité du mélanome en Seine-Maritime sur une période de 20ans.

BACKGROUND: The aim of this study was to assess changes in the incidence and mortality rates of malignant melanoma (MM) over a 20-year period in a region without a cancer registry. METHODS: All cases of MM studied were collected retrospectively from the databases of six private and three hospital-based histopathology laboratories in the Seine-Maritime region covering three 24-month periods each 10years apart: 1988-1989, 1998-1999 and 2008-2009, The incidence and mortality rates were estimated based on data provided by French National Institute for Statistics and Economic Studies (Insee) and French National Institute of Health and Medical Research (Inserm). RESULTS: Over the 20-year period, the incidence of MM increased from 8.6 to 21.2/100,000inhabitants per year (+147%, P<0.0001) while the mortality rate rose from 1.3 to 2.8/100,000inhabitants per year (+115%, P=0.0003). The incidence of invasive MM increased by +110%, while the incidence of MM in situ increased by +456%. The incidence and overall mortality rate of invasive MM increased particularly during the first 10-year period: +62% (P<0.0001) and +77% (P=0.01) respectively, and to a much lesser extent during the last 10-year period: +30% (P=0.0007) and +22% (P=0.22) respectively. This slowdown in the incidence of invasive MM and in overall mortality rates was even more pronounced in women over the last 10years (+17 and +9%), whereas these rates continued to increase in men (+49% and +35%, respectively). In contrast, the incidence of MM in situ increased above all during this same period (+257%). CONCLUSION: This study shows that while the incidence and mortality rate of invasive MM has increased little over the last 10years in the Seine-Maritime region, the incidence of MM in situ continues to rise sharply.

Sarcoidosis. Retroperitoneal fibrosis, renal arterial involvement, and unilateral focal glomerulosclerosis.

In one patient with pulmonary sarcoidosis, hypertension occurred during the course of the disease. Aortography showed extensive narrowing of the right renal artery. Surgical exploration disclosed extensive periaortic and perirenal fibrosis. This fibrosis encircled the right renal artery and caused extrinsic compression. Pathological examination disclosed a large amount of histiocyte epithelioid infiltration in various samples of the fibrosis and particularly in the adventitia of the renal artery, highly suggestive of sarcoidal angiitis. Surgical biopsy was performed on both kidneys. The right kidney, protected by arterial stenosis, was little altered, while the left kidney showed extensive interstitial, tubular, and glomerular lesions. The glomerular lesions were focal and segmental hyalinosis.

Coupling defect of thyrotropin receptor and adenylate cyclase in a pseudohypoparathyroid patient.

A patient with type I pseudohypoparathyroidism was found to have mild hypothyroidism. The patient had an elevated basal TSH level and an exaggerated TSH response to TRH. There was no goiter despite increased TSH levels, and the 131I thyroidal uptake was low before and after exogenous TSH administration. These studies suggested that the patient might have partial resistance to TSH. The binding of radioiodinated TSH to thyroid membranes obtained by biopsy was next studied. The displacement of iodinated TSH by unlabeled TSH was found to be identical to that in normal control membranes. The adenylate cyclase stimulation by a supramaximal dose of TSH, however, was blunted (120.1 +/- 11.5 vs. 387.2 +/- 40.3 pmol cAMP/min/mg protein), while basal and NaF-stimulated activities were quite similar to the activities in normal membranes. These findings suggested a lack of signal transmission between the TSH receptor and the catalytic unit. Incubation of control membranes with TSH and GTP resulted in a synergistic effect on the adenylate cyclase activity. This was not found with the patient's membranes and suggested that the coupling failure was due to a defective guanine nucleotide regulatory protein. We conclude that in this case of type I pseudohypoparathyroidism, the associated mild primary hypothyroidism was due to a partial TSH refractoriness caused by a coupling defect between the TSH receptor and adenylate cyclase. This observation suggests that a common pathogenetic mechanism might underly type I pseudohypoparathyroidism and its associated hypothyroidism.

Simultaneous occurrence of a T-cell lymphoma and a chronic myelogenous leukemia with an unusual karyotype.

The simultaneous occurrence of malignant T-cell lymphoma and chronic myelogenous leukemia is reported. The lymph nodes contained E rosette forming cells. Blood and bone marrow cell morphology were consistent with the diagnosis of chronic myelogenous leukemia. Lymph nodes, bone marrow and blood mitosis showed a t(6;8) (6pter----6q27 ::8p12----8pter;6qter----6q27 ::8p12----8qter) translocation. So far a number of recent reports have shown simultaneous B lymphoid and myeloid proliferations in some malignancies, this is apparently the first reported case of simultaneous T lymphoid and myeloid proliferations.

The hepatotoxicity of 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea (CCNU) in rats. Ultrastructural evidence of a delayed microtubular toxicity.

A few cases of liver involvement have been reported in patients receiving treatment with the antineoplastic nitrosourea CCNU. A single oral dose of 20 or 50 mg/kg CCNU in female Wistar rats induced an important increase in transaminases between day 2 and day 6, followed by a second, moderate increase between day 21 and day 28. Alkaline phosphatases and conjugated hyperbilirubinemia (threefold-increase) were noted for the two doses and were greater for the highest dose. Histological and ultrastructural studies disclosed hepatic lesions of two types: during the first phase of transaminase increase, inflammation of the portal tracts; during the second phase marked dilation of bile canaliculi and numerous filamentous bundles distributed at random throughout the liver cell cytoplasm like normal microtubules. Thus, CCNU induced pericholangitis and intrahepatic cholestasis with microtubular abnormalities. The long-term evolution of hepatic alterations revealed that in the 3rd month after a single oral dose of 20 mg/kg CCNU, lesions were persistent but stable; no reversibility was observed in the 3rd month after 50 mg/kg CCNU, and evolution towards cholangiolysis and biliary cirrhosis was noted. We suggest that CCNU causes a bimodal hepatotoxicity in rats: an early and prolonged ductal injury and a delayed anti-liver cell microtubule toxicity.

Selective reinnervation of the abductor and adductor muscles of the canine larynx after recurrent nerve paralysis.

Functional rehabilitation of the larynx after unilateral vocal cord paralysis was attempted in the dog by selective reinnervation of the laryngeal muscles. The intralaryngeal branches of the right recurrent nerve were dissected. The adductor branch was anastomosed with the ansa cervicalis; the abductor branch was anastomosed with the trunk of the phrenic nerve either within the larynx or through the recurrent nerve, the adductor branch of which was sectioned. Results could be analyzed in seven dogs: mobility of the vocal cord was checked, and electromyography, stimulation of the nerves, and histologic studies were performed. Functional reinnervation of both the adductor and abductor muscles was obtained in only one case, with good abduction. Adduction was recorded in five cases. False-positive results emphasize the necessity of collecting several types of data before concluding that functional reinnervation has been accomplished. The reliability of the procedure can and must be improved.

[Anorectal stenosis caused by suppositories of dextropropoxyphene and paracetamol (Diantalvic)]. / Sténose anorectale par suppositoires de dextropropoxyphène et paracétamol (Diantalvic).

Occasionally, administration of medical substances per annum may result in serious local lesions. The authors report the case of a 58 year-old woman presenting ulcerative and stenosing lesions of the anal canal which led to abdominoperineal resection. History revealed the administration of two suppositories daily, each containing an association of dextropropoxyphene and paracetamol, during seven years because of a cervical pain. Fourteen similar cases have been found in the literature, underlining the difficulties in clinical and pathological diagnosis. The exact mechanism of these lesions remains unknown.

[Liver lipogranulomas with log-term fever caused by paraffin oil]. / Lipogranulomes hépatiques à l'huile de paraffine responsables d'une fièvre au long cours.

In hepatic granuloma, identification of lipogranulomas generally leads to etiological diagnosis. The case reported here was granulomatosis due to mineral oil ingestion, revealed by prolonged fever. In the literature lipogranulomas are generally asymptomatic and diagnosed at autopsy.

[Hereditary visceral myopathy: an entity in idiopathic intestinal pseudo-obstruction]. / Myopathie viscérale héréditaire: une entité au sein des pseudo-obstructions intestinales idiopathiques.

A 17 year-old girl with chronic idiopathic intestinal pseudo-obstruction is reported. Abnormalities of smooth intestinal muscle were shown on light and electron microscopic studies of the excised small intestine and led to the diagnosis of visceral myopathy based on the following features: vacuolar degeneration of intestinal smooth muscle cells with replacement by fibrous preferential involvement of the external longitudinal muscle layer normal myenteric plexus. For the first time similar ultrastructural changes were found on histological study of the colon. Manometric studies revealed a diffuse disease involving the esophagus, small bowel, and bladder. Anorectal abnormalities, never described before, were reported. Family involvement was shown by abnormal esophageal and anorectal manometries in the patient's brother and by paternal history of fatal small intestine occlusion without mechanical obstruction. The prognosis of severe forms of visceral myopathy is generally poor because of the inefficiency of drugs. In this case, after a long period of parenteral nutrition with maintenance of a good nutritional status, a terminal ileostomy (with a special procedure to avoid evagination) associated with a second stage total colectomy allowed to stop parenteral nutrition.

[Acute systemic lupus erythematosus after thymectomy for myasthenia. Sequential study of lymphocyte subpopulations]. / Lupus érythémateux aigu disséminé survenu après thymectomie pour myasthénie. Etude séquentielle des sous-populations lymphocytaires.

A 35 year-old woman developed severe systemic lupus erythematosus 9 years after thymectomy for myasthenia gravis. "Seric Thymic Factor" (STF) was low; T helpers subset, T helpers/T suppressors ratio and to a lesser extent T suppressors subset were decreased. Suppressor cell function investigated by Concanavaline A lymphocyte reactivity was low. Under cyclophosphamide, plasmapheresis and steroids all clinical and biological symptoms improved but STF remained low; T helpers, T suppressors subsets and T helpers/T suppressors ratio increased but did not reach the normal range. Statistical and immunological arguments suggest that the association between systemic lupus erythematosus and myasthenia gravis did not occur only by chance. Moreover, thymectomy might have played a role by decreasing the number and function of some subpopulations of lymphocytes.

[Benign sub-periosteal osteoblastoma of the nasal cavity. histological and ultrastructural study of a case]. / Ostéoblastome bénin sous-périosté de la cavité nasale. Etude histologique et ultrastructurale d'une observation.

A case of periosteal benign osteoblastoma arising in the left nasal cavity is presented. The tumor develops on the surface of the middle turbinate without evidence of bone destruction. It has been locally excised and has not recurred in 3 1/2 years. The histological aspect is identical to that of the other cases of genuine osteoblastoma. The ultrastructure (the first of a periosteal osteoblastoma) is described. The osteoblasts resemble normal osteoblasts with a few exceptions: irregular nuclei, abundance of well-developed rough-surfaced endoplasmic reticulum and numerous fine filaments. The osteocytes and osteoclasts basically resemble their normal counterparts. The final diagnosis of benign osteoblastoma rests at the light microscopy level. The differential diagnosis are reviewed. Distinguishing histologic and ultrastructural characteristics between this tumor and other benign osteoblastic tumors are discussed.

[Mandibular condyle tumors (author's transl)]. / Les tumeurs du condyle mandibulaire.

The must frequently observed mandibular condyle tumors are benign in nature, followed by metastases; primary malignant tumors are very rarely encountered, following a review of the clinical and radiographic characteristics of all types of tumors that occur, three cases of condylar metastases are described. Treatment of primary tumors is exclusively surgical, that of secondary lesions being palliative because of the often poor prognosis related to the primary tumor.

[Morphometric study of variations related to human aging in pulp unmyelinated and myelinated axons]. / Etude morphométrique des variations liées au vieillissement humain des populations d'axones amyéliniques et myéliniques pulpaires.

The evolution of myelinated and unmyelinated axon population in dental pulps during human aging was followed by electron microscopy and morphometry. A total of 2684 nerve fibers were measured in 16 subjects aged 10 to 72 years. The total distribution is bimodal with a recovering zone of 9.8% between unmyelinated and myelinated fibers. The mean diameter of A delta fibers was 1.96 mu and that of C fibers was 0.52 micron. The old pulps showed a loss of A delta fibers of small sizes (0.35-2 micron), the presence of numerous unmyelinated C fibers of very small diameters (0.1-0.25 micron) and a decrease of unmyelinated fibers of 0.5 to 1 micron comparative to young pulps. The logarithms of the diameters unmyelinated axons varied linearly with that of the subjects ages (CR = 0.63; p = 8, 8.10(-3]. The older the patients, the more the fiber C diameters were reduced. The decrease in numbers of A delta fibers could result from a local reduction of pulpal volume related to age or a response to a more general aging process of cerebral origin. The decrease in diameter of unmyelinated fibers in aging pulps would be a reduction of metabolic energy.