Translocation t(3;22)(q23;q11) in three patients with diffuse large B cell lymphoma.

In our series of 134 patients with a diagnosis of non-Hodgkin's lymphoma (NHL) and clonal chromosomal abnormalities, three were found to show an identical t(3;22)(q28;q11) translocation. All were old patients with isolated lymphadenomegaly and diffuse large noncleaved cell lymphoma. All expressed a B cell immunophenotype, and all entered a complete remission when treated with aggressive chemotherapy. This translocation could, therefore, delineate a particular subtype of diffuse large cell NHL.

[Ablation of ventricular extra-systole in the left Valsalva sinus. A case report]. / Ablation d'une extrasystolie ventriculaire dans le sinus de Valsalva gauche. A propos d'un cas.

We report the case of a patient presenting with unrelenting isolated or repetitive monomorphic ventricular extra-systoles, with left block and right axis deviation, which appeared to arise from the right ventricular chamber, but for which ablation was finally performed in the left Valsalva sinus. The ECG and endocavity electro-physiological features which led us to suspect this atypical, although not exceptional, situation are reported, as well as the techniques for ablation available in this case.

Thymolipoma in association with myasthenia gravis.

A 52-year-old male presented with an anterior mediastinal tumor associated with a 2-year history of myasthenia gravis. The patient underwent thymectomy and a 185-g, 10 X 8 X 3.5 cm, well-delineated tumor was resected. On histologic examination the tumor proved to be a thymolipoma composed of mature adipose elements containing cords and nests of thymic tissue. The latter consisted mainly of cortical areas, the thymocytes of which displayed an immunohistochemical profile of cortical cells, i.e., CD 1+, CD 4+, CD 8+, and frequently Ki 67+. Ultrastructural study confirmed the predominant cortical differentiation of the thymic component. No germinal centers, dendritic reticulum cells, or myoid cells were detected by histologic, immunohistochemical, and ultrastructural studies. The association of thymolipoma with myasthenia gravis is rare; this case is the 10th reported. Our findings lead us to believe that (a) the cortical differentiation of the thymic component and the active thymocyte proliferation could represent a factor leading to myasthenia gravis; and (b) thymolipoma could be a peculiar form of thymoma rather than a mixed tumor of mesenchymal and entodermal origin, a lipoma, or a hamartoma of the thymic gland. The reported association of thymolipomas with other immune disturbances or with neoplastic conditions usually associated with true thymomas support these findings.

Translocation t(13;17)(q12-14;p12-13) in two patients with lymphocytic lymphoma.

Cytogenetic studies were performed at the time of diagnosis on two patients with diffuse small cell lymphocytic lymphoma. Both patients had a similar simple karyotype with a t(13;17)(q12-14;p12-13). These observations confirm the nonrandom involvement of band 13q13 in chronic lymphoproliferative diseases.

Prognostic value of the epithelial membrane antigen (EMA) in the case of Hodgkin's disease.

The study deals with the results obtained from 155 lymph node biopsies of patients suffering from Hodgkin's disease (HD) who were treated by MOPP and radiotherapy in the same establishment. The specimens in paraffin have been examined for the presence of antigens using several monoclonal antibodies, particularly epithelial membrane antigen (EMA). There exists no correlation between the immunophenotypes towards EMA and histological types. Thirty-three patients whose responses were EMA (+) have the assurance survival more than 10 years on the level 32.4% while those 122 patients with EMA(-) had the same survival on 90% level (p less than 0.001). Thus a new prognostic tool has been found which enables to detect the likely therapeutic failures in the case of Hodgkin's disease.

[Extra-articular cervical synovial sarcomas. Review of the literature apropos of 2 cases]. / Les synovialosarcomes cervicaux extra-articulaires. Revue de la littérature à propos de deux observations

Two cases of extra-articular synovial sarcoma are reported together with a review of the literature. The analysis of 44 cases shows that this tumor appears among young people particularly between 10 and 30 years of age and is twice more frequent among males. There can be metastasis mostly in lungs (11 times), which can sometimes appear much later (more than 10 years after the first diagnosis). The 5-year survival rate is here, 13/18; this is higher than for similar tumors located in the juxta-articular area. The great size of the tumoral nodule (more than 5 centimeters) is of a bad omen for the prognosis. As regards therapy, isolated surgical exeresis seems to have given results at least as good as these of surgery associated to radiotherapy. In all cases, the diagnosis is based on a light microscopic study which shows two tightly linked histological patterns: a proliferation of fusiform cells and glandular like formations. This feature is not definitely pathognostic and is very similar to tumors of glandular origin, with myoepithelial proliferation. Some examples could indeed be classified as extra-articular cervical synovialo-sarcoma. They could originate in pharyngeal and laryngeal mucous glands. This histogenetic hypothesis could account for the highly favorable evolution of some cases which were reported as synovialo-sarcoma.

[Extraneural metastasis of central nervous system tumours (author's transl)]. / Les métastases extranevraxiques des tumeurs du système nerveux central.

Extraneural metastases of central nervous system tumours are very rare (248 of histologically proven examples in the literature). They are mainly in adult males and in the following descending order of frequency: gliomas (39.5%), meningeal tumours and sarcomas (28.2%), neuronal tumours (14.1%), lymphomas-microgliomas (12.5%), pineal tumours (4%) and melanomas (1.6%); 10 cases out of 248 developed through a shunt. 19 were observed without any surgical operation. There are still many uncertainties about their pathogenesis. It seems however that in some tumours the increased survival time and the repetition of the craniotomy favorises the metastasis.

[Treatment of rhabdomyosarcoma in mice C3H/He by Co 60 and hyperbaric oxygen (author's transl)]. / Traitement du rhabdomyosarcome chez la souris C3H/He par cobalthérapie sous oxygène hyperbare.

UNLABELLED: Experimental study of rhabdomyosarcoma with successive transplantation upon C3H/He mice, treated by irradiation (Co 60) and combined irradiation-hyperbaric oxygen (HBO), dating from 3, 14 and 15 days after transplantation. The data (tumor volume evolution, histological modifications, pulmonary metastases) are compared with controls. CONCLUSIONS: curative radiotherapy depends on starting treatment as soon as possible with or without HBO. After the 14th day, sensitisation to combined HBO and C60 is seen. The extension of pulmonary metastases is a function of tumor growth. Paradoxically metastases were less frequent after HBO only and more frequent after HBO-Co 60.

[Primary microvascular lesions of the kidney or pre-hypertensive nephroangiosclerosis. 25 cases]. / Lésions microvasculaires primitives du rein ou néphroangiosclérose pré-hypertensive. 25 observations.

Isolated non inflammatory lesions of renal microarteries (eventually with mild thickening of tubular basement membranes, but with negative immunofluorescent glomerular studies) were observed in 25 patients (22 males) in whom renal biopsy have been performed for proteinuria (P). Selection criteria were: pathological lesions by definition; absence of hypertension (HT) in clinical and at the time of biopsy; minimum follow up of 4 years after the first statement of the proteinuria (4 to 29 years; mean 14 years). Three groups have been isolated: 1. 3 patients have had an acute glomerulonephritis followed by disappearance of proteinuria. It reappears 1 to 5 years later. HT was discovered 2, 8 and 11 years after the proteinuria. Renal failure occurred 1 and 3 years after HT. 2. 14 patients had hereditary or acquired vascular risk factors (obesity, smoking, ethylism). In 7, HT occurred 3 to 15 years after P. In 2, renal failure occurred 4 to 8 years later. 3. 8 patients had no vascular risk factor; in 3 of them Ht developed 7, 13 and 20 years after the first statement. A positive immunofluorescence with IgM or C3 on renal arterioles had been found in only 3 of the 10 patients who in group 2 and 3 became hypertensive. A proteinuria may precede the occurrence of HT without being induced by glomerulonephritis. Group 2 and 3 suggest that these renal lesions of arterial sclerosis precede and may be a factor of HT. Indeed, this entity may be considered as a prehypertensive condition.

[Study of ventricular repolarization in an experimental model of arterial hypertension associated with obesity]. / Etude de la repolarisation ventriculaire dans un modèle expérimental d'HTA associée à l'obésité.

UNLABELLED: High fat diet (HFD) in dogs is associated with obesity and hypertension (HTN) but also with a significant and early decrease in heart rate variability (HRV). Decreased HRV has been shown to be a good predictor of sudden cardiac death due mainly to arrhythmic event. The aim of this work was to investigate the changes in ventricular repolarization through 24 hours EKG recordings in dogs with hypertension and rendered obese by 20 weeks of HFD. This was achieved through 24 hour EKG recording analysis of QT parameters. The aims of this work was i) feasibility of this method in dogs and ii) identification of potential arrhythmic risk factors that could explain overmortality during obesity. METHOD: Six dogs received a high fat diet (HFD) ad libitum during 20 weeks. A 24 hour EKG recording was realized just before and after 20 weeks of HFD. The following parameters studying QT interval were collected: QT interval lasting from the beginning of the Q wave to the apex (QTa) and to the end of the T wave (QTe), QT intervals plotted against RR intervals and two regression lines were calculated characterized by their slope and intersection with the Y axis, QT dispersion (longest minus shortest QT interval for each RR value) as well as the difference of QT interval between night and day at a fixed RR value considered as a marker of the sympathovagal balance. Our results show that HFD significantly increased body weight, blood pressure, heart rate, left ventricular mass and insulinemia. QT dispersion was increased in a non-significant manner both during day (+35%) and night (16%) for QTa and only during day for QTe (+27%). This increased dispersion of QT was not associated to any increase of QT interval. There was no effect of HFD on QT dynamicity parameter nor on the night-day difference at any RR interval from 300 to 1,300 ms. CONCLUSION: HFD tend increase QT dispersion without any effect on QT interval. These results are compatible with a heterogeneous repolarization probably related to abnormal autonomic nervous system tone. This study could partly explain occurrence of lethal arrhythmias during obesity which might lead to overmortality of obese patients. These results are different for QTa and QTe, but these two parameters are characterizing different type of ventricular cells. This study confirms the feasibility of this method in an experimental model, but results need to be validated in larger groups and in human.

[Early atrial gene regulation of obesity-related arterial hypertension]. / Modifications précoces du transcriptome auriculaire dans l'hypertension artérielle associée à l'obésité chez le chien.

High fat diet (HFD) induces both arterial hypertension and tachycardia in dogs. Changes in heart rate occur early and are in part due to a decrease in the parasympathetic drive to the heart secondary to down-regulation of atrial muscarinic M2 receptors (Pelat et al. Hypertension 1999; 340: 1066-72). These data suggest that HFD is able to modify genic expression at atrial level. Thus, the aim of this work was to perform a systematic study of the genic expression profile in dogs made obese and hypertensive by 9 weeks of HFD. Blood pressure and heart rate were measured by telemetry implanted 15 days before starting regimen in 6 HFD and in 6 control dogs. HFD was the normal canine diet administered to controls but mixed with 300 g of beef fat. At the end of the experience, animals were sacrified and right atria were collected. Gene regulation was assessed in pooled tissue samples from both groups using suppressive substractive hybridization and microarray analysis. Genes with induction or repression rates of at least 20% when compared to controls were sequenced. As previously reported HFD induced a significant increase in body weight, blood pressure and heart rate when compared to controls. The results of SSH experiments led to the identification of 32 genes which are differentially regulated in atria from HFD dogs. Most are genes encoding proteins which have been previously shown to be regulated during various cardiopathies (MMP9, Na/K-ATPase 3...). These changes indicate the existence of early remodeling processes of atrial myocardium secondary to HFD. Other group of genes encodes proteins with no role identified in heart up today (lec-3, ERK-3, TRIP1, nucleophosmin...) or which function remains totally unknown. This work confirms that HFD is associated with early changes in gene expression in atrium. These changes are unlikely to be related to ventricular hypertrophy which is observed only during long-term HFD. Further studies are necessary to demonstrate the role of these modifications in the pathophysiological mechanisms leading to the increase in heart rate in this model of obesity-related arterial hypertension.

[Nervous system and lymphomatoid granulomatosis. Clinical, pathological and nosological considerations (author's transl)]. / Granulomatose lymphomatoïde et système nerveux. Aspects anatomo-cliniques et problèmes nosologiques.

This paper describes the first French case of lymphomatoid granulomatosis (LYG) in 46-year-old male. Autopsy revealed bilateral pulmonary involvement and multiple central nervous system (CNS) localizations. The originality of this study is due to 1st the scarcity of reported cases of LYG in Europe 2nd a detailed neuropathological study permitting diagnosis of multifocal central nervous involvement by LYG. In the literature only 12 previous cases of LYG have histologically proven CNS lesions. These lesions correspond to; 8 instances of LYG, one progressive multifocal leukencephalopathy,, one post-therapeutic disseminated necrotizing leukoencephalopathy, one immunoblastic sarcoma and one case of LYG associated with histiocytic malignant lymphoma. This paper also evokes clinical, histological, ultrastructural and prognostic characteristics of LYG gathered from the available literature. From a nosological point of view the relationship of LYG with Wegener granulomatosis, polymorphic reticulosis and certain lymphomas is still, to this day, not clearly established.

[The metastatic potential of primary central nervous tumours (author's transl)]. / Le potentiel métastatique des tumeurs primitives du systeme nerveux central.

Anatomical and clinical findings in 9 cases of primary brain tumors with metastases within (7 cases) or outside of (2 cases) the nervous system are reported. A review of the published literature revealed 248 cases of histologically-confirmed central nervous system tumors with metastases outside of the nervous system. These secondary sites are seen more frequently in adult males. Their frequency, in descending order, was gliomas (39,5 p. 100), meningeal tumors and sarcomas (28,2 p. 100), neuronal tumors (14,1 p. 100), lymphoma-microgliomas (12,5 p. 100), pineal body tumors (4 p. 100), and melanomas (1,6 p. 100). Ten cases out of 248 developed through a shunt. Nineteen cases were discovered without any surgical operation. There are still many unknown factors concerning their pathogenesis. It appears, however, than in some series of patients the prolongation of the survival-rate and the late effects of surgical operation are positive factors for metastatic dissemination.

[Mixed cryoglobulinemia with necrotizing angiitis. Apropos of 2 cases]. / Cryoglobulinémies mixtes avec angéites nécrosantes. A propos de deux observations.

Out of a series of 26 personal cases, 2 cases of mixed IgM-IgG cryoglobulinemia, one type II the other type III, are reported because they were associated with histologically proven necrotizing vasculitis. In both cases the numerous symptoms were due to renal damage (the vasculitis was discovered in the kidney) and to peripheral neuropathy. One of the patients died; the other had severely deteriorated general condition and required substitution hemodialysis. Cases of vasculitis associated with mixed cryoglobulinemia have often been published, but there are few reports mentioning necrotizing vasculitis; a search in the literature yielded only 9 cases. This small number does not mean that mixed cryoglobulinemia should not be listed among the causes of necrotizing vasculitis, but it makes it difficult to extract those specific features that would enable to predict which case of mixed cryoglobulinemia is associated or not with necrotizing vasculitis.

[Association of thymus carcinoma, Hashimoto's thyroiditis and polymyositis. Anatomoclinical case with autopsy findings]. / Association d'un carcinome thymique, d'une thyroïdite de Hashimoto et d'une polymyosite. Une observation anatomoclinique avec données autopsiques.

A 67-year-old woman presenting with a 9-month history of polymyositis, suddenly worsened her clinical state and died. An autopsy was performed, finding a tumor measuring 8 x 6 x 1 cm, located in the left pulmonary hilum. The thyroid gland, of normal volume, was firm. The histological analysis of the tumor, the thyroid gland and several skeletal muscles led to the following diagnoses: thymic carcinoma, Hashimoto's thyroiditis and polymyositis. The aim of the discussion is this thymic carcinoma, the originality of which are firstly its ectopic location, secondly its association with immune diseases usually described during the course of thymomas rather than thymic carcinomas.

[Glial fibrillary acidic protein and central nervous tumors. Immunohistochemical study of a series of 207 cases. 1: Astrocytomas. Glioblastomas. Ependymomas. Papillomas of the choroid plexus]. / Protéine gliofibrillaire acide (GFA) et tumeurs nerveuses centrales. Etude immunohistochimique d'une série de 207 cas. 1re partie: Astrocytomes. Glioblastomes. Ependymomes. Papillomes des plexus choroïdes.

The presence of glial fibrillary acidic protein (GFA) was tested in biopsy or autopsy specimens of 207 human central tumors. Samples were fixed, paraffin-embedded and GFA was detected using the peroxidase-antiperoxidase technic. The first part of this study dealt mainly with glioblastomas (36 cases) and astrocytomas (87 cases). Tumors were classified in three categories according to their histological grade: 24 astrocytomas grade I and II, 63 astrocytomas grade III and IV and 36 glioblastomas (grade IV). For each of these tumors GFA positive cells were counted in 5 different fields using objectives of 2,5, 10 and 25. The results were as follows: less than 50% GFA positive cells were found in 22 out of 24 low grade astrocytomas, 49 out of 63 high grade astrocytomas and 32 out of 36 glioblastomas. Conversely, over 50% GFA positivity was found in 2 benign astrocytomas, 14 malignant astrocytomas and 4 glioblastomas. In 3 high grade tumors (2 astrocytomas, 1 glioblastoma) GFA positivity was found to be over 75%. Of the 17 ependymomas, 14 were GFA positive; however, no correlation could be established between the degree of GFA positivity and histological grade. The three papillomas of the choroid plexus of the lateral ventricles found in children were GFA negative. In adults, a GFA positive focus of ependymal differentiation was found in a papilloma of the choroid plexus in the posterior cerebral fossa.

[Cranial fasciitis in children. Apropos of a case in a neonate with duramateral involvement]. / Fasciite cranienne de l'enfant. A propos d'un cas à révélation néonatale avec extension dure-mérienne.

A case of cranial fasciitis in the skull of a male child is described. At the age of a few weeks was noted a "lump" beneath the scalp of the right parietal region. Physical examination was otherwise normal. Radiographs showed erosion of the skull deep to the palpable mass. At operation, a firm, 2,5 X 2 X 2 cm nodule was found, which had destroyed the underlying bone and invaded the dura matter. This nodule both extra and intracranial was excised en bloc with the surrounding bone. Histological features were those of a fibroblastic proliferation closely resembling nodular fasciitis. The child is well and symptom-free a year after treatment. The authors also include diagnostic, prognostic and histogenetic comments about cranial fasciitis of childhood, new entity described by Lauer and Enzinger in 1980.

[Glial fibrillary acidic protein and central nervous system tumors. Immunohistochemical study of a series of 207 cases. 2: Medulloblastomas. Hemangioblastomas. Other tumors. Discussion]. / Protéine gliofibrillaire acide (GFA) et tumeurs nerveuses centrales. Etude immunohistochimique d'une série de 207 cas. IIe Partie: Médulloblastomes. Hémangioblastomes. Autres tumeurs. Discussion.

The presence of glial fibrillary acidic protein (GFA) was tested in biopsy or autopsy specimens of 207 human central nervous system tumors. Samples were fixed, paraffin-embedded and GFA was detected using the peroxidase-antiperoxidase technique. The second part of this study dealt mainly with medulloblastomas and hemangioblastomas. Of the 17 cerebellar medulloblastomas, 15 contained less than 5% GFA positive cells. These were classified according to the Mannoji et al., 1981, criteria. Ten medulloblastomas contained type 1, 2 and 3 cells; 1 was composed of only type 1 cells, and 4 were composed of only type 3 cells. Of the 8 cerebellar or medullary hemangioblastomas, 3 contained only a few GFA positive cells which were, in two cases stroma cells and in another, astrocytes. GFA positive cells were found in 7 oligoastrocytomas, 3 gangliogliomas and 1 hamartoma of the hypothalamus. Similarly, a GFA positive astrocytic differentiation was found in a pineocytoma. In a case of tuberous sclerosis no GFA was found in the giant cells of cortical tubers. Nor was GFA observed in one intracerebral tumor of tuberous sclerosis. GFA was not found in 5 meningiomas, 4 oligodendrogliomas, 4 pituitary adenomas, 3 neurinomas of the VIII cranial nerve, 1 primary cerebellar malignant lymphoma, 1 familial lymphohistiocytosis with cerebellar involvement and 4 brain metastases. The authors also include diagnostic, histogenetic and nosological comments -about neuroglial tumors. The findings in the two parts of this study fail to establish a correlation between GFA positivity and histological grade of astrocytomas and glioblastomas.

[Epidural angiolipoma: a rare and curable cause of spinal cord compression]. / L'angiolipome épidural: une cause rare et curable de compression de la moelle épinière.

A 48-year-old female developed a slowly progressive paraparesis due to a posterior epidural mass extended on several thoracic segments. Clinical and myelographic appearance was strongly in favour of metastasis, the most common tumors in the spinal epidural space. A laminectomy was carried out and disclosed an encapsulated epidural tumor which was loosely adherent to the dura matter. Histological features were those of an angiolipoma. A brief review of clinical and pathological characteristics of spinal lipomas is performed. Extradural spinal angiolipomas are unusual benign neoplasms. The results of surgical treatment in this location are mostly excellent.

[Spontaneous bone marrow micrometastasis of a cerebral glioma. Immunohistochemical diagnosis in a biopsy sample and review of the literature]. / Micrométastase ostéomédullaire spontanée d'un gliome cérébral. Diagnostic immunohistochimique sur un prélèvement biopsique et revue de la littérature.

A 55 year-old woman was admitted to hospital in January 1981 with transient expressive dysphasia. Past personal history was unremarkable except for a six-month history of renal colic and thrombophlebitis in the veins of the right leg. Computed tomographic scan of the head and carotid angiogram revealed a left calcified temporoparietal tumor. Because of pulmonary embolism it was decided to refute a cerebral biopsy. The patient also declined radiotherapy. In May 1983, a thorough workup revealed an incomplete fracture of the first lumbar vertebra and a diffuse demineralization of the rachis and pelvis. Four weeks later she developed temporal epilepsy and pulmonary embolism. A whole brain irradiation (60 Gy) was performed in August 1983. The patient's condition remained clinically stable until December 1984 when she was readmitted to hospital with a severe weight loss, diffuse osseous pain and pancytopenia. A bone marrow biopsy from the iliac crest showed a diffuse tumor involvement. Peroxidase-antiperoxidase staining using monoclonal antiserum to glial fibrillary acidic protein was strongly positive in numerous tumors cells. The pathological diagnosis was bone marrow metastasis by glioma. She died in March 1985, 4 years and 3 months after the first admission to hospital. Autopsy was not performed. A literature search reveals only 9 cases of extraneural spreading of astrocytomas and glioblastomas in the absence of previous craniotomy with post-mortem examination. The authors also comment on the clinical, pathological and histogenic aspects of extraneural metastasis of gliomas.