Hepatic undifferentiated embryonal sarcoma: malignant evolution of mesenchymal hamartoma? Study of one case with immunohistochemical and flow cytometric emphasis.
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Biomedical subjects
Publications and source records attributed to A De Mascarel.
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Pyoderma gangrenosum is an ulcerative disease of the skin. The histopathological lesions are nonspecific, characterized by a diffuse neutrophilic infiltrate in the dermis. Pyoderma gangrenosum is associated with inflammatory, digestive or articular disease, or acute or chronic hemotology disorders in 50% of the cases, more rarely with monoclonal gammapathy. A visceral localization of pyoderma gangrenosum is rare, simulating a systemic disease or an underlying neoplasia. We report a case of cutaneous pyoderma gangrenosum with splenic and hepatic localizatios associated with an IgG monoclonal gammapathy. We emphasize the efficacy of immunosuppressor treatment and the importance of long-term monitoring of these patients.
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Cytogenetic analyses have revealed that mantle cell lymphomas (MCL) are closely associated with the t(11;14)(q13;q32). This translocation juxtaposes the immunoglobulin heavy chain gene (IGH) sequences with the BCL-1 locus, leading to up-regulation of the CCND1 gene and consequently to an overexpression of cyclin D1 protein. We studied 27 MCL with characteristic morphological and immunological (CD5+, CD10-, CD20+, CD23-) features and 2 controls (reactionnal lymphadenitis) to evaluate the feasibility and the interest of FISH analysis on interphase cells from frozen or paraffin-embedded tissues. Sections (CC) and touch preparations (EC) of frozen tissues and sections of paraffin-embedded tissues (CF) were successfully hybridized with the Vysis LSI IgH/CCND1 dual color dual fusion translocation probe. The touch preparations presented a lower cellularity than sections, therefore allowing an easier analysis. Hybridization spots intensities were found stronger in CC and EC than in CF. The percentages of t(11;14) positive cells were similar in CC, EC and CF from a same patient. The percentage of non hybridized cells, analogous in CC and EC, was higher in CF. However, the CF were directly analysed on microscope without the need of any numerical picture treatment. The t(11;14) was detected in all the cases (27/27) and positive cells percentages were always higher than the probe cut-off (5%). The FISH analysis on interphase cells appears a performing and rapid technique to detect t(11;14) in MCL on both frozen and paraffin-embedded tissue, thus extending its practical and diagnostic use.
The course of mycosis fungoides (MF) is indolent except when transformation to a large T-cell lymphoma occurs. The diagnosis of transformed MF (T-MF) relies on the presence of more than 25% of large cells on biopsy of an MF lesion. We analyzed 45 patients with T-MF recorded by the French Study Group on Cutaneous Lymphomas to better determine clinicopathological features of MF transformation and to analyze their impact on prognosis. Median time from diagnosis of MF to transformation was 6.5 years. Extracutaneous progression was present in 20 patients. Mean survival from transformation to death was 22 months. In univariate analysis, only an extracutaneous progression was associated with a worse prognosis (5-year actuarial survival: 7.8% versus 32%). Neither sex, age, clinical and skin disease stage at transformation, transformation speed, nor percentage of large cells or CD30 expression (14 of 45) had a prognostic value. When performing multivariate analysis, age (at least 60 years), and extracutaneous spreading were found to be associated with a poor prognosis. There was no difference between survival curves of patients with T-MF and with pleomorphic large T-cell CD30- lymphomas. The main diagnostic pitfall was "histiocytic-rich" MF, requiring CD68 staining for the diagnosis of T-MF. Out of 45 patients, 6 presented an histologic transformation before clinical progression, suggesting that an early histopathological diagnosis may be performed by histological follow-up. The prognostic value of such early histopathological diagnosis must be confirmed by prospective studies.
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Primary Cardiac leiomyosarcoma is a very rare condition. The authors report the case of a 64 year old woman admitted to hospital for suspected pulmonary embolism, confirmed by thoracic angio-CT scan. Despite thrombolytic therapy, a persistent poor haemodynamic condition associated with a superior vena cava syndrome led to a transoesophageal echocardiographic examination which demonstrated an abnormal intra-right atrial mass obstructing the tricuspid valve. At surgery, a leio-myosarcoma was successfully resected under good conditions. During systematic three-monthly clinical and echocardiographic follow-up, transoesophageal echocardiography detected an asymptomatic local recurrence at 15 months, for which surgery was repeated with adjuvant chemotherapy.
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BACKGROUND: The necessity of increasing intragastric pH during eradication treatment in Helicobacter pylori infected patients is well established. However, the optimal dose of the proton pump inhibitors used in eradication regimen is still a subject of debate. AIMS: To compare the efficacy and tolerability of a double vs. a single daily dose of pantoprazole in a 7-day triple therapy in eradicating H. pylori. METHODS: In this regional, multicentre, comparative, randomized and double-blind study, H. pylori-positive patients with non-ulcer dyspepsia were treated for 7 days with clarithromycin 500 mg b.d. and amoxycillin 1000 mg b.d. and either a double (2 x 40 mg, Group 2PCA) or a single (40 mg, Group 1PCA) daily dose of pantoprazole. H. pylori infection was assessed at entry and at the end (day 38) of the study by histology and culture, or in some cases by 13C-urea breath test. RESULTS: From 203 patients recruited, 192 patients (96 in Group 2PCA and 96 in Group 1PCA) formed the intention-to-treat population. Twenty-six of them judged as major protocol violators were excluded from the per protocol analysis. H. pylori eradication rate was 75% in Group 2PCA and 56% in Group 1PCA in intention-to-treat analysis, and 80% in Group 2PCA and 59% in Group 1PCA in per protocol analysis (P < 0.05). The primary resistance to clarithromycin was 10.5%. The eradication rates for the clarithromycin susceptible strains were 86% for Group 2PCA and 71% for Group 1PCA in per protocol analysis (P < 0.05). Both regimens led to similar improvement of clinical symptoms and were equally well tolerated. CONCLUSION: A double (2 x 40 mg) daily dose of pantoprazole in a 7-day triple therapy is more effective than a single (40 mg) dose of this drug in eradication of H. pylori.
We studied 21 HIV-associated lymphomas with cutaneous presentation to determine whether they showed features of primary cutaneous lymphoma arising fortuitously or whether they represented the cutaneous involvement of AIDS systemic lymphoma. Besides rare mycosis fungoides (n = 3), which shared typical clinicopathologic lesions, nonepidermotropic large-cell lymphomas (n = 18) were predominant. They frequently presented as a solitary nodule or tumor. Seven of the eight large T-cell lymphomas had a CD30-positive (CD30+) phenotype but did not express ALK protein. Overexpression of p53 protein was observed in six cases. Although EBV-EBER transcripts were detected in two of them, LMP1 protein was absent. Except for their original prevalence, the features of these T-cell CD30+ cutaneous lymphomas were the same as in immunocompetent patients. The 10 B-cell cutaneous lymphoma were immunoblastic or centroblastic lymphomas, with a differential expression of BCL-6 and Syndecan. Four of them expressed CD30, EBER-EBV transcripts, and LMP1 and p53 proteins. This B-cell CD30+ EBV+ phenotype contrasts with cutaneous lymphoma in immunocompetent patients. Human herpesvirus 8 was not involved in lymphomagenesis since its sequences were detected in a single patient with Kaposi's sarcoma and Castleman's disease. These lymphomas occurred in severely immunocompromised patients with a low CD4 count. Death was due to immunodepression rather than to lymphoma spread, suggesting avoiding aggressive immunosuppressive treatment in such patients.
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We report a fatal primary cardiac non-Hodgkin's lymphoma in a 62 years old immunocompetent woman presenting with tamponade and complete atrioventricular block. CT-scan, echocardiography and autopsy examination showed a tumor largely infiltrating the heart without extracardiac involvement. A surgical biopsy revealed high grade B-cell non-Hodgkin's lymphoma with a misleading myelomonocytic CD68 (KPI) expression. Polymerase Chain Reaction analysis revealed a clonal rearrangement of the immunoglobulin heavy chain gene and confirmed the B-cell origin of the lymphoma. Our report also emphasizes the role of immunohistochemical and molecular techniques in the diagnosis.
Some thyroid neoplasms notoriously cause problems in the histologic diagnosis of their nature (follicular versus papillary for example) or their malignant potential (adenoma versus carcinoma) because of overlapping histologic features. Here, thyroid lesions are presented according to their histologic presentation (vesicular, papillary, trabecular, solid or cystic patterns, fibrosing thyroids and lymphoid infiltrate). For each entity, the most discriminant histologic features are described with emphasis on the diagnostic pitfalls and their histological clues.
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Hyperthyroidism is usually classified as follows: with diffuse or with toxic plurinodular goiter, respectively considered as autoimmune and non-autoimmune thyroid disorders. This classification seems partially inadequate as signs of thyroid immunity may be found in some plurinodular toxic goiter and alternatively may by lacking in some cases of Graves' disease. These observations led us to study the intensity of intrathyroidal autoimmune process (IAP) and the levels of TBIAb, TPO- and Thyroglobulin-antibodies in 105 cases with diffuse goiter, hyperthyroidism and elevated RAIU (92 women and 13 men, age 34 +/- 11, mean +/- SD). The intensity of intrathyroidal autoimmune process (IAP) was determined by one pathologist (HT) by semi quantitative method applicable to routine clinical use. Subtotal thyroidectomy was performed because of a large goiter (n = 29), a concomitant cold nodule (n = 20), a recurrent disease (n = 18), intolerance to antithyroid drugs (n = 5) or because patients chose surgical treatment (n = 33). All cases were rendered euthyroid at the time of surgery using antithyroid drugs or iodine. The results show a lack of IAP and undetectable levels of TBIAb, TPO- and Thyroglobulin-antibodies in 10%, 11%, 25% and 47% respectively. The intensity of IAP was not different in case of first episode or recurrence of hyperthyroidism and was not related to type or duration of medical treatment. Comparison of patients with or without IAP show higher levels of TPO- and thyroglobulin-antibodies but not of TBIAb in the former group (P < 0.005). TBIAb were higher when ophthalmopathy and/or dermopathy were present vs absent (p < 0.05) and were correlated with FT4 levels (p < 0.05). The negative predictive value of TBIAb, TPO- and thyroglobulin-antibodies to predict the lack of significant IAP was 42%, 65% and 64%. The total absence of clinical, biological and histological signs of thyroid autoimmunity was found in only one case (female aged 35 with first episode of hyperthyroidism and no family history of thyroid disease) (0.9%). These results suggest that routine available criteria of thyroid immunity (including IAP) have a low specificity. It follows that they are probably inadequate to screen cases of hereditary toxic familial hyperplasia, a rare entity of still unknown prevalence.