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Biomedical subjects

D Figarella-Branger

Publications and source records attributed to D Figarella-Branger.

At least 73 records · Page 4Linked to original sources

[Central neurocytoma revealed by intraventricular hemorrhage. A case report and review of the literature].

A case of intraventricular tumor presenting with intraventricular hemorrhage in a 38-year-old man is reported. Initial symptoms were an acute onset of headache with projectile vomiting. CT-scan revealed intraventricular hemorrhage. Cerebral angiography did not show any vascular malformation. MRI study, performed initially and several days after, disclosed and confirmed the presence of an intraventricular mass. The patient was operated on via a transfrontal route, and a complete tumor removal was achieved. Pathological examination was consistent with a central neurocytoma. Two similar cases from the literature are discussed with respect to differential diagnosis and practical management considerations.

Adult↗

[Burkitt lymphoma of the cavernous sinus. Apropos of a case].

Cavernous sinus lymphoma is a rare occurrence. We report a case of a 37-year-old woman who was admitted for an oculo-motor paresis and retro-orbital pain. CT scan and MRI disclosed a cavernous sinus tumor. A surgical biopsy was performed through a pterional approach and the histological diagnosis was a solitary primary Burkitt's lymphoma. Aggressive chemotherapy led to a complete and asymptomatic remission after a follow-up of 9 years. The mechanism of the primary localization in the cavernous sinus is discussed.

Adult↗

Immunohistochemical localization of cytokines, C5b-9 and ICAM-1 in peripheral nerve of Guillain-Barré syndrome.

The spectrum of the Guillain-Barré Syndrome (GBS) has recently been widened by the newly identified forms of acute motor axonal neuropathy (AMAN) and acute motor sensory axonal neuropathy (AMSAN). An important question has been raised regarding the possibility for the axon to be a target in immune-mediated damage. Although myelin breakdown is the characteristic feature of classic acute inflammatory demyelinating polyradiculoneuropathy (AIDP), axonal degeneration may occasionally be observed in this form, especially in cases with explosive onset and severe clinical course. Immunohistochemical findings of five frozen sural nerves biopsies of patients with GBS (AIDP variant) tested with a panel of monoclonal antibodies raised against different molecules implicated in immune-mediated processes have principally disclosed an immunoreactivity of tumor necrosis factor-alpha (TNF-alpha) on both Schwann cell membranes and in myelinated and unmyelinated axons. On the other hand, interleukin 1-beta (IL1-beta) labeled Schwann cells, endothelial cells and macrophages; interferon-gamma (IFN-gamma) was observed both in endothelial cells and lymphocytes. Membrane attack complex (C5b-9) deposits were observed on Schwann cell membranes and finally intercellular adhesion molecule-1 (ICAM-1) was localized both on endothelial cells and macrophages. Our findings strongly suggest that TNF-alpha is an important factor in the cascade of events leading to immune-mediated demyelination and axonal damage in GBS.

Adult↗

Polysialylated-neural cell adhesion molecule expression in rat pituitary transplantable tumors (spontaneous mammotropic transplantable tumor in Wistar-Furth rats) is related to growth rate and malignancy.

Pituitary adenomas are usually benign neuroendocrine tumors. However, some of those that are histopathologically undistinguishable behave aggressively and metastasize. The polysialylated neural cell adhesion molecule (PSA-NCAM), which is highly expressed during the development of the brain and pituitary, is detected in some neuroendocrine tumors and might be relevant as a prognostic marker in pituitary tumors. In the present study, we have searched for PSA-NCAM expression in four lineages of rat pituitary transplantable tumors (SMtTW). Each lineage, maintained by serial tumor grafts under the kidney capsule and skin, differed in its GH/Prl secretion, growth rate, and malignant behavior. PSA-NCAM expression, detected by immunohistochemistry and Western blotting and quantified by ELISA, varied according to the SMtTW lineage. The benign tumors, SMtTW2, with a low growth rate never expressed PSA-NCAM. Another benign lineage, SMtTW3, with a high growth rate expressed a low amount of PSA-NCAM. The highest PSA-NCAM expression was seen in tumors that grew beneath the skin, invaded the kidney, and metastasized (SMtTW4). Tumors of the SMtTW10 lineage, which behaved as either benign or malignant tumors, were heterogeneous in terms of PSA-NCAM expression. In this rat transplantable pituitary tumor model, PSA-NCAM expression correlated in decreasing order with: (a) invasiveness (P < 0.0001), (b) metastases (P = 0.004), (c) ability to grow under the skin (P = 0.006), and (d) growth rate under the kidney capsule (P < 0.01), but not with hormone secretion (r = 0.207). This model, which is very similar to the human pathology, suggests that PSA-NCAM evaluation is of interest in the diagnosis of malignancy and the prognosis of human pituitary tumors. In addition, the SMtTW tumors could be instrumental in evaluating the effects of new therapeutic agents modulating PSA-NCAM expression.

Animals↗

Papillary glioneuronal tumour: clinicopathological and biochemical study of one case with 7-year follow up.

Among the mixed glioneuronal tumours, a new variant called papillary glioneuronal tumour has recently been delineated. A case occurring in a 23-year-old man is reported. The tumour was cystic with a mural nodule enhanced by gadolinium injection. It was located within the left parieto-occipital lobe. Surgical excision showed a greyish friable tumour with cystic areas. Histopathological examination revealed a pseudopapillary component comprising a single layer of regular cells, arranged around hyalinised vessels. These cells were immunoreactive with anti-glial fibrillary acidic protein and HNK1 antibodies. A neurocytoma-like component coexisted with round blind cells and focal fibrillary rosettes. These cells were immunostained by anti-neuron-specific enolase and anti-synaptophysin antibodies. Neither mitoses nor ganglioid cells were seen. HNK1, the three isoforms of NCAM, and the L1 adhesion molecule were detected by Western blot analysis. Ultrastructural study showed three different types of cells. The first contained gliofilaments, the second showed long processes with true synapses, and the third was poorly differentiated. However, all had identical nuclei and contained dense bodies. These findings suggest a common origin for the tumour cells derived from a bipotential neuroglial progenitor. As for other mature mixed neuroglial tumours, the prognosis is good. Our patient is free of disease 7 years after complete surgical treatment.

Adult↗

Intracranial pseudolymphoma.

Intracranial pseudolymphoma is a rare tumor of the central nervous system. A 35-year-old woman presented with a frontal subcutaneous tumor. Magnetic resonance imaging revealed a left frontal meningeal tumor involving subcutaneous tissue without bone involvement. The mass was completely removed and the histological aspect of all tumor sections was that of a lymphoid hyperplasia with polyclonal proliferation. These findings were characteristic of pseudolymphoma defined as a hyperplasia of follicular and diffuse lymphoid type with assessment of its polyclonality by immunophenotyping on frozen sections, completed by molecular biology techniques.

Adult↗

Linkage of X-linked myopathy with excessive autophagy (XMEA) to Xq28.

X-linked myopathy with excessive autophagy (XMEA, MIM 310440) is a rare inherited mild myopathy. We have used 32 polymorphic markers spanning the entire X chromosome to exclude most of the chromosome except the Xq28 region in a large XMEA family. Using three additional families for linkage analysis, we have obtained a significant two-point lod score with marker DXS1183 (Z = 2.69 at theta = 0). Multipoint linkage analysis confirmed the assignment of the disease locus with a maximal lod score of 2.74 obtained at recombination fraction zero. Linkage of XMEA to the Xq28 region is thus firmly established. In addition, we have ruled out the Emery-Dreifuss muscular dystrophy to be allelic with XMEA by direct sequencing of the emerin gene in three of our families.

Biopsy↗

Prognostic factors in intracranial ependymomas in children.

OBJECT: The occurrence of intracranial ependymomas in children is relatively infrequent, and their prognostic factors are still controversial, especially regarding histological composition. METHODS: A retrospective study was conducted of 37 children treated during the last 20 years for intracranial ependymomas at the Hôpital de la Timone. Both univariate and multivariate statistical analyses were performed to assess the prognostic relevance of patient age and sex, extent of tumor removal, location of the tumor (supratentorial compared with infratentorial, median compared with lateral), tumor histological composition, and adjuvant therapies in affecting the 5-year progression-free survival (PFS) rate and overall survival (OS) rate. The following histopathological features, either alone or in combination, were analyzed: endothelial proliferation, necrosis, loss of differentiating structures (present compared with absent), the number of mitotic figures per 10 hpf, and cellularity (number of nuclei/5 hpf). In addition, immunohistochemical detection of Ki-67 antigen was performed and the Ki-67 labeling index (LI) evaluated in all cases. The 5-year OS and PFS rates were 45% and 25%, respectively (median follow up 34 months). Four patients died of disease without remission (median 163 days) and disease in 21 patients relapsed: 18 in situ and three both in situ and distantly. On univariate analysis total surgical resection and median infratentorial location were associated with a better outcome (p < 0.002) for both OS and PFS. Loss of differentiating structures was associated with poor prognosis (p < 0.008) and the combination of necrosis, endothelial proliferation, and mitotic index greater than 5 was also a negative predictive factor for both OS (p < 0.002) and PFS (p = 0.02). The PFS time was shorter in patients younger than 4 years of age and in patients in whom a Ki-67 LI greater than 1 was found (p = 0.03 and 0.006, respectively). Adjuvant radiotherapy and chemotherapy were not relevant to prognosis. Moreover, among the 15 patients in whom total excision was achieved, OS was better in those who did not receive adjuvant therapies. In contrast, adjuvant therapies significantly enhanced PFS time in patients in whom tumor excision was incomplete. CONCLUSIONS: This study and analysis of the literature further highlight that total tumor removal is the treatment of choice for ependymomas in children. Postoperative measurement of residual tumor is required, especially because a subgroup of patients might be treated by surgery alone. Median infratentorial ependymomas have to be distinguished from the lateral type. Appropriate and reproducible histological parameters and Ki-67 LI are of interest as predictors of outcome.

Adolescent↗

[Pseudometabolic distrophinopathy without immunohistochemical anomaly].

A 9 year old boy suffering from migraines, vomiting and exercise intolerance was hospitalized. Clinical examination revealed calf hypertrophy only. There was no muscular deficit and cardiac examination was normal. Creatine kinase and transaminase were elevated. Muscle biopsy revealed fibers of various sizes, centrally located nuclei, occasional necrotic and regenerative fibers. Interstitial tissue was normal. Immunohistochemistry with various antibodies directed against the membranous dystrophin complex was normal. Western Blot analysis revealed dystrophin of abnormal size, and multiplex PCR confirmed the dystrophinopathy showing an absence of exon 43 and 44. This observation highlights the occurrence of unusual dystrophinopathies revealed by exercise intolerance and pseudo-metabolic syndrome. Normal anti-dystrophin immunostaining does not rule out the diagnosis which may only be made by Western Blot analysis or genetic studies.

Child↗

[Synovial cyst of cervical spine].

The synovial cyst is a rare cause of cervical medullar compression. We report on one case of a 74-year-old man hospitalized for electric discharge in the limbs at neck extension. The MRI and CT-scan revealed a posterior extradural cystic lesion at the level of C5-C6. The surgical excision of the lesion resulted in completed recovery. The histological examination confirmed the diagnosis of a synovial cyst. Through this case and the review of the 9 reported cases in the literature, we discuss the etiopathogenesis, diagnosis and treatment of the lower cervical synovial cyst.

Aged↗

[Contribution of immunohistochemistry and electron microscopy for the diagnosis of meningeal hemangiopericytomas. 15 case reports].

Meningeal hemangiopericytomas (HPC) are rare CNS tumors with a pour prognosis compared to meningiomas. In order to define diagnosis criteria, we performed an immunohistochemical and ultrastructural study in respectively 15 and 5 meningeal HPC. The following antibodies anti-KL1, EMA, vimentin, CD34, factor VIII, alpha-smooth actin, estrogen and progesteron receptors (RE, RP) were used in paraffin embedded sections whereas anti-NCAM and E-cadherin antibodies were used on frozen sections when available. We can differentiate meningeal HPC from meningioma because of a complete lack of immunostaining with epithelial markers as well as with NCAM antibody or RE and RP receptors. Besides a positivity with CD34 and alpha-smooth actin antibodies was always observed even focally in HPC. On the other hand, solitary fibrous tumor showed a strong and diffuse positivity with anti CD34 and anti-vimentin antibodies. Electron microscopy can be helpful in some instances showing membrane basal-like substance and absence of desmosomes.

Adult↗

Sequence of expression of MyoD1 and various cell surface and cytoskeletal proteins in regenerating mouse muscle fibers following treatment with sodium dihydrogen phosphate.

An immunohistochemical study was performed in order to evaluate the sequence of expression of various cell surface proteins [neural cell adhesion molecule (NCAM) and its polysialylated isoform, PSA NCAM, and utrophin], cytoskeletal proteins (myosin heavy chain isoforms, desmin) and the transcription factor MyoD1 in regenerating mouse muscle fibers following treatment with sodium dihydrogen phosphate. The sequence of the regeneration process with this new myotoxic agent is similar to that which can be observed with other myotoxic substances (local anaesthetics such as bupivacaine or snake venoms). The results show that NCAM, PSA NCAM and desmin were already present on the first day after injury in the presumptive myoblasts. The highest level of all of these proteins was observed on the third day. At this stage, regenerating muscle fibers also strongly and diffusely expressed myosin heavy chain isoforms and utrophin throughout their sarcolemma, whereas MyoD1 expression was observed in the regenerating myonuclei. PSA NCAM and MyoD1 had gradually disappeared from the muscle fibers by the seventh day, by which time, the expression of the other developmentally regulated proteins had also decreased. On the 21st day after injury, a few fibers still expressed NCAM but not the other proteins. This study first shows that sodium dihydrogen phosphate is a new myotoxic agent that is cheap, widely available and easy to handle. It also establishes the schedule of expression of various developmentally regulated proteins in regenerating mouse muscle fibers.

Animals↗

Sequence of expression of MyoD1 and various cell surface and cytoskeletal proteins in regenerating mouse muscle fibers following treatment with sodium dihydrogen phosphate.

An immunohistochemical study was performed in order to evaluate the sequence of expression of various cell surface proteins [neural cell adhesion molecule (NCAM) and its polysialylated isoform, PSA NCAM, and utrophin], cytoskeletal proteins (myosin heavy chain isoforms, desmin) and the transcription factor MyoD1 in regenerating mouse muscle fibers following treatment with sodium dihydrogen phosphate. The sequence of the regeneration process with this new myotoxic agent is similar to that which can be observed with other myotoxic substances (local anaesthetics such as bupivacaine or snake venoms). The results show that NCAM, PSA NCAM and desmin were already present on the first day after injury in the presumptive myoblasts. The highest level of all of these proteins was observed on the third day. At this stage, regenerating muscle fibers also strongly and diffusely expressed myosin heavy chain isoforms and utrophin throughout their sarcolemma, whereas MyoD1 expression was observed in the regenerating myonuclei. PSA NCAM and MyoD1 had gradually disappeared from the muscle fibers by the seventh day, by which time, the expression of the other developmentally regulated proteins had also decreased. On the 21st day after injury, a few fibers still expressed NCAM but not the other proteins. This study first shows that sodium dihydrogen phosphate is a new myotoxic agent that is cheap, widely available and easy to handle. It also establishes the schedule of expression of various developmentally regulated proteins in regenerating mouse muscle fibers.

Animals↗

Local expression of monocyte chemoattractant protein-1 (MCP-1) in idiopathic inflammatory myopathies.

The idiopathic inflammatory myopathies (IIM), including dermatomyositis (DM), polymyositis (PM), and inclusion body myositis (IBM), are a group of autoimmune diseases characterized by the recruitment of lymphocytes and monocytes to the site of affection. The mechanism for recruitment of these cells likely involves chemokines. The monocyte chemoattractant protein-1 (MCP-1), a chemoattractant to T lymphocytes and monocytes, may play an important role in the pathogenesis of IIM. Frozen muscular tissues were obtained from eight cases of DM, five PM, and four IBM. We investigated the MCP-1 expression by the reverse transcription-PCR technique, immunohistochemistry and in situ hybridization. MCP-1 mRNA was markedly expressed in all the IIM cases. Greater amounts of MCP-1 mRNA were observed in DM, and in situ hybridization showed MCP-1 mRNA accumulation in perivascular mononuclear cells. Immunohistochemistry showed MCP-1 expression in vessels (endothelial cells and walls of veins and arteries) in all IIM cases. Very few mononuclear cells in DM perivascular infiltrates expressed MCP-1, whereas in PM and IBM, it was strongly expressed by mononuclear cells partially invading non-necrotic muscle fibers. These findings suggest that MCP-1 can contribute to the inflammatory response by attracting monocytes and T lymphocytes to sites of cell-mediated immune injury. The morphological characteristics and distribution of positive cells indicate that macrophages, lymphocytes, and endothelial cells previously reported to produce MCP-1, contribute to its production in IIM lesions.

Adult↗

The PEN5 epitope identifies an oligodendrocyte precursor cell population and pilocytic astrocytomas.

PEN5 is a sulfated polylactosamine carbohydrate epitope first described in a subpopulation of mature natural killer cells. Here we report that it is also expressed in a developmentally regulated fashion in human and rat central nervous systems and that its protein carrier is P-selectin glycoprotein ligand-1 (PSGL-1), a ligand for selectins. In rat neural primary cultures, PEN5 is transiently and selectively expressed by oligodendrocyte precursor cells and marks the transition from proliferative to postmitotic stages. In concordance, in human central nervous system tumors, PEN5 is observed in a subset of oligodendrogliomas and in all pilocytic astrocytomas, a class of tumor of uncertain histogenesis. These data suggest that PEN5-PSGL-1 plays a role in the differentiation of oligodendrocytes and that pilocytic astrocytomas are likely to result from a dysregulation occurring in oligodendrocyte precursor cells at the crucial stage of exit from the cell cycle.

Amino Sugars↗

Cytogenetic study of 33 ependymomas.

Ependymomas are glial tumors. They constitute approximately 5-10% of intracranial tumors. Ependymomas are tumors which can recur. Predictive factors of outcome in ependymomas are not well-established. Karyotypic studies on ependymomas are relatively scarce, and no specific chromosomal change has been described in these neoplasms. We performed a cytogenetic study of 33 ependymomas, of which eight were recurrent tumors, to determine the type and incidence of cytogenetic changes.

Adolescent↗