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PubMed2006

Clinical characteristics in focal cortical dysplasia: a retrospective evaluation in a series of 120 patients.

Focal cortical dysplasias (FCDs) are increasingly diagnosed as a cause of symptomatic focal epilepsy in paediatric and adult patients. However, little is known about the clinical characteristics of epilepsy in these patients. In order to elucidate the clinical characteristics of their epilepsy, 120 pharmacoresistant patients including children and adults with histologically proven FCD were studied retrospectively. Age at seizure onset was analysed in the total group and compared between subgroups with different localization and different histological subtypes of FCD. The role of febrile seizures with respect to dual pathology was investigated. Seizure semiology was analysed focusing on initial seizure type and change of seizure semiology during the course of disease. Finally, transient responsiveness to antiepileptic drug therapy was studied. In the majority of patients, epilepsy began in the first 5 years of life. However, onset of epilepsy could also occur in the second or third decade until the age of 60. Age at epilepsy onset was not significantly different between temporal, extratemporal and multilobar localization of FCD. Patients without cytoarchitectural abnormalities (mild malformations of cortical development, FCD 1a according to Palmini) had significantly later epilepsy onset (P= 0.001) compared with patients with cytoarchitectural abnormalities (FCD 1b, 2a and 2b according to Palmini). In patients with additional hippocampal sclerosis (dual pathology) febrile seizures were significantly more frequently reported (P = 0.02) than in patients without dual pathology. Moreover, patients with dual pathology and febrile seizures significantly more frequently presented with severe hippocampal sclerosis (Wyler Grade 3-4) as compared with patients with dual pathology in the absence of febrile seizures (P = 0.03). First observed seizures were mainly tonic or generalized tonic-clonic. A change of seizure semiology seemed to be age-dependent and occurred between the age of >1 and 14 years. About 15.8% of the patients presented with status epilepticus during the course of disease. About 17% of the patients showed transient responsiveness (> or =1 year seizure freedom) to antiepileptic drug therapy either after initial therapy (50%) or later in the course of epilepsy (50%). Patients with FCD represent a heterogeneous group. Different age at epilepsy onset and transient responsiveness to antiepileptic drugs in approximately 17% of patients may reflect different dynamics in epileptogenicity of the underlying FCD. Dual pathology may be associated with different pathomechanisms in patients with and without febrile seizures.

Adolescent↗

PubMed1996

The U.S.-Japan Cooperative Cancer Research Program: some highlights of seminars, interdisciplinary program area, 1981-1996.

Thirty-one seminars have been held in the 16 years since 1981. A principal interest from the beginning was the genetics of cancer, well before this subject became widely popular. This interest arose in part because of marked binational differences in type-specific cancer rates, such as the very low rates among Japanese for Hodgkin's disease in the young, testicular cancer, Ewing's sarcoma, superficial spreading melanoma, chronic lymphocytic leukemia, and Wilms' tumor (half the U.S. frequency). Three seminars were devoted to the seeming reciprocal relationship between B-cell lymphoma (low in Japan) and certain autoimmune diseases (high in Japan), which is perhaps similar in origin to the male/female differences in the rates for these diseases. A seminar on Li-Fraumeni syndrome led to the recognition of cases among Japanese pedigrees brought to the meeting, and generated a study of its occurrence in Japanese families with adrenocortical carcinoma in a child. Another seminar revealed a marked clustering of rare cancers in Werner's (premature aging) syndrome in Japan, and led to a binational study and analysis of case-reports worldwide. Three seminars on pathology heightened appreciation of the importance of subclassifying cancer by subsite and subtype for racial and other comparisons. Four seminars on biostatistics in cancer research generated a substantial exchange of specialists and trainees in this field.

Adult↗

PubMed2004

Cyclooxygenase-2 expression and relationship to tumour progression in human renal cell carcinoma.

AIMS: Cyclooxygenase (COX), which catalyses the synthesis of prostaglandins from arachidonic acid, has two isoforms; COX-1 and COX-2. There is ample evidence to suggest an important role for COX-2 in cancer. The aim of this study was to evaluate the clinical significance of COX-2 expression and its localization in the development and progression of human renal cell carcinoma (RCC). METHODS AND RESULTS: The expression and localization of COX-2 were evaluated in human RCC tissues from 75 patients by immunohistochemistry. Immunoreactive COX-2 protein was observed in all cases of RCC, and the levels of COX-2 expression were correlated with tumour grade and pathological stage. Expression of COX-2 was higher in the granular cell subtype than in the clear cell subtype of RCC. Immunoelectron microscopy revealed that COX-2 was expressed in the nuclear membrane, rough endoplasmic reticulum, Golgi complex and mitochondrial membrane of RCC cells. CONCLUSION: COX-2 overexpression within these intracellular organelles in RCC may be associated with renal cell carcinogenesis and COX-2 may be a useful biomarker in RCC.

Adult↗

PubMed2000

Histological subtypes of symptomatic central nervous system tumours in Singapore.

The objective was to identify the different subtypes of symptomatic CNS tumours that are encountered in Singapore. Our hospital pathology and operative records from 1994 to 1998 were reviewed and information regarding all patients who underwent biopsy or resection as part of their diagnostic and therapeutic evaluation was extracted. Only histologically confirmed tumours were included in this analysis. Meningiomas made up the largest subgroup of tumours, accounting for 35.1% of all tumours. In order of decreasing frequency, the remaining most often reported histologies were pituitary adenomas (11.8%), secondary neoplasms (10%), tumours of nerve sheath (9.4%), glioblastoma multiforme (9.3%), astrocytomas including anaplastic, diffuse and pilocytic (9.2%), primary CNS lymphomas (2.9%), oligodendrogliomas (2.2%), hemangioblastomas (2. 2%), craniopharyngiomas (1.7%), and embryonal tumours (1.2%). Genetic and environmental factors may be responsible for the proportionately higher than expected percentage of meningiomas seen and further study is required to identify these factors.

Adolescent↗

PubMed2004

Bronchioloalveolar carcinoma: a model for investigating the biology of epidermal growth factor receptor inhibition.

Bronchioloalveolar carcinoma (BAC) is a previously uncommon subset of non-small cell lung cancer (NSCLC) with unique epidemiology, pathology, clinical features, radiographic presentation, and natural history compared with other NSCLC subtypes. Recent data suggest that the incidence of BAC is increasing, notably in younger nonsmoking women. Despite reports of prolonged survival after repeated surgical resection of multifocal lesions and slow growth kinetics, advanced bilateral or recurrent diffuse BAC remains incurable, with the vast majority of patients dying of respiratory failure or intercurrent pneumonia within 5 years. Limited data suggest that chemotherapy may yield poor results in BAC. However, anecdotal reports of prolonged complete response to tyrosine kinase inhibitors of the epidermal growth factor receptor (EGFR), a member of the human epidermal growth factor receptor (erbB) family, have raised considerable interest in studying this NSCLC subset. Here we present clinical data and preliminary results of correlative science studies analyzing human epidermal growth factor receptor pathways from the following two prospective Southwest Oncology Group clinical trials performed in advanced stage BAC: S9714 testing a 96-h continuous infusion of paclitaxel (Taxol) and S0126 evaluating the small molecule EGFR inhibitor gefitinib (ZD1839 or Iressa). These studies provide a biological rationale for investigating BAC as a model of predictive markers of EGFR inhibition.

Adenocarcinoma, Bronchiolo-Alveolar↗

PubMed2003

Incidence, risk factors and prognosis of stroke and TIA: the need for high-quality, large-scale epidemiological studies and meta-analyses.

Stroke is a considerable clinical, social and economic burden. In recent clinical trials, a number of strategies have been shown to reduce the risk of stroke and transient ischaemic attack (TIA) in both primary and secondary prevention settings. Whether these treatments are leading to a significant reduction in the incidence of first and recurrent stroke in the clinic, however, remains unclear due to a paucity of high-quality epidemiological data. A similar lack of reliable epidemiological studies has undermined our understanding of the relationship between many potentially important vascular risk factors and stroke risk. Improvement in our knowledge of stroke epidemiology is a prerequisite for the planning of stroke services, the effective application of current stroke prevention strategies, the development of new strategies, and our understanding of the mechanisms of stroke. Future studies must take into account the clinical and pathological heterogeneity of TIA and stroke, and must be powered to allow subtype differences in risk factor relationships and prognosis to be determined reliably. In many cases, this will require meta-analysis of detailed individual patient data from multiple independent studies.

Epidemiologic Studies↗

PubMed1992

Recurrence of Hodgkin's disease after 10 years: observation of 5 cases.

Five patients whose Hodgkin's disease recurred 10 years or more after the achievement of complete remission are reported. Four out of 5 patients had shown an advanced stage at the onset of disease and therefore had received combination chemotherapy (ABVD or MOPP-like) as primary treatment. Involved-field irradiation was delivered as consolidation therapy in 1 patient only. All pathologically proven relapses occurred in a previously affected area and the histologic subtype differed from the primary subtype. The time interval between the achievement of the first complete remission and the relapse was 120, 170 and 190 months in 3, 1, and 1 patient, respectively. All 5 patients are alive in second complete remission. This paper emphasizes the risk of late relapse even after a prolonged disease-free interval for patients with Hodgkin's disease. Close follow-up is therefore recommended also in this clinical setting.

Adult↗

PubMed1983

Treatment of small cell carcinoma of the bronchus by combined chemotherapy--clinicopathological correlation with response.

Thirty-seven consecutive patients, referred to a respiratory unit with a histological diagnosis of small cell carcinoma of the bronchus, were treated with a combined chemotherapy regime of adriamycin, vincristine and cyclophosphamide. Palliative radiotherapy was given for the control of symptoms. Thirty of 37 patients responded to treatment, 11/37 having a complete and 19/37 a partial response. The median survival of those patients with a complete response was 55 weeks, significantly higher (P less than 0.01) than either those with partial or no response to treatment. Those patients with limited disease survived longer. A high proportion of patients relapsed at the primary site of the tumour in the lungs. The relationship between the therapeutic response and the histological subtype of small cell carcinoma suffered from the limitations of the available pathological material. However, more of the patients with a complete response were of the lymphocytic-like pattern of small cell carcinoma. Useful survival in these patients with inoperable small cell carcinoma was achieved using this regime of chemotherapy, even in those patients with extensive disease, five of whom had survived for over one year.

Adult↗

PubMed1993

Species-specific properties of the glomerular mesangium.

Mesangial cells play a central role in the physiology and pathophysiology of the glomerulus. To date, most of the in vitro studies have been performed in cultured rat mesangial cells, with only 10% of them performed in human mesangial cells. In this article, the major differences between results obtained with these two types of cultured cells will be reviewed. In rats and in humans, most of the mesangial cells appear to be of smooth muscle origin. In the rat, some of the cultured cells also express a phenotype suggesting a monocyte/macrophage origin. Phagocytosis and synthesis of cytokines or proinflammatory proteins that have been described in cultured rat cells seem mostly linked to this monocyte/macrophage subtype of resident mesangial cells. In humans, macrophages are only detected in pathologic conditions, suggesting that they are not resident but rather infiltrating cells. Mesangial receptors, most notably angiotensin II receptors, are clearly present on mesangial cell membranes and are linked to prostaglandin E2 synthesis and to cell contraction. In humans, spontaneous prostanoid synthesis is low and is increased by the induction of cyclooxygenase by sodium butyrate in the medium. Even so, the amount of prostaglandin E2 synthesized by human mesangial cells is quantitatively low comparatively with that in rats. In rats, accordingly, mesangial cells play a role in the regulation of single-nephron GFR. In humans, angiotensin II also exerts a control on GFR but it is more difficult to demonstrate its contractile effect on human than on rat mesangial cells.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

PubMed1998

Tall cell variant of papillary thyroid cancer.

Papillary histologic type is the most common form of thyroid carcinoma amounting to 85% cases. This pathology presents a rather good prognosis, but a few years ago, new subtypes have been described. Some of these variants show a fairly good prognosis i.e follicular, macropapillary, encapsulated while others appear to have a decidedly worse one, columnar cells, diffused sclerosing, or even to be clearly malignant as in the case of the tall cell variant. The authors report a case treated by a combined surgical and radiometabolic therapy and evidence the main characteristics of this rare and underestimated neoplasia.

Carcinoma, Papillary↗

PubMedDate not supplied

Colloid carcinoma of the breast with concomitant metastasis and a tuberculous lesion in the axillary lymph nodes. A case report.

A 30-year-old woman presented with a lump in the left breast and left axillary lymphadenopathy that, on fine needle aspiration cytology (FNAC), proved to be duct cell carcinoma with metastasis. Histology of the radical mastectomy specimen showed a mixed colloid carcinoma. Axillary lymph nodes revealed a variety of pathologic changes consisting of reactive hyperplasia, tuberculosis and metastasis. A combination of a tuberculous lesion and metastasis in the same lymph nodes was also found. During follow-up, after radiotherapy, the patient developed left supraclavicular and right cervical lymphadenopathy that, on FNAC, revealed a tuberculous lesion and metastasis, respectively. The rarity of this condition with double pathology is highlighted, and the reason behind the limitations of FNA in subtyping the primary malignancy and its failure to detect the tuberculous lesion in the axillary lymph node are discussed.

Adenocarcinoma, Mucinous↗

PubMedDate not supplied

Quantitative analysis of p53 expression and cell proliferation in gastric carcinomas. An immunohistochemical study.

BACKGROUND/AIMS: Adenocarcinoma of the stomach is still among the leading malignancies in human morbidity and mortality statistics in spite of endoscopic screening of the high-risk patients. Investigation of prognostic factors of gastric cancer disease seems to be still very important. The authors present a clinicopathological study based on the analysis of 49 gastric carcinomas. METHODOLOGY: P53 overexpression and proliferation activity of the cells were examined by immunohistological method with peroxidase-antiperoxidase technique. The percentage of the positive cells was calculated after counting of 300 tumor cells in each case. The rate of the labeled cells was related to different pathological characteristics of the carcinomas i.e., TNM stage of the tumor, histological subtypes of Ming's as well as Laurén's and Goseki's classification respectively, grade of differentiation and lymph node status. RESULTS: According to the above-mentioned parameters, p53 overexpression was significantly higher in carcinomas of the cardiac region than in those of the distal parts of the stomach. These findings are consistent with results published in the literature: cell proliferation rate alone is not an independent prognostic factor, but the degree of cell proliferation activity and p53 expression are changing usually parallel with each other and with other prognostic markers as well. CONCLUSIONS: The assessment of p53 activity and cell proliferation rate in gastric carcinoma is of prognostic value especially if evaluated together with other clinical and histopathological characteristics. The examination of these markers is useful in detecting early gastric cancer, in selecting high-risk patients and in planning proper individual treatment.

Adenocarcinoma↗

PubMed2004

[Treatment of primary mediastinal large B-cell lymphomas].

INTRODUCTION: Primary mediastinal large B-cell non-Hodgkin's lymphoma is a relatively rare disease with specific clinical symptoms. This tumour originates from a subset of B-cells of the thymus and at the time of the diagnosis the disease is predominantly localised in the mediastinum. The tumor grows rapidly and frequently involves other thoracic structures. The majority of the patients are young females. There are no histologic features that reliably distinguish these tumors from other diffuse large B-cell lymphomas. This is the only lymphoma subtype which can only be defined by the combination of clinical and pathologic features. Analysis with DNA microarrays verified that primary mediastinal and diffuse large B-cell lymphomas are different diseases. AIMS: Comparing the effectiveness of two types of anthracycline-based standard chemotherapy regimens and the evaluation of the prognostic markers which are applied in large B-cell lymphomas. METHODS: 27 patients with primary mediastinal lymphoma were treated by the authors with anthracycline-based polychemotherapy with complementary radiotherapy from January 1995 to December 2002. RESULTS: Complete remission was obtained in 15 patients (56%) and no relapse was observed in this group. 9 additional patients (33%) achieved partial remission, while in 3 cases (11%) the treatment was ineffective. The patients who failed to achieve complete remission were subsequently treated with more intensive chemotherapy. Afterwards, those patients who were chemosensitive, underwent high-dose chemotherapy with autologous peripheral blood stem-cell transplantation. The chemoresistant patients received palliative chemotherapy. The 5-year overall survival rate of the 27 patients was 62.11%. CONCLUSION: The authors found that the procarbazine, prednisolone, adriamycin, cyclophosphamide, etoposide, cytosine-arabinoside, bleomycin, vincristine, methotrexate treatment was more effective than the cyclophosphamide, adriamycin, vincristine, prednisolone combination. The expected 5-year overall survival rates were 83.57% vs. 33.33%, respectively. This difference was significant (p = 0.017). No prognostic value of age adjusted international prognostic index, LDH- and b2-microglobulin levels were found. The results with the new standard of combined immuno-chemotherapy (rituximab--cyclophosphamide, adriamycin, vincristine, prednisolone) seem to be hopeful and more effective than earlier treatments.

Adult↗

PubMedDate not supplied

[Graf's method in the ultrasound diagnostics of developmental dislocation of the hip].

The authors present the possibilities of ultrasound diagnostics of developmental dislocation of the hip. Special attention has been paid to Graf's method, originally developed in Austria and Germany that has been introduced in many countries all over Europe, including our country. The method in details, normal and pathological anatomy of the infant's hip, all the sonographic types and subtypes with therapeutic implications were described.

Hip Dislocation, Congenital↗

PubMed2006

[Neuroimaging in the diagnosis of human transmissible spongiform encephalopathies].

INTRODUCTION: The role of neuroimaging in the diagnosis of the Human Transmissible Spongiform Encephalopathies (HTSE) has been changing from the exclusion of other conditions to the contribution of diagnostic data having incalculable utility, such as basal ganglia and cerebral cortex in different magnetic resonance imaging (MRI) sequences. METHODS: Search in Medline. Reading and analysis of related papers. RESULTS: There are alterations such as increased of the signals in the caudate nucleus, putamen and cerebral cortex in the sporadic Creutzfeldt-Jakob disease (CJD) or increased signal intensity in the pulvinar for the variant Creutzfeldt-Jakob disease (vCJD). These signals are associated with the molecular subtype in sporadic cases, stage and duration of the disease and with pathological findings. CONCLUSIONS: Although magnetic resonance imaging (MRI) is only currently included in the vCJD diagnostic criteria, it is a useful diagnostic test in the diagnosis of other HTSE, especially diffusion-weighted and FLAIR images.

14-3-3 Proteins↗

PubMed1990

Clinical and pathologic features of ceroid lipofuscinosis in two Australian cattle dogs.

A lysosomal storage disease was diagnosed in 2 Australian Cattle Dog siblings, using light and electron microscopic evaluation. Both dogs developed clinical signs of disease at about 1 year of age. Vision and motor function deteriorated over several months; by 2 years of age, the dogs were blind and had progressive ataxia. Cytoplasmic inclusions with ultrastructural patterns characteristic of ceroid lipofuscin were observed in most neurons examined and in the cells of several other parenchymatous tissues. Biochemical studies, including determination of lysosomal enzyme activities, excluded several other lysosomal storage diseases. In these dogs, the clinical and pathologic features of the disease were similar to those of the juvenile subtype of ceroid lipofuscinosis (Batten disease) in human beings.

Adrenal Medulla↗

PubMed1986

Lymphocyte-depletion Hodgkin's disease. Histopathologic diagnosis of marrow involvement.

Lymphocyte-depletion Hodgkin's disease (LDHD) is a rare and often misdiagnosed form of HD. Although marrow involvement is frequent in this disease, most pathologists are not familiar with the marrow lesion of LDHD, since the underlying disease is so unusual. In order to characterize the marrow lesion produced by LDHD, we reviewed biopsies or aspirates from the initial presentation of 22 patients meeting all the clinical and pathologic criteria for LDHD. These included 11 cases of the diffuse fibrosis subtype, eight cases of reticular subtype, and three cases not subclassified. Fifty-four percent of cases, primarily of the diffuse fibrosis subtype, had marrow involvement. Aspirations and biopsies were positive with essentially the same frequency. LDHD produces a characteristic consolidated lesion readily recognized on low-power examination and composed of amorphous, nonbirefringent eosinophilic background material, an inflammatory infiltrate, and Reed-Sternberg (RS) cells. Involvement may be focal and RS cells are generally scarce, requiring examination of multiple sections of well-fixed and stained material. Uninvolved marrow tends to be normocellular and frequently has increased numbers of eosinophils. The original diagnosis of LDHD often may be made from marrow examination alone. Early recognition of marrow involvement is important in facilitating prompt treatment and providing accurate staging without laparotomy.

Adult↗

PubMed1994

[Instability of the shoulder joint in the athlete].

In all shoulder instabilities it is very important to classify the type of instability precisely in order to choose the right form of therapy and predict the results. The acronyms TUBS, which means traumatic instability, unidirectional, Bankart lesion, and good response to surgery, and AMBRI, which means atraumatic aetiology, multidirectional, and good for rehabilitation, represent the complete range of possible instabilities. We discuss the subtypes in the differentiation of various instabilities and the different causes and pathologies for instability, the clinical and radiological tests possible, and the different surgical treatment options, as well as the results in the literature. A modification of the Bankart procedure and the arthroscopic Caspari capsulorrhaphy procedure for traumatic instabilities are described. The capsular T-shift by Neer and Foster is explained as a surgical treatment for multidirectional instability.

Arthroscopes↗