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Clivus chordoma.

Chordomas are slow growing, locally invasive tumors that most commonly present as midline masses in the sacrococcygeal or clival regions. The case presented in this paper demonstrates the typical MRI appearance of a clival chordoma in a patient presenting with classic symptoms of cranial nerve compression and headache. While the exact signal characteristics of these lesions seen on MRI may vary with pathologic subtypes, MRI has proven essential in the diagnosis, surgical planning and post-treatment evaluation of patients with these lesions.

Chordoma↗

Prognostic factors among 130 patients with osteosarcoma.

One hundred thirty patients with high grade osteosarcoma were enrolled in a randomized prospective multidisciplinary treatment that included intraarterial chemotherapy, local irradiation, limb salvage surgery, and prophylactic whole lung irradiation. The patients were evaluated to stage the prognostic factors. In a multivariate analysis, a minimal level of serum lactic acid dehydrogenase less than 300 IU/L showed a significant prognostic value. The history of trauma before diagnosis of disease, local irradiation of the affected site, histologic response to preoperative multidisciplinary therapy, and prophylactic whole lung irradiation were associated with significantly better prognosis in the log rank test. Patient age, site of the primary tumor, presentation of fracture, pathologic subtype, signs and symptoms, serum alkaline phosphatase level, and erythrocyte sedimentation rate were not found to be prognostic factors. The 9-year survival rate of the whole group was 55%.

Adolescent↗

[Prognostic factors in uterine sarcomas: a 21-year retrospective study at the Clinic and Provincial Hospital of Barcelona].

BACKGROUND: Uterine sarcomas show low incidence and poor outcome despite the treatment. The prognostic factors for the survival were determined in this study. PATIENTS AND METHODS: Thirty-nine females with sarcoma of the uterus have been studied retrospectively from January 1975 to December 1996. They were treated in the Gynecology and Radiation Oncology Departments at Hospital Clínic i Provincial of Barcelona. Thirty-seven patients had surgery, 22 radiotherapy and 4 chemotherapy. The influence on the disease-specific survival, disease-free survival, local relapse disease-free survival and metastasis disease-free survival from the following pronostic factors was studied: age, pathologic subtype, miometrial invasion, mitosis, vascular and lymphatic invasion, tumor size, stage, radiotherapy and local relapse. RESULTS: 1) The disease-specific survival at 2 and 5 years was 51.5% and 42.5% respectively, and the disease-free survival at 2 and 5 years was 39%; the incidence of local and distant relapses--was 28 and 33%. 2) The multivariate analysis showed that the overall survival and the disease free survival were affected by the vascular invasion (odds ratio [OR] 12 and 32.6, respectively) and the local failure (OR = 3 and 25.5, respectively); the only factor that affected the local relapse-free survival and metastasis free survival was the III and IV stages (OR = 5.6 in both cases). CONCLUSIONS: In uterine sarcomas, the vascular invasion and the local relapse were prognostic factors for overall survival and for disease-free survival. In stages III and IV there was a decrease in the local relapse-free survival and metastasis-free survival. A correlation between vascular invasion and advanced stages was found. The outcome of the uterine sarcomas is poor, local and distant failure being responsible for this bad prognosis.

Adult↗

Guillain-Barré syndrome: recent advances.

Guillain-Barré syndrome is an autoimmune disease of peripheral nerves. Sufferers may require prolonged intensive care and may be permanently disabled. New understanding of the aetiology is revealing different pathological subtypes and helping predict the outcome. Recent advances have improved immunomodulatory and general treatment.

Critical Care↗

Intraluminal mucin pool in mucinous gastric adenocarcinoma: a case report.

Mucinous gastric carcinoma is a rare pathologic subtype of gastric adenocarcinoma. Whether the mucin behaves aggressively as in mucinous colorectal carcinoma is still controversial. Most mucinous gastric carcinomas are diagnosed from surgical specimens. The mucinous gastric carcinoma in this case report was discovered preoperatively according to its characteristic presentation. An upper gastrointestinal endoscopic examination showed a round protruding tumor of greater than 4 cm in size on the mid-body of the stomach; it had an uneven, friable and shiny surface. The surface was coated with a sticky layer of mucin-like substance, which persisted even after the aspiration of the gastric juice. Endoscopic ultrasonography (EUS) revealed a large heterogeneous hyperechoic tumor mass, originating from the mucosal and submucosal layers, on the body of the stomach. The mass was covered with a thick layer of hypoechoic amorphous substance. Hence, a mucin-producing tumor was suspected. Subsequent surgical biopsy proved the mass to be a moderately differentiated mucinous adenocarcinoma. This case illustrates the first endoscopic ultrasonographic report of an intraluminal mucin pool as a hypoechoic substance, which is quite different from the hyperechoic presentation of intramural mucin lakes. In preoperative evaluation, EUS is not only important for determining the depth of tumor invasion, but it is also useful in differentiating mucinous gastric carcinoma from nonmucinous gastric carcinoma.

Adenocarcinoma, Mucinous↗

Follicular lymphoma without t(14;18) and with BCL-6 rearrangement: a lymphoma subtype with distinct pathological, molecular and clinical characteristics.

Translocations involving the BCL-6 gene are frequently observed in diffuse large B cell lymphoma, but have rarely been reported in follicular lymphoma (FL). We studied a distinct cohort of FLs with a 3q27/BCL-6 gene rearrangement, but lacking the t(14;18) translocation. In 13/15 cases, translocations involved the 3q27 and the 14q32 regions. All cases displayed a marked follicular growth pattern and, in some instances, a monocytoid component. Tumor cells were CD5(-) CD20(+) CD23(-) CD43(-) BCL-6(+), and in the main CD10 negative (n = 10, 71%) and BCL-2 negative (n = 11, 78%). When compared to 20 typical t(14;18)(+) FLs, the presence of large follicles (P = 0.01) and a CD10(-)/BCL-2(-) phenotype were more frequently observed (P = 0.001) in our cohort. Clonal mutations arising in the BCL-6 first intron were observed in 5/7 cases with evidence of intraclonal heterogeneity, consistent with a germinal center origin. No significant difference was found in comparison to t(14;18)(+) FL regarding age, sex, performance status, bone marrow involvement or overall survival. However, in the 3q27(+) FL group, a stage III/IV disease and a bulky mass were less frequently observed. This study indicates that 3q27(+) FL without t(14;18) translocation have peculiar clinico-pathologic features and may correspond to a rare and distinct subtype of lymphoma originating from the germinal center.

Adult↗

Perfectionism in anorexia nervosa: variation by clinical subtype, obsessionality, and pathological eating behavior.

OBJECTIVE: The purpose of this study was to examine the role of perfectionism as a phenotypic trait in anorexia nervosa and its relevance across clinical subtypes of this illness. METHOD: The Multidimensional Perfectionism Scale and the perfectionism subscale of the Eating Disorder Inventory were administered to 322 women with a history of anorexia nervosa who were participating in an international, multicenter genetic study of anorexia nervosa. All participants were additionally interviewed with the Yale-Brown Obsessive Compulsive Scale and the Yale-Brown-Cornell Eating Disorder Scale. Mean differences on dependent measures among women with anorexia nervosa and comparison subjects were examined by using generalized estimating equations. RESULTS: Persons who had had anorexia nervosa had significantly higher total scores on the Multidimensional Perfectionism Scale than did the healthy comparison subjects. In addition, scores of the anorexia subjects on the Eating Disorder Inventory-2 perfectionism subscale exceeded Eating Disorder Inventory-2 normative data. For the anorexia nervosa participants, the total score on the Multidimensional Perfectionism Scale and the Eating Disorder Inventory-2 perfectionism subscale score were highly correlated. Total score on the Multidimensional Perfectionism Scale was also significantly related to the total score and the motivation-for-change subscale score of the Yale-Brown-Cornell Eating Disorder Scale. CONCLUSIONS: These data show that perfectionism is a robust, discriminating characteristic of anorexia nervosa. Perfectionism is likely to be one of a cluster of phenotypic trait variables associated with a genetic diathesis for anorexia nervosa.

Adolescent↗

[Pathologic diagnosis and subtyping of lymphoma in bone marrow biopsies using histologic examination, immunohistochemistry and gene rearrangement studies].

OBJECTIVE: To assess the value of histologic examination, immunohistochemistry and gene rearrangement studies in the diagnosis and subtyping of lymphoma with bone marrow involvement (BMI). METHODS: Sixty-two formalin fixed, paraffin embedded bone marrow biopsy specimens were studied. Immunohistochemical and immunoglobulin heavy chain (IgH) and T-cell receptor gene rearrangement studies were performed in each case. RESULTS: Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) demonstrated mainly and interstitial infiltration by dysplastic lymphocytes, with intertrabecular nodular arrangement or in dispersion. Sometimes, pseudofollicles may be noted. A predominantly para- or intertrabecular infiltration by nodules of lymphoma cells was characteristic of follicle center cell lymphoma (FCL) cases. In most lymphoplasmacytoid lymphoma (LPL) cases, there was infiltration by small lymphocytes and plasma cells between bony trabeculae. In marginal zone cell lymphoma (MZL), vague inter- or para-trabecular nodules of polymorphic lymphoma cells with clear cytoplasm might be noted. Small to medium-sized dysplastic lymphocytes, with absence of paraimmunoblasts or pseudofollicles, were the most frequent findings in mantle cell lymphoma (MCL). Hairy cell leukemia (HCL) might be identified by the presence of distinct cell membrane and abundant clear cytoplasm, resulting in a "fried-egg" appearance. Tumor cells with large nuclei and eosinophilic nucleoli were characteristically seen in lymphomatosis diffusa (Hodgkin's disease, HD). In T-cell non-Hodgkin lymphoma with BMI, dispersed or clusters of intertrabecular neoplastic lymphoid cells with clear cytoplasm and gyriform nuclei were often observed. In diffuse large B-cell lymphoma (DLBL), the tumor cells were large and isolated or arranged in diffuse pattern. Immunohistochemically, a panel of markers, including CD3 CD20, and CD79 are valuable for the differential diagnosis of T- and B-cell lymphomas. The neoplastic cells in MCL were cyclin D1- and CD5-positive, while BCL2- and CD10-positivity was characteristic for FCL. CLL/SLL cells might be stained with CD5 and CD23, in addition to CD20 and CD79. CD25 expression might be noted in HCL: the positivity for CD15, CD30 and fascin suggests HD. There was a higher positivity rate for IgH gene rearrangement in CLL/SLL, LPL MZL and DLBL (80%, 60%, 66.7%, 70% respectively) and for T- cell receptor gamma gene rearrangement in T-cell lymphoma (66.7%). CONCLUSION: A combination of histopathology, immunohistochemistry and IgH / T-cell receptor gamma gene rearrangement studies may be of aid to the diagnosis and subtyping of lymphoma with BMI, especially if there is only a small number of tumor cells present in the specimen.

Adolescent↗

[Pathologic anatomy of subtypes of immunoblastic lymphosarcoma].

Morphological parameters for immunoblastic lymphosarcoma (IL) subtypes: plasmocytic IL (PIL) and polymorphocellular IL (PMIL) were determined through a comparative morphological analysis of 56 sections and 64 respective diagnostic biopsies. PIL was found to more frequently affect the gastrointestinal tract. Tumors were found to be selective in damaging the organs, e.g. white pulp was affected in PIL, marginal follicular areas, in PMIL. Clinical and anatomical analyses of the death causes in patients with IL showed that they all had died of neoplastic progression (67.9%), concurrent diseases (23.2%) or complications resulted from the therapy performed (8.9%) upon tumor progression.

Adolescent↗

Postinfectious glomerulonephritis. Subtypes, clinico-pathological correlations, and follow-up studies.

APGN (WHO: diffuse endocapillary proliferative glomerulonephritis) has long been considered one of the best described kidney afflictions, clinically characterized by the sudden development of a nephritic syndrome after a latency period following a nasopharyngeal infection or pyoderma. Proliferation of mesangial and endothelial cells in the endocapillary space, aggregation of polymorphonuclear granulocytes in the capillary lumina, and deposition of predominantly subepithelial immune complexes on the glomerular basement membrane (so-called "humps") are to the present day considered characteristic of renal morphology. However, the nature of the antigen (or antigens) as well as the determining mechanisms in the pathogenesis of APGN still are unclear. Considerable disagreement also exists regarding the prognosis. An analysis of APGN is once again presented to elucidate whether the morphological picture of APGN is really as uniform as has been generally assumed. A large number of kidney biopsies was examined and subjected to the triad of light microscopy (LM), immunofluorescence microscopy (IFM) and electron microscopy (EM). The findings, which were recorded at an early stage of APGN in all cases (i.e. during the first 9 weeks), were related to clinical data, age (childhood or adulthood), and etiology (e.g. streptococci, staphylococci). In addition, clinical and morphological follow-up over a period of up to 10 years in those cases, which had been carefully documented in the early stages, afforded an insight into the dynamics and the prognosis of APGN. Light microscopy of APGN showed a certain spectrum of variation even during the rather limited period of 9 weeks, due to the varying number of granulocytes and a varying degree of cell proliferation, as we could show semiquantitatively. With the triad of methods, especially by IFM and EM, three separate morphological patterns were distinguishable: the starry sky pattern, the garland pattern and the mesangial pattern. Based on clinico-pathological correlations, these patterns were shown to permit the nosological subdivision of APGN. The following features merit special emphasis: The starry sky pattern occurred most often during the first few weeks, the mesangial pattern increased in frequency after the 3rd week, and the garland pattern could occur at any time. In the starry sky and garland patterns immunoglobulins (mainly IgG) generally appeared in combination with C 3. The mesangial pattern was characterized by C 3 appearing alone. These three immunohistological patterns, which also showed transitional and combined forms, had certain characteristic features by electron microscopy.(ABSTRACT TRUNCATED AT 400 WORDS)

Acute Disease↗

[Cardiac pathology in various in various pathogenetic subtypes of ischemic stroke].

Heart pathology was studied in various pathogenetic subtypes of ischemic stroke. A total of 330 stroke patients were divided into 4 groups. Group 1 consisted of 104 (31.5%) patients with cardioembolic stroke, group 2--of 72 (21.8%) with hemodynamic, group 3--of 71 (21.5%) patients with atherothrombotic and group 4--of 83 (25.2%) patients with lacunar stroke. Cardiac pathology was found in all patients of group 1 (ischemic heart disease-50.0%, inflammatory-infectious lesions-27.9%, other lesions-22.1%), in 49 (68.1%) patients of group 2, 36 (50.7%) of group 3 and 31 (37.3%) of group 4. Ischemic heart disease was prevalent (85.7, 86.1, 83.9% vs group 1). Potential sources of cardiogenic cerebral embolia were found in patients of all the groups: 100, 62.5, 43.7 and 33.7%, respectively. Thus, most patients with ischemic stroke have different heart defects related to the subtype of ischemic stroke. Potential causes of cardiocerebral embolism can be detected in any subtype of ischemic stroke.

Adolescent↗

Measurable differences between sequential and parallel diagnostic decision processes for determining stroke subtype: a representation of interacting pathologies.

Stroke diagnosis depends on causal subtype. The accepted classification procedure is a succession of diagnostic tests administered in an order based on prior reported frequencies of the subtypes. The first positive test result completely determines diagnosis. An alternative approach tests multiple concomitant diagnostic hypotheses in parallel. This method permits multiple simultaneous pathologies in the patient. These two diagnostic procedures can be compared by novel numeric criteria presented here. Thrombosis, a type of ischemic stroke, results from interaction between endothelium, blood flow and blood components. We tested for ischemic stroke on thirty patients using both methods. For each patient the procedure produced an assessment of severity as an ordered set of three numbers in the interval [0, 1]. We measured the difference in diagnosis between the sequential and parallel diagnostic algorithms. The computations reveal systematic differences: The sequential procedure tends to under-diagnose and excludes any measure of interaction between pathologic elements.

Algorithms↗

Responsiveness to prostaglandin E1 of different subtypes of normal and pathological committed granulomonopoietic precursors.

The influence of prostaglandin E1 (PGE1) on colonies produced by different subtypes of committed granulomacrophagic precursors (CFU-GM) was studied. Even though they represent a more immature class of precursors than day 7 CFU-GM, day 14 CFU-GM have the same sensitivity to PGE1: their growth is significantly inhibited by low PGE1 concentrations (10(-10) M or higher). Macrophagic colony formation is most sensitive to PGE1. Pure granulocytic colonies are relatively resistant and significantly inhibited by high concentrations only. Mixed granulomacrophagic colonies display an intermediate degree of sensitivity. Both day 7 and day 14 CFU-GM from chronic myeloid leukemia and idiopathic myelofibrosis patients are significantly less sensitive than normal CFU-GM. This insensitivity is not dependent on a lack of production of macrophagic colonies, since these may even be present in a higher than normal frequency.

Alprostadil↗

Evidence that severe asthma can be divided pathologically into two inflammatory subtypes with distinct physiologic and clinical characteristics.

The mechanisms associated with the development of severe, corticosteroid (CS)-dependent asthma are poorly understood, but likely heterogenous. It was hypothesized that severe asthma could be divided pathologically into two inflammatory groups based on the presence or absence of eosinophils, and that the inflammatory subtype would be associated with distinct structural, physiologic, and clinical characteristics. Thirty-four severe, refractory CS-dependent asthmatics were evaluated with endobronchial biopsy, pulmonary function, allergy testing, and clinical history. Milder asthmatic and normal control subjects were also evaluated. Tissue cell types and subbasement membrane (SBM) thickness were evaluated immunohistochemically. Fourteen severe asthmatics [eosinophil (-)] had nearly absent eosinophils (< 2 SD from the normal mean). The remaining 20 severe asthmatics were categorized as eosinophil (+). Eosinophil (+) severe asthmatics had associated increases (p < 0.05) in lymphocytes (CD3+, CD4+, CD8+), mast cells, and macrophages. Neutrophils were increased in severe asthmatics and not different between the groups. The SBM was significantly thicker in eosinophil (+) severe asthmatics than eosinophil (-) severe asthmatics and correlated with eosinophil numbers (r = 0.50). Despite the absence of eosinophils and the thinner SBM, the FEV(1) was marginally lower in eosinophil (-) asthmatics (p = 0.05) with no difference in bronchodilator response. The eosinophil (+) group (with a thicker SBM) had more intubations than the eosinophil (-) group (p = 0.0004). Interestingly, this group also had a decreased FVC/slow vital capacity (SVC). These results suggest that two distinct pathologic, physiologic, and clinical subtypes of severe asthma exist, with implications for further research and treatment.

Adult↗

Survival analysis of pseudomyxoma peritonei patients treated by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.

OBJECTIVE: To evaluate the survival of patients with pseudomyxoma peritonei (PMP) treated by cytoreductive surgery and intraoperative hyperthermic intraperitoneal chemotherapy (HIPEC), and to identify factors with prognostic value. SUMMARY BACKGROUND DATA: PMP is a clinical syndrome characterized by progressive intraperitoneal accumulation of mucous and mucinous implants, usually derived from a ruptured mucinous neoplasm of the appendix. Survival is dominated by pathology. METHODS: A total of 103 patients (34 men and 69 women) treated at The Netherlands Cancer Institute between 1996 and 2004 were identified. Survival was calculated from date of initial treatment and corrected for a second procedure. PMP was pathologically categorized into disseminated peritoneal adenomucinosis (DPAM), peritoneal mucinous carcinomatosis (PMCA), and an intermediate subtype (PMCA-I). Clinical and pathologic factors were analyzed to identify their prognostic value for survival. RESULTS: Median follow-up was 51.5 months (range, 0.1-99.5 months). Recurrence developed in 44%. A second procedure for recurrence was performed in 11 patients. The median disease-free interval was 25.6 months (95% confidence interval [CI], 14.8-43.6 months). The 3-year and 5-year disease-free survival probability was 43.6% (95% CI, 34.4%-55.2%) and 37.4% (95% CI, 28.2%-49.5%), respectively. The disease-specific 3-year and 5-year survival probability was 70.9% (95% CI, 62.0%-81.2%) and 59.5% (95% CI 48.7%-72.5%), respectively. Factors associated with survival were pathological subtype, completeness of cytoreduction, and degree and location of tumor load (P < 0.05). The main prognostic factor, independently associated with survival, was the pathologic subtype (P < 0.01). CONCLUSION: Cytoreductive surgery in combination with intraoperative HIPEC is a feasible treatment strategy for PMP in terms of survival. The pathologic subtype remains the dominant factor in survival. Patients should be centralized to improve survival by a combination of surgical experience and adequate patient selection.

Adult↗

Different T-cell subtypes are associated with pathologically distinct forms of Moloney leukemia virus (M-MuLV)-induced lymphoma.

The T-cell enzyme markers, terminal deoxy-nucleotidyltransferase (TDT) and 20 alpha hydroxysteroid dehydrogenase (20 alpha SDH), were used to classify lymphomas induced by the Moloney leukemia virus (M-MuLV). Different subtypes of T cells were shown to be involved in different types of lymphoma. Thymomas were TdT-positive and grew as subcutaneous solid tumors at the site of inoculation. Spleen cells from mice with generalized lymphoma were of two types. In the majority of cases the lymphomas consisted of 20 alpha SDH-positive cells that homed to spleen and lymph nodes upon transplantation. In a few cases the cells of enlarged spleens were TdT-positive and, like the TdT-positive thymomas, could be transplanted as subcutaneous tumors. Thus, TdT-positive and 20 alpha SDH-positive T-cell lymphomas can be distinguished by their homing properties. Preleukemic thymus cells from M-MuLV inoculated mice can, after transfer to 400 -R irradiated syngeneic hosts, induce new lymphomas by virus release or grow in an autonomous fashion in the recipients. Whether of donor or recipient type, these lymphomas are TdT-positive. In contrast, preleukemic bone marrow cells give lymphomas of donor type which are as heterogeneous for T-cell enzymes as are lymphomas induced by neonatal inoculation of M-MuLV.

20-Hydroxysteroid Dehydrogenases↗

[The role of gamma-delta T-lymphocyte subtypes in normal and pathologic conditions].

In the review data about origin, spreading in the organism, differentiation, physiologic sense, role in diversity of pathologic processes, and also pharmaco- and immunocorrection's probable foundations of T-lymphocyte's new population with T cell receptor (TcR) gamma delta (TL gamma delta) are resumed. In the phylogenesis TL gamma delta are supposed to be older than T lymphocytes with TcR alpha beta. The conclusion is based upon domination of this lymphocyte population at gestation's early stage and huge representation of pseudogenes in the DNA region that encodes TcR structure of TL gamma delta. A morphological and functional resemblance of the given population with natural killer cells is underlined. It is paid attention to wide representation of TL gamma delta in the periphery tissues, poverty of TcR diapason that may evidence about postdifferentiation's process of TL gamma delta to be accomplished due to TcR genes rearrangement. This process appears to supply the enhancement of antigen-specific TL gamma delta in foreign agent's inculcation. A maturation process and probable mechanisms of these lymphocytes education in thymus is described. A classification of TL gamma delta depending on TcR structure and cytokine profile of the lymphocytes that subdivides them on T-helper/cytotoxic (Vg9/Vd2 phenotype) and cytotoxic/suppressor (Vd1 or Vd3 phenotype) was proposed. In the review the role of TL gamma delta in mucous immunity supporting and possible participating in systemic regulation of the immune response is emphasized. A physiologic role of TL gamma delta, namely ability to identify non-protein antigens (lipopolysaccharides, polyphosphates, glycolipids) and heat shock proteins, is also described. Evidences about uncertainty of incomplete phagocytosis phenomenon in vivo in case of TL gamma delta's normal function are given. Particularly it is made out that these lymphocytes are able to activate inducible NO-synthase in the macrophages that enhances their phagocytic activity in tens time. A function of these lymphocytes in defense against infection of bacterial, viral, protozoa, fungal origin, and also in tumor growth and autoimmune diseases is represented. Methods of specific therapeutic influence upon TL gamma delta, both pharmacological (pamidronat, 2,3-diphosphoglyceric acid) and immunologic profile (monoclonal antibodies conjugated with cytotoxic agent) are given.

Animals↗