Multilobated mantle cell lymphoma.
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Aged↗
Biomedical subjects
C Thorns
Publications and source records attributed to C Thorns.
[Mycosis fungoides in childhood and adolescence with clonal T-cell receptor gamma gene rearrangement. Two cases].
Mycosis fungoides (MF) is a cutaneous T-cell lymphoma (CTCL) characterized by its typical progress in three stages: the patch-, the plaque- and the tumour-stage. The incidence of mycosis fungoides rises with age and the average age at presentation is about 50. Children and adolescents are rarely affected and there are only few reports in the literature. We report a 12- and a 15-year-old boy showing refractory skin lesions not typical for mycosis fungoides. The histo- and immunohistological investigations and the detection of clonal T-cell receptor gamma gene rearrangements confirmed the diagnosis of early onset mycosis fungoides in both cases.
Adolescent↗
VEGF-, KIT protein- and neutral endopeptidase (NEP/CD10)-positive myofibroblasts-precursors of angiogenesis in chorioangiomas?
Chorioangiomas are benign angiomatous tumours of the placenta occurring with a frequency of approximately one per cent of all examined placentae. Hypoxia and genetic factors are discussed to be predisposing factors for chorioangiomas. However, not much is known about the tumorigenesis of these benign tumours. Screening with various antibodies in a rare case of chorangiomatosis, we found disseminated spindle cells coexpressing vascular epithelial growth factor (VEGF), neutral endopeptidase 24.11 (NEP/CD10), and KIT protein (CD117) within the tumour stroma. A possible involvement of such factors in angiogenesis and tumorigenesis of chorioangiomas/chorangiomatosis has not been studied so far.Seven placentae with chorioangiomas (n=6) or chorangiomatosis (n=1), six normal placentae, and four cutaneous haemangiomas were analysed immunohistochemically (ABC and APAAP methods) using antibodies against VEGF, NEP, KIT protein, as well as endothelial markers like PECAM-1 (CD31), CD34, v. Willebrand factor (factor VIII), and ulex europaeus. In addition, analysis of c-kit 'gain of function' mutation Asp 816 to Val by means of Hinfl digestion and direct sequencing of semi-nested polymerase chain reaction products was performed. All chorioangiomas and haemangiomas strongly expressed the endothelial markers CD34, CD31, and FVIII, while only weak expression of ulex lectin was noted. Disseminated groups of VEGF-, NEP-, and KIT protein-positive spindle cells, which coexpressed vimentin and smooth-muscle actin were identified as myofibroblasts in the stroma of four chorioangiomas. These spindle cells were quantified as numerous in two and as rare in two other cases. No VEGF-positive myofibroblasts, however, were detected in the villous stroma of normal control placentae and haemangiomas. Only scattered perivascular myofibroblasts expressing KIT protein and NEP were detected in early gestational placenta controls. In all chorioangiomas and chorangiomatosis PCR analysis failed to unveil c-kit 'gain of function' mutation Asp 816 to Val in KIT protein-positive spindle cells. Moreover, a significant increase in mast cells was observed only in the haemangiomas. As expected, endothelial origin of chorioangiomas/chorangiomatosis was verified by CD31, CD34, FVIII expression. Myofibroblastic spindle cells expressing VEGF and NEP may be precursor cells in these peculiar angiomatous tumours. Although activating c-kit mutation Asp 816 to Val was not detected by PCR, the presence of KIT protein (CD117)-positive intratumoral myofibroblastic spindle cells in chorioangiomas and chorangiomatosis might suggest involvement of the stem cell factor (SCF)-receptor in pathologically enhanced angiogenesis.
Adult↗
Prognostic significance of a grading system considering tumor heterogeneity in muscle-invasive urothelial carcinoma of the urinary bladder.
The fact that urothelial carcinomas (UC) often contain areas with different histologic grades has been recently shown to bear some prognostic relevance. Here we examined the prognostic significance of a grading system considering tumor heterogeneity in muscle-invasive bladder carcinomas. 151 UC treated by radical cystectomy were included. According to the World Health Organization/International Society of Urological Pathology (WHO/ISUP) classification, histologic grade was low-grade (LG) in 8 and high-grade (HG) in 143 cases. 65 HG tumors which focally harbored LG areas were assigned to mixed-type (MT) carcinomas. Mean follow-up was 50 months. While the WHO/ISUP classification showed no significant correlation with disease-specific survival (p = 0.3995 by log-rank test), stratification into LG/MT and HG tumors had a significant prognostic relevance (p = 0.0404). Nodal status was identified as the only independent prognostic factor (p = 0.0001 by multivariate analysis). In this respect, stratification into LG/MT and HG tumors missed the level of statistical significance by a norrow margin (p = 0.07 by multivariate analysis), but it turned out better than tumor category (p = 0.08). In conclusion, a grading system considering tumor heterogeneity may improve the predictive power of the WHO/ISUP classification in muscle-invasive UC of the urinary bladder. Although the two-tired grading system proposed in this study was not identified as an independent prognostic factor, it may help to obtain additional prognostic information on patients with advanced bladder cancer treated by radical cystectomy.
Adult↗
Primary cutaneous T-cell lymphoma of the penis.
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Biomarkers, Tumor↗
Chronic cholecystitis and myeloid metaplasia.
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Aged↗
[Anatomy of the shoulder joint].
The shoulder joint and its associated joints form one of the most complex joint systems of the human locomotor apparatus. Its large range of motion is made possible by the interplay of 5 joints: sternoclavicular-joint, acromioclavicular-joint, glenohumeral joint, thoracoscapular joint and subacromial joint. The rotator cuff works mostly as an active stabilizer of the shoulder joint. The supraspinatus muscle causes a compression of the humerus in the glenoid mainly, furthermore it effects synergistic the abduction with the delta muscle. On the basis of its lever-arm the supraspinatus works between 0 and 60 degrees abduction the most optimally. With failure of the supraspinatus, the deltoideus can almost completely take its function. The inferior glenohumeral ligament-complex is the main passive stabilizer. The blood supply of the humerus head is ensured mainly by the a. circumflexa anterior and its rami ascendents, by several small branches from the a. circumflexa posterior and over intraosseous anastomoses. The most important vessel of the cap is the intraosseous a. arcuata out of the ramus ascendens lateralis of the a. circumflexa anterior.
Acromioclavicular Joint↗
Anti-CD20 monoclonal antibody (Rituximab) and Cidofovir as successful treatment of an EBV-associated lymphoma with CNS involvement.
BACKGROUND: Epstein-Barr virus(EBV)-associated posttransplant lymphoproliferative disease (PTLD) is a serious complication after allogeneic hematopoietic stem cell transplantation (HSCT). Especially in cases with involvement of the central nervous system (CNS) treatment is difficult because the efficacy of most chemotherapeutic agents as well as EBV-specific cytotoxic donor T cells in liquor is uncertain. In the last years the anti-CD20 monoclonal antibody Rituximab was intensively investigated in the treatment of EBV-PTLD. However, only 8 patients with B-cell lymphoma and CNS involvement treated with Rituximab were reported. CASE REPORT: A 24-year-old female patient with acute T-lymphoblastic leukemia in second complete remission had received allogeneic, unrelated, T-cell depleted HSCT. 10 months later an EBV-associated PTLD was diagnosed. Beside peripheral lymphomas and B symptoms the patient showed neurological symptoms. Examination of the cerebrospinal fluid (CSF) revealed a meningeosis lymphoblastica caused by the EBV lymphoma. Treatment with Rituximab and the antiviral drug Cidofovir led to complete remission with regression of the peripheral lymphomas and disappearance of the neurological symptoms. In addition, the PCR control on EBV DNA became negative in the plasma as well as in CSF. CONCLUSION: The combination of Rituximab and Cidofovir appears as an interesting alternative treatment in patients with EBV-associated PTLD and CNS involvement.
Adult↗
Maternally-inherited diabetes and deafness: report of two affected German families with the A3243G mitochondrial DNA mutation.
In two German families four patients containing the A3243G mutant in the mitochondrial DNA suffered from maternally-inherited diabetes and deafness (MIDD). DNA was isolated from oral mucosa cells. Using the polymerase chain reaction with not yet published primers, we obtained after digestion with the restriction endonuclease BSP 1201 two oligonucleotides of comparable size increasing the sensitivity of our method two times. Under these conditions we were able to detect 0.8% of the mutated DNA. In general, the patients show the characteristics proposed for MIDD by Maassen et al. (1997), however, there are some differences: age at onset (in our study 36-45 y), body mass index (>26 kg/m2). In addition, our study shows that MIDD may be combined with neuronal disorders (M. Parkinson, epilepsy) and/or endocrinopathies (M. Addison). Our data indicate that MIDD is a heterogeneous disease. On the other hand, in one family there are healthy probands with a high concentration of mutated mitochondrial DNA.
Adolescent↗
Adhesion in vitro and in vivo associated with an adhesive antigen (F41) produced by a K99 mutant of the reference strain Escherichia coli B41.
A K99-negative mutant of the K99 reference strain Escherichia coli B41 (O101:K99) was isolated (strain B41M). It did not react with OK antiserum to a K12 K99+ recombinant or with OK antiserum to K99-positive organisms from the O8, O20, or O64 serogroups, but it did react with OK antiserum to K99-positive organisms from the O101 and O9 serogroups. The mutant hemagglutinated sheep erythrocytes and attached in vitro to calf enterocytes when cultured at 37 degrees C but not when grown at 18 degrees C. Attachment was mannose resistant but susceptible to heating and formaldehyde. These properties were associated with the presence of fimbriae. The isolated hemagglutinin migrated to the anode in immunoelectrophoresis experiments, competitively inhibited attachment of strain B41M to calf enterocytes, and could be demonstrated adhering to these cells in vitro by indirect immunofluorescent staining. The anionic hemagglutinin is referred to provisionally as F41. Germfree piglets infected with strain B41M developed diarrhea within 16 h. Scanning electron microscopy showed groups of bacteria adherent to the microvilli of villous enterocytes. Indirect immunofluorescent staining demonstrated the presence of F41 antigen in vivo.
Animals↗
Matrix-metalloproteinases in Hodgkin lymphoma.
BACKGROUND: Classical Hodgkin lymphomas are characterized by relatively few tumour cells and prominent proliferation of plasma cells, histiocytes, lymphocytes and eosinophils. In addition there is a varying degree of sclerosis, which is especially prominent in nodular sclerosis. These morphological peculiarities led to the idea that the interaction between tumour cells and bystander cells as well as the extracellular matrix may be important in Hodgkin lymphomas. MATERIALS AND METHODS: Thirty-four classical Hodgkin lymphomas (CHL) were analysed regarding the expression of EMMPRIN, MMP-2, -7, -9, -10 and-11 using immunohistochemistry. RESULTS: The tumour cells were positive for EMMPRIN in 100% of the cases. In 82% of CHL the Hodgkin and Reed-Sternberg cells (HRS) were negative for MMP-2. In contrast the surrounding non-neoplastic cells were MMP-2-positive in 71% of the cases. The HRS cells stained positive for MMP-7 in 68% of CHL, whereas only a few surrounding cells were positive for this marker. In all but one case (97%) the HRS cells were negative for MMP-9. However, the surrounding cells stained positive in 32%, thus resembling the staining pattern for MMP-2. Only scattered cells of both populations, HRS cells as well as bystander cells, stained for MMP-10 and -11, and no specific staining pattern was observed. CONCLUSION: Our data indicate a complex interaction between tumour cells and bystander cells with regard to metalloproteinases. The expression of EMMPRIN in the tumour cells may induce the expression of MMP-2 in the surrounding non-neoplastic cells. MMP-2 can be activated by MMP-7, which is expressed in the tumour cells. It is tempting to speculate that an interruption of this cycle could be of therapeutic benefit.
Antigens, CD↗
Expression of MMP-2, MMP-7, MMP-9, MMP-10 and MMP-11 in human astrocytic and oligodendroglial gliomas.
The members of the matrix metalloproteinase family (MMP) have the ability to degrade macromolecules of the extracellular matrix and are responsible for tumor invasion and infiltration, limiting the effectiveness of the neurosurgical resection of brain tumors. Among the glial brain tumors, astrocytomas and oligodendrogliomas are the most important tumor entities and require a different therapeutic approach. To determine the pattern of MMP expression in astrocytic and oligodendroglial tumors, sections of astrocytic and oligodendroglial differentiated glioblastomas (WHO grade IV), as well as of anaplastic oligodendrogliomas (WHO grade III) and anaplastic gemistocytic astrocytomas (WHO grade III) were immunostained for MMP-2, MMP-7, MMP-9, MMP-10 and MMP-11. MMP-7, MMP-10 and MMP-11 were strongly expressed by neoplastic gemistocytic astrocytes while oligodendrocytic tumor regions showed only a low immunoreaction. In contrast, MMP-2 and MMP-9 mainly immunolabeled vascular structures. These data indicated that MMP-7, MMP-10 and MMP-11 contribute to the worse prognosis of astrocytic tumors when compared to oligodendrogliomas, while MMP-2 and MMP-9 might play an important role in neo-angiogenesis and tumor vascularization.
Astrocytoma↗