Interdisziplin?re Leitlinien zur Diagnostik und Therapie der extrazerebralen Amyloidosen

Ohne Zusammenfassung     

Esthesioneuroblastoma in a young child. Case report

Esthesioneuroblastoma is an uncommon malignant neoplasm that arises from olfactory mucosa, often with intracranial extension.A case report of the youngest victim in the literature (2-year-old boy) is presented.     

A large primary malignant melanoma of the female urethra

We present a case of large, primary malignant melanoma of the female urethra with poor prognosis in this case report. Malignant melanoma is one of the rare tumors of the female urethra and accounts for 0.2% of all malignant melanoma cases. Large (5.6 cm in diameter), primary malignant melanoma of the female urethra is exceedingly rare.     

Expression of receptor activator of nuclear factor kappaB ligand on bone marrow plasma cells correlates with osteolytic bone disease in patients with multiple myeloma.

PURPOSE: Increased bone resorption is a hallmark of multiple myeloma and is attributable to osteoclast activation. Recent studies showed that the receptor activator of nuclear factor kappaB ligand (RANKL) is the key mediator of osteoclastogenesis and plays a crucial role in bone destruction in malignant bone disease. We found that human myeloma cells express RANKL and analyzed the association of the RANKL expression with the presence of osteolytic bone disease in patients with multiple myeloma. EXPERIMENTAL DESIGN: Flow cytometry was performed on bone marrow samples derived from controls and multiple myeloma patients with or without osteolytic bone lesions on conventional radiography. Plasma cells were identified as CD38++/CD138+ cells. The level of RANKL expression on the surface of bone marrow plasma cells was correlated with the bone status of the patients. RESULTS: The bone marrow plasma cells from controls showed no or only a weak surface expression of RANKL, and the median mean fluorescence index (MFI) was 6. In contrast, expression of RANKL could be detected on bone marrow plasma cells from all of the patients with multiple myeloma, and median MFI was 47. The difference in MFI for RANKL expression of bone marrow plasma cells from controls and myeloma patients was highly significant (P < 0.0005). Myeloma patients with osteolytic bone lesions showed a significantly higher expression of RANKL (median MFI = 60; range, 16-2494) compared with patients without osteolysis (median MFI = 16; range, 6-229; P < 0.0005). CONCLUSIONS: These results show for the first time that the level of RANKL expression by myeloma cells correlates significantly with osteolytic bone disease.     

Clinical and psychometric validation of a questionnaire module, the EORTC QLQ-STO 22, to assess quality of life in patients with gastric cancer

The purpose of this study was to define the measurement properties and clinical validity of the European Organisation for Research and Treatment of Cancer (EORTC) questionnaire module to assess health-related quality of life (HRQL) in gastric cancer. The EORTC gastric cancer module, QLQ-STO 22, was administered with the QLQ-C30, core questionnaire, to 219 patients undergoing treatment with curative or palliative intent before and after treatment. Reliability and validity of the module was tested and patients' debriefing comments analysed. Compliance rates were high, questionnaires well accepted and less than 4% of items had missing data. Multi-trait scaling analyses and face validity refined the module to five scales and four single items. Scales distinguished between clinically distinct groups of patients and demonstrated treatment-induced changes over time. Test-retest scores demonstrated good reliability. The EORTC QLQ-STO 22 demonstrates psychometric and clinical validity that supports its use to supplement the EORTC QLQ-C30 to assess quality of life in patients with gastric cancer undergoing surgery, surgery and chemoradiotherapy, palliative chemotherapy, palliative surgery and best supportive care.     

Viral Agents as Potential Drivers of Diffuse Large B-Cell Lymphoma Tumorigenesis

Among numerous causative agents recognized as oncogenic drivers, 13% of total cancer cases occur as a result of viral infections. The intricacy and diversity of carcinogenic processes, however, raise significant concerns about the mechanistic function of viruses in cancer. All tumor-associated viruses have been shown to encode viral oncogenes with a potential for cell transformation and the development of malignancies, including diffuse large B-cell lymphoma (DLBCL). Given the difficulties in identifying single mechanistic explanations, it is necessary to combine ideas from systems biology and viral evolution to comprehend the processes driving viral cancer. The potential for more efficient and acceptable therapies lies in targeted medicines that aim at viral proteins or trigger immune responses to either avoid infection or eliminate infected or cancerous cells. In this review, we aim to describe the role of viral infections and their mechanistic approaches in DLBCL tumorigenesis. To the best of our knowledge, this is the first review summarizing the oncogenic potential of numerous viral agents in DLBCL development.     

Which is the dominant factor for perception of rheumatic pain: meteorology or psychology?

It is believed that there is an association between the weather and rheumatic symptoms. We aimed to investigate what kind of association is present and what are the factors which determine the nature of this association. Fifty-six subjects with rheumatic disease (31 RA, 15 SpA, 10 OA) who live in Antalya were followed between December 2005 and July 2006. Patients were asked to fill diaries which contain questions regarding the symptoms of their rheumatic diseases everyday. In every monthly visit, disease activity measurement, laboratory assessment and Beck depression inventory assessment were recorded. The symptomatic and psychological measurements were matched with the meteorological data of Antalya Regional Directorate of Meteorological Service of Turkish State. Correlation of symptoms with weather variables was investigated. Contributory effect of weather and of psychologic factors on symptom scores were evaluated by stepwise multiple regression analysis. Eighty-four percent of subjects belive in an association between weather and rheumatism, while 57% claimed to have ability to forecast weather. The maximum correlation coefficient between weather and arthritis symptoms was −0.451 and the maximum contribution of weather on symptoms was 17.1%. Arthritis symptoms were significantly contributed by Beck depression score. The belief about presence of weather–arthritis association was found to be stronger than its statistical power. Our results did not prove or rule out the presence of weather–rheumatism association. As long as the scientific attempts result in failure, the intuitive support in favour of the presence of weather–arthritis association will go on forever.