Genotype-phenotype associations within the Li-Fraumeni spectrum: a report from the German Registry.

Li-Fraumeni syndrome (LFS) is a cancer predisposition syndrome caused by pathogenic TP53 variants. The condition represents one of the most relevant genetic causes of cancer in children and adults due to its frequency and high cancer risk. The term Li-Fraumeni spectrum reflects the evolving phenotypic variability of the condition.

Prognostic impact of nutritional and inflammation-based risk scores in follicular lymphoma in the era of anti-CD20 targeted treatment strategies.

Background: The composition of the tumor microenvironment (TME) is conditioned by immunity and the inflammatory response. Nutritional and inflammation-based risk scores have emerged as relevant predictors of survival outcome across a variety of hematological malignancies.

Methods: In this retrospective multicenter trial, we ascertained the prognostic impact of established nutritional and inflammation-based risk scores [Glasgow Prognostic Score (GPS), C-reactive-protein/albumin ratio (CAR), neutrophil-lymphocyte ratio (NLR), prognostic nutritional index (PNI), and prognostic index (PI)] in 209 eligible patients with histologically confirmed CD20 follicular lymphoma (FL) of WHO grade 1 (37.

The Glasgow Prognostic Score at Diagnosis Is a Predictor of Clinical Outcome in Patients with Multiple Myeloma Undergoing Autologous Haematopoietic Stem Cell Transplantation.

Immunity and inflammatory response affect the tumour microenvironment and the progression of malignancies. Metabolic and inflammatory parameters and ratios of the peripheral blood correlate with outcome in cancer patients. There exist several established and validated inflammation-based scores of prognostic significances including the Glasgow Prognostic Score (GPS).

Trofosfamide in the treatment of elderly or frail patients with diffuse large B-cell lymphoma.

Purpose: The introduction of immunochemotherapy has led to a significant improvement in treatment results and prognosis of diffuse large B-cell non-Hodgkins lymphoma (DLBCL) both at initial diagnosis and in relapse. Trofosfamide, an oxazaphosphorine derivative, has been utilized as alternative treatment option for patients with lymphoproliferative diseases unsuitable for conventional chemotherapy agents and protocols because of age, comorbidity, or poor performance score. While data on the activity and safety of single-agent trofosfamide have been published, the potential value of this agent in immunochemotherapy in combination with anti-CD20 antibodies such as rituximab has not been investigated to our knowledge.

Fusions in Wild-Type Pancreatic Cancer.

We used whole-genome and transcriptome sequencing to identify clinically actionable genomic alterations in young adults with pancreatic ductal adenocarcinoma (PDAC). Molecular characterization of 17 patients with PDAC enrolled in a precision oncology program revealed gene fusions amenable to pharmacologic inhibition by small-molecule tyrosine kinase inhibitors in all patients with wild-type () tumors (4 of 17). These alterations included recurrent rearrangements predicted to drive PDAC development through aberrant ERBB receptor-mediated signaling, and pharmacologic ERBB inhibition resulted in clinical improvement and remission of liver metastases in 2 patients with -rearranged tumors that had proved resistant to standard treatment.

[Anaemia in the elderly].

In the elderly, even mild anaemia leads to significantly decreased quality of life and reduced survival rate. Therefore even mild anaemias should be worked up especially in the elderly. More than 75 % of all anaemias have a specific and treatable cause.

Endovascular recanalization of a port catheter-associated superior vena cava syndrome.

Purpose: Superior vena cava (SVC) syndrome owing to benign etiology is rare and endovascular techniques have been advocated as the treatment of choice. We report a case of endovascular revascularization of a port catheter-associated complete occlusion of the SVC with reversed flow in the azygos vein.

Methods: In this setting using a sheath in combination with its dilatator to pass the occlusion of the SVC after neither a diagnostic catheter nor a PTA balloon would pass the lesion may be a valid option.

Poly (ADP-ribose) polymerase inhibitor efficacy in head and neck cancer.

Objectives: Poly (ADP-ribose) polymerase inhibitors (PARPi) have shown single agent activity against tumors with deficiencies in the DNA repair mechanism homologous recombination including, but not limited to those harboring BRCA mutations. We hypothesized that, in the context of homologous recombination deficiency (HRD), PARPi could have an effect in head and neck cancer (HNC).

Materials And Methods: We evaluated TCGA data for evidence of HRD using a copy number data signature established for breast cancer.

Treatment of indolent lymphoma in Germany - results of a representative population-based survey.

Background: In advanced-stage indolent lymphoma, therapeutic approaches may vary from watch and wait, antibody monotherapy, immunochemotherapy, or dose-intensified consolidation up to allogeneic strategies. In this nationwide survey, representative hematologic/oncologic centers monitored current treatment strategies in indolent lymphoma in general practice.

Methods: Four hundred ninety-five centers involved in the treatment of indolent lymphoma including university hospitals, community hospitals, and oncologists in practice were identified and contacted.

Use of magnetic resonance imaging to detect neoplastic meningitis: limited use in leukemia and lymphoma but convincing results in solid tumors.

Background: An early diagnosis of meningitis is important to improve patients' survival. Data about a direct comparison of cerebrospinal fluid cytology (CSF-cytology) and MRI are very limited. Therefore, the aim of this study was to compare these two diagnostic modalities in diagnosing meningitis in patients with hematopoietic and solid malignancies.

Population-based patterns of care in the first-line treatment of patients with advanced esophagogastric adenocarcinoma in Germany.

Background: Randomized studies proved the efficacy of new drugs for the systemic treatment of advanced gastric cancer in the past 5 years. However, little is known about the use of firstline chemotherapy in clinical practice in patients with advanced or metastatic adenocarcinoma of the esophagogastric junction (AEG) and the stomach. We investigated temporal trends in therapy and factors influencing treatment decisions for these patients during a 4-year period.

Five-year, disease-free survival after repeat palliative multimodality therapy in a patient with recurrent metastatic bladder cancer.

In appropriately selected cases, palliative therapeutic strategies can be adapted to those special features of cancer biographies that indicate an atypical course of disease. Elucidating these features, and adapting multimodal treatment strategies to them, can lead to significantly superior effects when compared to the routine application of conventional treatment algorithms. A case of regionally metastatic bladder cancer is presented that documents the value of repeat debulking-surgery and repeat radiotherapy leading to unexpected short-term and long-term treatment results.

Primary B cell lymphoma of the sphenoid sinus: CT and MRI characteristics with correlation to perfusion and spectroscopic imaging features.

Primary non-Hodgkin lymphoma (NHL) of the paranasal sinuses is a rare neoplasm that cannot be easily diagnosed and differentiated as its clinical, histological, and imaging features are similar to those of other inflammatory and tumorous diseases in their early stages. We evaluated the morphological and functional imaging characteristics of primary NHL of the sphenoid sinus using CT and MR imaging. Morphological CT and MR imaging as well as perfusion CT imaging and proton MR spectroscopy (PRESS technique, TE = 135) was performed in three patients with the histological diagnosis of highly malignant primary B cell lymphoma of the sphenoid sinus.

[Vibrio vulnificus sepsis].

A 59-year-old female patient with a history of malignant lymphoma presented with symptoms of septicaemia. The skin of the extremities showed bullous, necrotizing plaques. Blood culture revealed Vibrio vulnificus as the causative organism.

Long-term disease-free survival in patients with angioimmunoblastic T-cell lymphoma after high-dose chemotherapy and autologous stem cell transplantation.

Background And Objectives: Patients with angioimmunoblastic T-cell lymphoma (AIL) have a poor prognosis with conventional treatment.

Design And Methods: We initiated an EBMT-based survey studying the impact of high-dose chemotherapy (HDCT) and autologous hematopoietic stem cell transplantation in patients with AIL. Data on 29 patients, who were transplanted between 1992 and 1998 in 16 transplant centers, were collected on standardized documentation forms.

The role of high-dose chemotherapy in the treatment of non-small cell lung cancer.

The literature on high-dose chemotherapy in non-small cell lung cancer (NSCLC) with autologous bone marrow or peripheral blood stem cell transplantation does not - as of yet - provide evidence of relevant benefits. At the same time, the significant risks of treatment-related morbidity and mortality associated with dose-intensified chemotherapy in this vulnerable patient population are increasingly recognized. Whether the advent of new cytotoxic agents such as the Taxans or newer Topoisomerase inhibitors will help to improve the hitherto unsatisfying results of high-dose chemotherapy in NSCLC, remains to be determined.