Upper tract urothelial cancers account for 5% of all urothelial cancers. Among these, more than 90% of tumours are located in the renal pelvis, while ureteric tumours are extremely rare. The distal ...ureter is the most common location, although multifocal implants may also occur. Megaureter is a common cause of obstructive uropathy in neonates and children. Nevertheless, it may be unnoticed, if asymptomatic. Megaureter may be obstructing or refluxing. Long-standing urinary stasis and recurrent urinary tract infection in megaureter may cause chronic irritation of the ureteric mucosa leading to dysplasia and malignancy. We report a 55-year-old man diagnosed with bilateral obstructive megaureter with right lower ureteric urothelial cancer and review the current literature.
Background
In indolent systemic mastocytosis (ISM), several risk factors of disease progression have been identified. Previous studies, performed with limited patient numbers, have also shown that ...the clinical course in ISM is stable and comparable to that of cutaneous mastocytosis (CM). The aim of this project was to compare the prognosis of patients with ISM with that of patients with CM.
Methods
We employed a dataset of 1993 patients from the registry of the European Competence Network on Mastocytosis (ECNM) to compare outcomes of ISM and CM.
Results
We found that overall survival (OS) is worse in ISM compared to CM. Moreover, in patients with typical ISM, bone marrow mastocytosis (BMM), and smoldering SM (SSM), 4.1% of disease progressions have been observed (4.9% of progressions in typical ISM group, 1.7% in BMM, and 9.4% in SSM). Progressions to advanced SM were observed in 2.9% of these patients. In contrast, six patients with CM (1.7%) converted to ISM and no definitive progression to advanced SM was found. No significant differences in OS and event‐free survival (EFS) were found when comparing ISM, BMM, and SSM. Higher risk of both progression and death was significantly associated with male gender, worse performance status, and organomegaly.
Conclusion
Our data confirm the clinical impact of the WHO classification that separates ISM from CM and from other SM variants.
Division of mastocytoses according to updated WHO Classification is clinically relevant. Overall survival is worse in indolent systemic mastocytosis compared to cutaneous mastocytosis. Trephine biopsy is necessary to distinguish indolent systemic mastocytosis from cutaneous mastocytosis.
Background Although completion axillary lymph node dissection (CALND) is the gold standard for evaluating axillary status after identification of a positive sentinel lymph node (SLN) in breast ...cancer, almost 40% to 70% of SLN-positive patients will have negative non-SLNs. To predict non-SLN metastases (NSLNM) in patients with a positive SLN biopsy, four different nomograms have been created. The aim of this study was to evaluate the accuracy of four different nomograms in our SLN-positive breast cancer patients. Study Design We identified 319 patients who had a positive SLN biopsy and CALND at our hospital during an 8-year period. Breast cancer nomograms developed by Memorial Sloan-Kettering Cancer Center, Tenon Hospital, Cambridge University, and Stanford University were used to calculate the probability of NSLNM. The area under the receiver operating characteristics curve was calculated for each nomogram, and values greater than 0.70 were accepted as demonstrating considerable discrimination. Results One hundred seven of 319 patients (33.5%) had positive axillary NSLNM. The mean number of SLNs was 2.01 (range, 1 to 11 nodes), and the mean number of positive SLNs was 1.44 (range, 1 to 9 nodes). The area under the curve values were 0.70, 0.69, 0.69, and 0.64 for the Memorial Sloan-Kettering Cancer Center, Tenon, Cambridge, and Stanford models, respectively. Conclusions We found that the Memorial Sloan-Kettering Cancer Center nomogram was more predictive than the other nomograms, but the Cambridge model and the Tenon model reached borderline values for accurate prediction. Nomograms developed at other institutions should be used with caution when counseling patients about the risk of additional nodal disease.
A small renal mass is defined as a tumour <4cm. The standard treatment of choice for small renal masses is partial or radical nephrectomy, depending on the tumour anatomy, and has good overall and ...cancer-specific survival. Its association with lymph node metastasis and inferior vena cava (IVC) thrombus is very uncommon. We describe a case of a right small renal mass with a large metastatic paracaval lymph node with IVC level I thrombus who was treated with right radical nephrectomy with thrombus removal and lymph node excision.
Renal cell cancer (RCC) accounts for 3% of all solid malignancies. Synchronous tumour thrombus in the renal vein or inferior vena cava is reported in 4-10% cases and is a surgical challenge. ...Contemporary imaging modalities that are used to define the presence and extent of venous thrombus include colour Doppler, contrast-enhanced computed tomography and magnetic resonance venography. Surgical management depends upon the degree of tumour thrombus. We report isolated recurrence of RCC in the inferior vena cava 2 years after radical nephrectomy, and discuss its pathophysiology and management.
Mutational analysis of the c-kit gene in a patient with a previously undescribed variant of mast cell disease revealed a germline mutation, Phe522Cys, within the transmembrane portion of the Kit ...receptor protein. Transfection experiments revealed that the mutation caused ligand-independent autophosphorylation of Kit, which was inhibited by the tyrosine kinase inhibitor imatinib mesylate. The patient's bone marrow biopsy and aspirate displayed unique pathologic features with the presence of excessive numbers of mature-appearing mast cells and absence of aberrant mast cell surface expression of CD2, CD25, and CD35. Therapy with imatinib mesylate resulted in a dramatic improvement in mast cell burden and clinical symptoms. These results highlight the significance of the transmembrane region of Kit in activation of the molecule and its importance in mast cell development and suggest a role for screening for transmembrane c-kit mutations in patients with mastocytosis in association with the decision to use imatinib mesylate. (Blood. 2004;103:3222-3225)
Objectives: This report presents final results (24 months of follow-up) from the first prospective, national study of frontline nilotinib in chronic myeloid leukemia (CML) patients in Turkey.
...Methods: Patients with newly diagnosed Philadelphia chromosome-positive CML in chronic phase (CML-CP; N = 112) received nilotinib 300 mg twice daily. The primary endpoint, which was the cumulative rate of major molecular response (MMR; BCR-ABL1 ≤ 0.1% on the International Scale BCR-ABL1
IS
) by 12 months, was previously reported (66.1% 80% CI, 59.7%-72.0%). ClinicalTrials.gov identifier NCT01274351
Results: By 24 months, 83.0% of patients achieved MMR, and 50.9% achieved MR
4.5
(BCR-ABL1
IS
≤0.0032%). Safety results at 24 months were consistent with those at 12 months. No additional deaths or disease progressions to accelerated phase/blast crisis were observed between 12 and 24 months.
Discussion: Treatment with nilotinib 300 mg twice daily for 2 years provided high MMR with a good safety/tolerability profile in newly diagnosed CML-CP patients in Turkey. Assessment of MMR across time points showed increasing rates through 18 months, after which as lower rate of increase was observed. The safety profile of nilotinib 300 mg twice daily with 24 months of follow-up was similar to that observed at 12 months, and no new safety concerns were identified. These efficacy and safety findings are consistent with the results from the 12-month analysis of this study and from previous nilotinib studies. These findings support nilotinib as an option for frontline treatment of CML-CP.
Conclusion: Frontline nilotinib treatment provided sustained efficacy, with good tolerability, over 24 months in newly diagnosed CML-CP patients.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
Objective: Nilotinib is a BCR-ABL1 tyrosine kinase inhibitor approved for the treatment of patients with chronic myeloid leukemia in chronic phase (CML-CP). This study was the first prospective ...evaluation of the efficacy and safety of nilotinib in Turkish patients with newly diagnosed CML-CP. The primary endpoint of the study was the rate of major molecular response (MMR; BCR-ABL1 ≤ 0.1% on the International Scale BCR-ABL1
IS
) by 12 months.
Methods: Patients with newly diagnosed CML-CP were treated with nilotinib 300 mg twice daily. This analysis was based on the first 12 months of follow-up in a 24-month study.
Results and Conclusions: Of 112 patients enrolled, 66.1% (80% CI, 59.7-72.0%) achieved MMR and 22.3% achieved a deep molecular response of MR
4.5
(BCR-ABL1
IS
≤ 0.0032%) by 12 months. During the first year of treatment, 1 patient progressed to blast crisis and 2 patients died. Safety results were consistent with previous studies. Most adverse events (AEs) were grade 1/2. Most frequently reported nonhematologic AEs of any grade were elevations in bilirubin, alanine aminotransferase, and triglycerides. These results support the use of nilotinib 300 mg twice daily as a standard-of-care treatment option for patients with newly diagnosed CML-CP.
Abstract
Activation induced cytidine deaminase (AID) enzyme, which converts cytosine into uracil and is expressed only by activated B lymphocytes, plays a role in B cells in both the mechanisms of ...somatic hypermutation (SHM) and class switch recombination (CSR). There are studies showing that AID can cause numerous translocations in different lymphoproliferative diseases. Chronic lymphocytic leukemia (CLL) is characterized by the accumulation of monoclonal B cells in bone marrow and peripheral blood. The predictability and clinical status of B-CLL are difficult to determine. About 30-50% of patients have chromosomal abnormalities. AID, which is thought to create fraction segments for translocations, might also cause deletions in DNA regions of 17p13, 11q22.3, 13q14 and 13q34 that are associated with prognostic implications in patients with CLL. In this study, the AID gene expression in patients with CLL with and without deletions was investigated. When compared to healthy subjects and patients without deletions, increased levels of AID expression in patients with deletions of 17p13, 11q22.3 or 13q14 were found, but not for the 13q34 region. Our results show that AID expression may be associated with deletions in patients with CLL.
Celotno besedilo
Dostopno za:
DOBA, IJS, IZUM, KILJ, NUK, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
Nilotinib is a BCR-ABL1 tyrosine kinase inhibitor approved for the treatment of patients with chronic myeloid leukemia in chronic phase (CML-CP). This study was the first prospective evaluation of ...the efficacy and safety of nilotinib in Turkish patients with newly diagnosed CML-CP. The primary endpoint of the study was the rate of major molecular response (MMR; BCR-ABL1 ≤ 0.1% on the International Scale BCR-ABL1
) by 12 months.
Patients with newly diagnosed CML-CP were treated with nilotinib 300 mg twice daily. This analysis was based on the first 12 months of follow-up in a 24-month study. This study is registered with ClinicalTrials.gov (NCT01274351).
Of 112 patients enrolled, 66.1% (80% CI, 59.7-72.0%) achieved MMR and 22.3% achieved a deep molecular response of MR
(BCR-ABL1
≤0.0032%) by 12 months. During the first year of treatment, one patient progressed to blast crisis and two patients died. Safety results were consistent with previous studies. Most adverse events (AEs) were grade 1/2. Most frequently reported nonhematologic AEs of any grade were elevations in bilirubin, alanine aminotransferase, and triglycerides.
These results support the use of nilotinib 300 mg twice daily as a standard-of-care treatment option for patients with newly diagnosed CML-CP with low and intermediate risk.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
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