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Dismal Outcome (dismal + outcome)
Selected AbstractsHigh incidence of t(7;12)(q36;p13) in infant AML but not in infant ALL, with a dismal outcome and ectopic expression of HLXB9GENES, CHROMOSOMES AND CANCER, Issue 8 2006Anne R. M. von Bergh The t(7;12)(q36;p13) is a recurrent translocation involving the ETV6/TEL gene (12p13) and a heterogeneous breakpoint at 7q36. A fusion transcript between HLXB9 and ETV6 in AML with t(7;12) is occasionally found. To study the incidence of t(7;12) in infant and childhood acute leukemia, we screened 320 cases <36 months using FISH. Additionally, 28 pediatric cases >36 months with cytogenetic breakpoints at 12p and 7q were investigated. We studied the presence of an HXLB9-ETV6 fusion transcript and quantified the expression of various genes located in the 7q36 breakpoint region. In total, six AML patients carried the t(7;12) of which five were infants and one child of 18 months. Only one out of 99 infant ALL patients harbored the t(7;12). No t(7;12) was found in older children with AML or ALL. AML patients carrying a t(7;12) had a poor outcome with a 3-year EFS of 0%. A fusion of HLXB9 to ETV6 was found in four AML cases with t(7;12). The 7q36 genes NOM1, LMBR1, RNF32, and SHH were equally expressed among t(7;12)-positive AML versus t(7;12)-negative AML, t(7;12)-negative ALL, or normal bone marrow. However, the HLXB9 expression was highly increased in t(7;12)-positive cases, including those with an HLXB9-ETV6 fusion. We conclude that the t(7;12) is almost exclusively present in infant AML and covers 30% of infant AML, while it is extremely rare in infant ALL and older children. The t(7;12) is associated with a poor outcome and an ectopic expression of HLXB9 is commonly involved in this genetic subtype of leukemia. © 2006 Wiley-Liss, Inc. [source] Peripheral T-cell lymphomas, unspecified (or not otherwise specified): a reviewHEMATOLOGICAL ONCOLOGY, Issue 1 2008Delvys Rodriguez-Abreu Abstract Peripheral T-cell lymphomas (PTCL) comprises a heterogeneous group of haematological tumours, which originate from mature T-cells, and constitute less than 15% of all non-Hodgkin's lymphomas (NHLs) in adults. The current WHO classification recognizes nine distinct clinicopathologic peripheral T-cell NHLs, being the ,unspecified variant' (PTCL-U) the most common subtype. These neoplasms often present in advanced stage at diagnosis, and most commonly have an aggressive clinical course requiring prompt treatment. The rarity of these tumours requires additional studies to better understand their biology and search for new therapies which may hopefully improve the dismal outcome of most patients. This review aims to describe the pathobiological aspects as well the clinical characteristics and current therapeutic strategies of the PTCLs, with special attention to the group of PTCL-U Copyright © 2007 John Wiley & Sons, Ltd. [source] Primary biliary cirrhosis in Singapore: Evaluation of demography, prognostic factors and natural course in a multi-ethnic populationJOURNAL OF GASTROENTEROLOGY AND HEPATOLOGY, Issue 4 2008Reuben-Km Wong Abstract Background and Aim:, Primary biliary cirrhosis (PBC) is infrequent in Asians. Among Asian patients with PBC, information on natural course is scarce. The aim of this study was to study the clinical course and prognosticators among Asians with PBC. Methods:, During 1990,2005, patients diagnosed with PBC at the National University Hospital, Singapore, constituted the retrospective cohort. Their demographic characteristics were evaluated. To evaluate the prognostic factors and natural course, two outcome measures were assessed: hepatic decompensation and death or liver transplant. Multivariate analysis was undertaken to identify factors associated with hepatic decompensation and terminal event (death or liver transplantation). Results:, Thirty-four PBC patients aged 56.8 ± 1.8 years (mean ± SEM) of whom 32 (94%) were women were included. Thirty-two (94%) of them were of Chinese origin. At presentation, 18 (53%) were symptomatic in the form of jaundice (n = 9, 26.5%), pruritus (n = 6, 17.6%) and fatigue (n = 5, 14.7%). During 4.80 ± 0.7 (range 0.02,15.03) years follow up, 6/16 (37.5%) asymptomatic patients developed symptoms. After 5 years, 17.6% (n = 6) and 8.8% (n = 3) had hepatic decompensation and terminal event, respectively. Sicca syndrome was present in 26% (n = 9) of patients. Multivariate analysis revealed that serum bilirubin level at presentation was the sole determinant of decompensation. Rate of change of laboratory indices did not predict either event. Conclusion:, In Singapore, Chinese women constitute most of the PBC patients. Elevated serum bilirubin level at presentation was the sole predictive marker associated with dismal outcome. [source] Desmoplastic small round cell tumor in childhood: The St. Jude Children's Research Hospital experiencePEDIATRIC BLOOD & CANCER, Issue 3 2007Raya Saab MD Abstract Background Desmoplastic small round cell tumor (DSRCT) is a rare, primarily intra-abdominal tumor that has a poor outcome with current therapies. Procedure We retrospectively reviewed patient characteristics, presenting symptoms, tumor pathology, treatment, and outcome of 11 pediatric patients with DSRCT at our institution. Results The cohort included 1 female and 10 male patients. Median age at diagnosis was 14 years (range 5,21 years). In eight (73%) patients, the primary tumor was abdominal or pelvic, and in one patient each, it was submental, mediastinal, and paratesticular. Nine (82%) patients had metastatic disease. All tumors showed polyphenotypic differentiation by immunohistochemistry. The EWS-WT1 transcript was detected in six of seven tumors tested. One tumor showed rhabdomyoblastic differentiation after therapy. All patients received chemotherapy; eight underwent surgical resection, seven received primary site radiation, and four received myeloablative chemotherapy with stem-cell support. Three (27%) patients are alive 23 months, 8 years, and 10 years from diagnosis. Two died of treatment-related toxicity, six died of disease. None of the patients in whom surgery and initial chemotherapy failed to induce complete remission survived. Conclusions DSRCT is an aggressive malignancy that does not respond well to contemporary treatments, and patients who do not enter complete remission after initial chemotherapy and surgery appear to have a particularly dismal outcome. Patients with localized extra-abdominal disease have a better prognosis, most likely due to increased feasibility of resection. Better understanding of molecular and genetic mechanisms of tumorigenesis and treatment-related changes may contribute to development of more effective therapy for DSRCT. Pediatr Blood Cancer 2007;49:274,279. © 2006 Wiley-Liss, Inc. [source] Predictors of androgen independence in metastatic prostate cancerBJU INTERNATIONAL, Issue 9 2004H.G. Sim OBJECTIVE To assess the factors that influence the onset of androgen independence (AI, which heralds a dismal outcome) in patients with metastatic prostate carcinoma. PATIENTS AND METHODS The records of 361 consecutive patients with prostate carcinoma diagnosed and treated in the authors' institution from 1 January 1996 to 31 December 1999 were reviewed retrospectively; 92 with metastatic prostate carcinoma were assessed (median age 71.0 years, range 42,93). Patients were included if they developed metastatic disease from prostate cancer at the time of diagnosis. The nadir for prostate specific antigen (PSA) level was defined as the date of the lowest PSA level after hormonal therapy, and AI was defined as the date of the third consecutive PSA increase above the nadir value by any threshold. RESULTS The median Gleason sum was 8 and the modal Gleason score 4 + 5. The median (range) pretreatment PSA level was 274.0 (1.3,2179) ng/mL. Of the 92 men, 57 (62%) attained a nadir PSA, including 23 with a nadir of <,2 ng/mL; 32 (35%) progressed to AI within 2 years and 27% reached a nadir PSA but did not develop AI. The mean (sd) time from diagnosis to the nadir PSA was 13.7 (11.8) months, while the mean time from diagnosis to progression to AI was 30.3 (15.6) months. Univariate analysis showed that a nadir PSA level after treatment of ,,1 ng/mL (P = 0.0128) was an early predictor of progression to AI; a nadir PSA level of ,,2 ng/mL (P = 0.0216) was a predictor of poor overall survival. CONCLUSION Failure to attain a nadir PSA of <,1 ng/mL after treatment predicts progression to AI and a nadir PSA of >,2 ng/mL predicts poorer overall survival. The development of skeletal events predicts the onset of AI but occurs late in the disease and is unsuitable as an early prognostic marker. [source] High-dose Ara-C and beam with autograft rescue in R-CHOP responsive mantle cell lymphoma patientsBRITISH JOURNAL OF HAEMATOLOGY, Issue 4 2009Mars B. Van't Veer Summary Mantle cell lymphoma (MCL) has a dismal outcome when treated with conventional chemotherapy. This single arm phase 2 study evaluated intensive consolidation treatment of patients with newly diagnosed MCL up to the age of 65 years, responsive to R-CHOP (rituximab, cyclophosphamide, oncovin, adriamycin, prednisolone). Endpoints for evaluation were toxicity, failure-free survival (FFS) and overall survival (OS). Eighty-seven patients were treated with three cycles of R,CHOP. Sixty-six patients responded to R-CHOP with at least a partial response, 62 continued protocol treatment with high-dose cytarabine (Ara-C; 2000 mg/m2, bid. over 4 d) and 61 patients received rituximab and stem cell harvest, followed by BEAM (carmustine, etoposide, Ara-C, melphalan) and autologous stem cell rescue. Non-haematological toxicity, grades III and IV, was seen in 8% of the patients after R-CHOP, in 22% after high-dose Ara-C and in 55% after BEAM. The overall response rate was 70% (complete response rate 64%, partial response rate 6%), FFS and OS at 4 years were 36 ± 7% and 66 ± 6%, respectively. The FFS and OS at 4 years from the evaluation after BEAM in the 61 R-CHOP responsive patients was 46 ± 9% and 79 ± 7%, respectively. In conclusion, high-dose Ara-C and BEAM with stem cell rescue in newly diagnosed MCL patients responsive to R-CHOP is a manageable treatment with respect to toxicity. This regimen leads to long-term, but probably not durable, remissions. [source] Neonatal testicular torsion , a lost cause?ACTA PAEDIATRICA, Issue 4 2008Cheri Mathews John Abstract Twenty-four neonates presented with signs of testicular ischaemia over a 13-year period. They had a mean birth weight of 3.706 kg. The right testicle was affected in 13, the left in 9 and there was bilateral torsion in 2 babies. Two babies had no twist in the cord, but the testicles were nonviable macroscopically and microscopically. Twenty-one babies had primary exploration revealing necrotic testes in all patients and they underwent orchidectomies. The other three babies had conservative management and the affected testes had atrophied on follow-up. Sixteen babies had contralateral orchidopexy. Doppler ultrasound scans were reported as normal in 2 of 13 babies who had scans. No testes were salvaged following surgery. Conclusion: The incidence of testicular torsion in the neonatal period was calculated as 6.1 per 100 000 live births. No testis was salvaged following surgery in our series of 24 patients. This dismal outcome underlines that immediate surgical exploration, although commonly performed, rarely saves torted testes. [source] Adjuvant radiation therapy is associated with improved survival for gallbladder carcinoma with regional metastatic diseaseJOURNAL OF SURGICAL ONCOLOGY, Issue 1 2007Pablo Mojica MD Abstract Background Gallbladder carcinoma is a rare malignancy and is associated with dismal outcomes. The aim of this study was to better define the role of adjuvant radiation therapy in the management of gallbladder carcinoma. Methods The Surveillance, Epidemiological, and End Results (SEER) survey from the National Cancer Institute was queried from 1992 to 2002. Retrospective analysis was done. The end-point of the study was overall survival. Results There were a total of 3,187 cases of gallbladder carcinoma in the registry from 1992 to 2002. Of the surgical group, 35% were stage I, 36% were stage II, 6% were stage III, and 21% were stage IV. Adjuvant radiation was used in 17% of the cases. The median survival for those patients receiving adjuvant radiation therapy was 14 months compared to an 8 months median survival for those treated without adjuvant radiation therapy (P,,,0.001). The survival benefit associated with radiation use was only presenting those patients with regional spread (P,=,0.0001) and tumors infiltrating the liver (P,=,0.011). Conclusion The use of adjuvant radiation therapy is associated with improved survival in patients with locally advanced gallbladder cancer or gallbladder cancer with regional disease. J. Surg. Oncol. 2007;96:8,13 © 2007 Wiley-Liss, Inc. [source] | ||||||||||||||||||||||