scholarly journals Epidemiology and clinical features of retinoblastoma

2020 ◽  
Vol 09 (01) ◽  
pp. 56-58
Author(s):  
Maneya Padma ◽  
Nuthan Kumar ◽  
Prerana S. Nesargi ◽  
B.S Aruna Kumari ◽  
L. Appaji ◽  
...  
Keyword(s):  
High Risk ◽  
Bone Marrow Involvement ◽  
Intracranial Extension ◽  
Delayed Presentation ◽  
Male Predominance ◽  
Lower Socioeconomic ◽  
Case Files ◽  
Choroidal Invasion ◽  
Eye Examination ◽  
Epidemiological Profile

Abstract Introduction: Retinoblastoma (RB) is a prototype of heritable cancers. It is more common in the lower socioeconomic strata. Delayed presentation significantly reduces the overall outcome. We have analyzed the epidemiological and clinical data of children who were diagnosed with RB between the years 2009 and 2014. Aim: RB being a disease of the poor, delayed presentation is common due to lack of awareness. We have analyzed the epidemiological profile of our patients and tried to establish the link between delayed presentation and the presence of high-risk features. High-risk features are associated with higher chance of metastasis and poor rates of vision salvage in RB. Methodology: Data were collected in a retrospective manner from the patient case files retrieved from the Medical Records Department, Kidwai cancer Institute. The data were analyzed using Excel and SPSS software (IBM Corp. released 2016, IBM SPSS statistics software for Mac OS, version 24, IBM Corp., Armonk, NY). Results: A total of 53 patients were diagnosed with RB in the years 2009–2014. There was a male predominance with 1.2:1 incidence. Bilateral RB was present in 21 cases. The mean age of children with bilateral RB was 2.1 years, against 1.5 years in unilateral cases. High-risk features such as optic nerve invasion, choroidal invasion, intracranial extension, and orbital involvement were found in 12, 6, 5, and 5 eyes, respectively. Bone marrow involvement was detected in 5% and lung metastasis in 2%. Intracranial involvement was found in 10.4% and cerebrospinal fluid positivity in 15%. Children with high-risk features had a significant delay in presentation in comparison to those without high-risk features (P = 0.035). Conclusion: Incidence of metastatic disease and delayed presentation is still high in developing countries. Routine eye examination during vaccination visits can ensure early diagnosis and appropriate referral in many of these children.

scholarly journals Intensive Multiagent Therapy, Including Dose-Compressed Cycles of Ifosfamide/Etoposide and Vincristine/Doxorubicin/Cyclophosphamide, Irinotecan, and Radiation, in Patients With High-Risk Rhabdomyosarcoma: A Report From the Children's Oncology Group

2016 ◽  
Vol 34 (2) ◽  
pp. 117-122 ◽  
Author(s):  
Brenda J. Weigel ◽  
Elizabeth Lyden ◽  
James R. Anderson ◽  
William H. Meyer ◽  
David M. Parham ◽  
...  
Keyword(s):  
Risk Factors ◽  
Risk Factor ◽  
High Risk ◽  
Bone Marrow Involvement ◽  
High Risk Group ◽  
Historical Cohort ◽  
Dismal Prognosis ◽  
Risk Patients ◽  
Metastatic Sites

Purpose Patients with metastatic rhabdomyosarcoma (RMS), except those younger than 10 years with embryonal RMS, have an estimated long-term event-free survival (EFS) of less than 20%. The main goal of this study was to improve outcome of patients with metastatic RMS by dose intensification with interval compression, use of the most active agents determined in phase II window studies, and use of irinotecan as a radiation sensitizer. Patients and Methods Patients with metastatic RMS received 54 weeks of therapy: blocks of therapy with vincristine/irinotecan (weeks 1 to 6, 20 to 25, and 47 to 52), interval compression with vincristine/doxorubicin/cyclophosphamide alternating with etoposide/ifosfamide (weeks 7 to 19 and 26 to 34), and vincristine/dactinomycin/cyclophosphamide (weeks 38 to 46). Radiation therapy occurred at weeks 20 to 25 (primary) but was also permitted at weeks 1 to 6 (for intracranial or paraspinal extension) and weeks 47 to 52 (for extensive metastatic sites). Results One hundred nine eligible patients were enrolled, with a median follow-up of surviving patients of 3.8 years (3-year EFS for all patients, 38% [95% CI, 29% to 48%]; survival, 56% [95% CI, 46% to 66%]). Patients with one or no Oberlin risk factor (age > 10 years or < 1 year, unfavorable primary site of disease, ≥ three metastatic sites, and bone or bone marrow involvement) had a 3-year EFS of 69% (95% CI, 52% to 82%); high-risk patients with two or more risk factors had a 3-year EFS of 20% (95% CI, 11% to 30%). Toxicity was similar to that on prior RMS studies. Conclusion Patients with metastatic RMS with one or no Oberlin risk factor had an improved 3-year EFS of 69% on ARST0431 compared with an historical cohort from pooled European and US studies; those with two or more risk factors have a dismal prognosis, and new approaches are needed for this very-high-risk group.

The predictive value of magnetic resonance imaging of retinoblastoma for the likelihood of high-risk pathologic features

2018 ◽  
Vol 29 (2) ◽  
pp. 262-268 ◽  
Author(s):  
Jamila G Hiasat ◽  
Alaa Saleh ◽  
Maysa Al-Hussaini ◽  
Ibrahim Al Nawaiseh ◽  
Mustafa Mehyar ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Optic Nerve ◽  
High Risk ◽  
Magnetic Resonance ◽  
Predictive Value ◽  
Resonance Imaging ◽  
Cut Edge ◽  
Pathologic Features ◽  
Nerve Invasion ◽  
Choroidal Invasion

Purpose: To evaluate the predictive value of magnetic resonance imaging in retinoblastoma for the likelihood of high-risk pathologic features. Methods: A retrospective study of 64 eyes enucleated from 60 retinoblastoma patients. Contrast-enhanced magnetic resonance imaging was performed before enucleation. Main outcome measures included demographics, laterality, accuracy, sensitivity, and specificity of magnetic resonance imaging in detecting high-risk pathologic features. Results: Optic nerve invasion and choroidal invasion were seen microscopically in 34 (53%) and 28 (44%) eyes, respectively, while they were detected in magnetic resonance imaging in 22 (34%) and 15 (23%) eyes, respectively. The accuracy of magnetic resonance imaging in detecting prelaminar invasion was 77% (sensitivity 89%, specificity 98%), 56% for laminar invasion (sensitivity 27%, specificity 94%), 84% for postlaminar invasion (sensitivity 42%, specificity 98%), and 100% for optic cut edge invasion (sensitivity100%, specificity 100%). The accuracy of magnetic resonance imaging in detecting focal choroidal invasion was 48% (sensitivity 33%, specificity 97%), and 84% for massive choroidal invasion (sensitivity 53%, specificity 98%), and the accuracy in detecting extrascleral extension was 96% (sensitivity 67%, specificity 98%). Conclusions and relevance: Magnetic resonance imaging should not be the only method to stratify patients at high risk from those who are not, eventhough it can predict with high accuracy extensive postlaminar optic nerve invasion, massive choroidal invasion, and extrascleral tumor extension.

scholarly journals Epidemiology of Thalassemia in Gulf Cooperation Council Countries: A Systematic Review

2020 ◽  
Vol 2020 ◽  
pp. 1-15
Author(s):  
Amani Abu-Shaheen ◽  
Humariya Heena ◽  
Abdullah Nofal ◽  
Doaa A. Abdelmoety ◽  
Abdulrahman Almatary ◽  
...  
Keyword(s):  
Systematic Review ◽  
High Risk ◽  
United Arab Emirates ◽  
Observational Studies ◽  
Gulf Cooperation Council ◽  
Cross Sectional ◽  
Gcc Countries ◽  
Premarital Screening ◽  
Risk Areas ◽  
Epidemiological Profile

Background. Thalassemia has a burden on the healthcare systems of many countries. About 56000 conceptions result in thalassemia, globally. Objective. To assess the epidemiological profile of thalassemia in the Gulf Cooperation Council (GCC) countries. Methods. A systematic search was conducted in MEDLINE/PubMed (National Library of Medicine), CINAHL, and Embase. Relevant observational studies reporting the epidemiology of thalassemia among the GCC population were selected. Data on the prevalence, frequency, and complications of thalassemia were extracted. The quality of the retrieved studies was assessed according to the Newcastle–Ottawa Quality Assessment Scale. Results. Eighteen studies (14 cross-sectional studies, two retrospective observational studies, and two retrospective analysis) with a total of 3343042 participants were included in this systematic review. Of the 18 studies, 11 studies were conducted in Saudi Arabia, two in the Kingdom of Bahrain, one in Kuwait, three in the United Arab Emirates (UAE), and one in Qatar. The prevalence of thalassemia among children below five years of age ranged from 0.25% to 33%, while it was 0.9% in children above five years and from 0.035% to 43.3% among adult thalassemia patients. The most-reported risk factors were consanguineous marriage and high-risk marriage. There was a lack of data regarding mortality rates in thalassemia. Conclusions. Despite the premarital screening and genetic counseling (PMSGC) program for thalassemia, the incidence of high-risk couple marriages in GCC countries cannot be effectively diminished. This study suggested that the PMSGC program should adopt more attention for the high-risk areas, to enhance the level of consciousness about the hemoglobinopathy diseases and the consequences of consanguinity among the at-risk couple.

scholarly journals Epidemiological Profile of Malignant Brain Neoplasms in the Northern Region of Brazil: data from the Cancer Hospital Registry of the Instituto Nacional de Câncer

2017 ◽  
Vol 38 (02) ◽  
pp. 094-101
Author(s):  
Carlos Eduardo Adriano Filho ◽  
Lenon Machado Pinto ◽  
Joel Monteiro de Jesus
Keyword(s):  
Brain Neoplasms ◽  
Age Groups ◽  
Northern Region ◽  
Astrocytic Tumors ◽  
Embryonal Tumors ◽  
Male Predominance ◽  
Cancer Hospital ◽  
Incidence And Mortality ◽  
Epidemiological Profile ◽  
Hospital Registry

Objective Tumors of the central nervous system (CNS) are considered rare, with an incidence of 3.4 cases per 100,000 individuals worldwide. Although uncommon, CNS tumors have been gaining epidemiological importance due to their increased incidence and mortality. In Brazil, there is a lack of population research regarding CNS cancer, especially in the Northern region. Thus, the authors aim to trace an epidemiological profile of malignant brain neoplasms in the Northern region from 2001 to 2013. Methods Data were collected from the Cancer Hospital Registry of the Instituto Nacional de Câncer (RHC-INCA, in the Portuguese acronym) and stratified according to origin, gender, age, detailed primary location, and histological type. A total of 742 cases were analyzed. Most of the cases came from inland areas, with a male predominance. Results The most affected age groups were between 0 and 9 years old and between 30 and 49 years old, with an accentuated decrease in incidence starting at the age of 70 years old. The frontal lobe was the most affected area, followed by the temporal and parietal lobes. Astrocytic tumors accounted for 64.3% of cases, followed by embryonal tumors (18.2%), and ependymal tumors (7.4%). Among the astrocytic tumors, astrocytoma, not otherwise specified (NOS), and glioblastoma, NOS corresponded to 82.2% of the cases. Among embryonal tumors, medulloblastoma accounted for 71.9% of the cases. Conclusion More epidemiological studies in this area, especially in the Northern region, are required to identify risk factors and allow prevention and early diagnosis.

scholarly journals 983 Epidemiological Profile of the Service Plan for the High Risk in Maternal-Fetal Public Teaching Hospital

2012 ◽  
Vol 97 (Suppl 2) ◽  
pp. A281-A281
Author(s):  
A. Veiga ◽  
E. Veiga ◽  
M. Soares ◽  
N. Moliterno ◽  
S. Nogueira ◽  
...  
Keyword(s):  
High Risk ◽  
Teaching Hospital ◽  
Service Plan ◽  
Epidemiological Profile

High-risk neuroblastoma in a sub-Saharan African country: telling it like it is

2017 ◽  
Vol 47 (4) ◽  
pp. 370-374 ◽  
Author(s):  
GP Hadley ◽  
J van Heerden
Keyword(s):  
High Risk ◽  
Poor Prognosis ◽  
African Country ◽  
Primary Tumour ◽  
Healthcare Providers ◽  
Current Treatment ◽  
Delayed Presentation ◽  
High Risk Disease ◽  
Sub Saharan ◽  
The Cost

Neuroblastoma is uncommon in Africa, but when seen usually presents as high-risk disease with a poor prognosis. This aggressive biology of the tumour is frequently augmented by delayed presentation. Current treatment depends upon technologies and skills that are scarce in developing countries and the cost involved is generally beyond the means of healthcare providers who are faced with a myriad more pressing healthcare issues. The presentation, treatment and outcome of 45 African children with neuroblastoma are described. Due to a lack of resources precise risk stratification was impossible but visceral or bone metastases were present in 73% of patients at diagnosis. In 91% the primary tumour was intra-abdominal. Three children (7%) were paraplegic on admission. A localised tumour was seen in one child (2%). Fifteen children (33%) underwent a surgical procedure, with intent to cure in five among whom resection was incomplete in three. For all other children, treatment was palliative using chemotherapy with judicious use of radiotherapy. Thirteen children (29%) survived longer than six months. Overall survival at three years was 4%.

Prolonged Survival of Lymphoma Patients Achieving Complete Remission Following High-Dose Sequential Chemotherapy and Autograft (HDS Program): A GITIL (Gruppo Italiano Terapie Innovative Nei Linfomi) Retrospective Study on 1,266 Patients.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3046-3046
Author(s):  
Corrado Tarella ◽  
Manuela Zanni ◽  
Alessandro Rambaldi ◽  
Michele Magni ◽  
Marco Sorio ◽  
...  
Keyword(s):  
High Risk ◽  
Complete Remission ◽  
Treatment Options ◽  
Bone Marrow Involvement ◽  
Salvage Treatment ◽  
Prolonged Survival ◽  
High Dose ◽  
Low Grade ◽  
Term Outcome ◽  
Long Term Outcome

Abstract Introduction. The high-dose sequential (HDS) chemotherapy approach is characterized by early dose-intensification followed by autograft with peripheral blood progenitor cells (PBPC). The HDS program was introduced several years ago (Gianni & Bonadonna, 1989); subsequently, it has been increasingly used in the management of both non-Hodgkins (NHL) and Hodgkins Lymphoma (HL). The outcome of a large series of lymphoma patients treated with the HDS approach at 10 Centers associated to GITIL is reported. Patients and Methods. Data have been collected on 1,266 patients, who received either the original or slightly modified HDS regimens. There were 213 HL and 1,053 NHL patients (630 intermediate/high-grade, 423 low-grade); median age was 46 yrs, 57% were male. Overall, 671 (53%) patients received HDS as salvage treatment after one or more recurrence; 595 (47%) had HDS front-line, either for high-risk clinical presentation or unfavorable histology, i.e. mantle-cell l. Most patients were autografted with PBPC, few received BM cells (alone or with PBPC); 158 (12%) patients did not undergo autograft, due to several reasons, namely: toxicity, disease progression, poor harvests. Results. Overall, 1,013 (80%) patients reached Complete Remission (CR) following the HDS program. Up to now, 93 (7%) patients died for early/late toxicities, 328 (26%) died for lymphoma, 844 are known to be alive; at a median follow-up of 5 yrs, the 5-yr Overall Survival (OS) projection is 64% (s.e. 2%). As shown in Figure 1 A and B, a significantly higher survival was observed in patients receiving HDS at diagnosis vs. those at relapse and in those achieving CR vs. no CR patients. On multivariate Cox survival analysis, these two parameters maintained a significant impact on the 5-yr survival (relapse status at HDS: HR 1.39, c.i.: 1.12–1.72; CR achievement: HR 0.12, c.i.: 0.10–0.16). Also some histological features (low grade vs intermediate/high; B-cell vs. T-cell) had a significant impact on OS, whereas other parameters, including sex, bone marrow involvement, HL vs NHL, use of hd-Ara-C, had no relevance. Conclusions. the HDS program including PBPC collection and re-infusion is feasible in a multicenter setting and allows prolonged survival in a good proportion of lymphoma patients presenting with unfavorable prognosis; the long-term outcome is definitely good in patients achieving CR; given their poor outcome, early salvage treatment options, including allogeneic transplant, should be considered for those patients unable to reach CR following a HDS treatment approach. Figure 1. OS according to status at HDS ( A ) and response following HDS ( B ) in 1,266 high-risk lymphoma patients Figure 1. OS according to status at HDS ( A ) and response following HDS ( B ) in 1,266 high-risk lymphoma patients

Addition of Rituximab to Mobilization and Conditioning Overcomes the Predictive Value of Follicular Lymphoma International Prognostic Index (FLIPI) Score in Patients with Relapsed FL Undergoing Autologous Stem Cell Transplantation (AUTO).

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 1140-1140 ◽  
Author(s):  
Issa F Khouri ◽  
Roland Bassett ◽  
Barry Samuels ◽  
Farzaneh Maadani ◽  
Grace-Julia Okoroji ◽  
...  
Keyword(s):  
Stem Cell ◽  
High Risk ◽  
Bone Marrow Involvement ◽  
International Prognostic Index ◽  
Low Risk ◽  
Disease Stage ◽  
High Dose ◽  
Prior Chemotherapy ◽  
Morbidity Score ◽  
Co Morbidity

Abstract FLIPI is emerging as an important prognostic factor in patients (pts) with FL. In order to determine its significance in FL pts undergoing AUTO, we examined the outcome of 75 consecutive pts transplanted at the MD Anderson Cancer Center between 02/94 and 04/08. Pts were eligible if they had relapsed chemosensitive disease, and had no HLAidentical sibling donor. Twenty-nine pts were transplanted without rituximab (AUTO-R), and 46 pts received high-dose rituximab (AUTO+R) during stem cell mobilization and on days +1 and + 8 after transplantation as previously described (Khouri, JCO, 2005). Median age (range) at AUTO was 54 (33–76) and 49 (35–63) for the AUTO+R and AUTO-R groups respectively (P =0.002). FLIPI was determined at the time of transplantation; more patients had intermediate-high risk in AUTO+R than in AUTO-R (58% vs 27.5%, respectively, P = 0.011). Other patients characteristics were balanced for gender, time from diagnosis, histology subtypes (grades 1,2, 3a, and 3b), disease stage, LDH, bulk, B-symptoms, B-2microglobulin, bone marrow involvement, number of prior chemotherapy regimens received, remission status (CR vs PR), functional imaging, and co-morbidity score. Median follow-up (range) in months was 20 (1–88) for AUTO+R and 70 (4–167) for AUTO-R. Progression-free survival (PFS) was significantly different between AUTO+R and AUTO-R (P = 0.004), with estimated three-year PFS of 48% for AUTO-R and 79% for AUTO+R. Using Cox proportional hazards regression models, the # of prior chemotherapy regimens received (&lt;3 vs &gt;/=3) (P = 0.015) was the only factor associated with PFS in the AUTO+R group, whereas both age (P=0.010), and risk based on FLIPI (P=0.019) were independently associated with PFS in the AUTO-R group. Pts with low-risk vs. intermediate/high-risk had three-year PFS of 62% and 13%, respectively, in the AUTO-R group, whereas in the AUTO+R group, three-year PFS was 90% and 76% in the low-risk and intermediate/high-risk pts, respectively (Figure). Conclusions: These results suggest that the addition of R to the mobilization and conditioning improves the outcome in pts with relapsed chemosensitive FL treated by AUTO. The number of prior chemotherapy regimens received rather than FLIPI score is the most important determinant of outcome in this setting. Figure Figure

Radioimmunotherapy (RIT) with 90yttrium Zevalin Followed by BEAM Conditioning Regimen (Z-BEAM) and Autologous Stem Cell Transplantation (ASCT) for the Treatment of High Risk Relapsed/Resistant Non Hodgkin's Lymphoma (NHL)

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 3107-3107
Author(s):  
Barbara Botto ◽  
Chiara Ciochetto ◽  
Marilena Bellò ◽  
Roberto Passera ◽  
Giulia Benevolo ◽  
...  
Keyword(s):  
High Risk ◽  
Conflicts Of Interest ◽  
Standard Dose ◽  
Bone Marrow Involvement ◽  
Conditioning Regimen ◽  
Progression Free Survival ◽  
Matched Pair ◽  
High Dose ◽  
Bulky Disease ◽  
Response Status

Abstract Abstract 3107 High dose chemotherapy (HDC) and ASCT is actually considered an effective treatment for relapsed NHL. Standard dose Zevalin (0.4 mCi/kg) combined with conventional BEAM (Z-BEAM) is a promising conditioning regimen for the treatment of high risk relapsed/resistant NHL. We evaluated the feasibility and the efficacy of Z-BEAM in a group of relapsed/refractory patients treated in a single institution. Between October 2006 and December 2010 twenty nine pts were treated with Zevalin (day –14) followed by standard dose BEAM (day –7 to –1) and ASCT. Patients were included into the study and considered at high risk of failure if showed: progression or early relapse (<1 year) from previous therapy or multiple relapses. Rituximab followed by standard dose DHAP or ICE were used as debulking and mobilizing schedule. Clinical characteristics were as follows: 14 refractory and 15 early or multiple relapse; 8 grade I-II follicular, 16 PML/DLBCL, 3 MCL, 2 indolent non follicular; 6 stage II and 23 stage III-IV; 10 patients had bulky disease and 15 bone marrow involvement; 9 LDH level above normal. 13 patients received only one previous line of treatment and 16 were treated with 2 or more lines before Z-BEAM, all containing Rituximab. Only 5/29 patinets received a reducted dose of 0.3 mCi/kg Zevalin because of low platelets counts. Response status before RIT was: 14 CR (49%), 9 PR (33%), 3 SD (9%) and 3 PD (9%). At the end of treatment response status is actually available in 26/29 pts: CR 18(69%), PR 5(19%) and PD 3 (12%). Overall response rate was converted from 81% before Z-BEAM and ASCT to 88% at the end of the entire program. Median CD34+ cells infused was 7.26 106/kilograms (range 4.43–8.9). All pts engrafted with median time to platelet and neuthrophils count higher than 20×109/l and 0.5×109/L of 11 and 10 days respectively. Febrile neutropenia occurred in 12/29 pts. One pulmonary Aspergillosis and 8 bacteriemia were documented. One patient experienced an intestinal perforation during aplasia and one cardiac failure was documented in a woman previously treated with cumulative antraciclines doses and mediastinal radiotherapy. With a median follow up of 15 months progression free survival (PFS) is 79% and overall survival is 83%. 4/14 pts before Z-BEAM showed a subsequent progression and 2/15 relapsed: five pts died of lymphoma. No toxic deaths were recorded. In this group of pts with high risk relapsed/resistant NHL Z-BEAM+ASCT is able to achieve a good response with engraftment and toxicity not different from standard BEAM. This approach needs to be tested in a larger multicenter study. A matched pair analysis to compare this group with a similar group treated with standard BEAM without Zevalin is actually planned in our institution Disclosures: No relevant conflicts of interest to declare.

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