scholarly journals Small-Area Analysis of Incidence and Localisation of Vulvar Cancer

2010 ◽  
Vol 2010 ◽  
pp. 1-6 ◽  
Author(s):  
Klaus H. Baumann ◽  
Olga Müller ◽  
Helke B. Naujok ◽  
Ellen Mann ◽  
Peter Barth ◽  
...  
Keyword(s):  
Older Women ◽  
Small Area ◽  
Vulvar Cancer ◽  
Disease Onset ◽  
University Hospital ◽  
Hospital Unit ◽  
Local Extension ◽  
Disease Characteristics ◽  
Recent Developments ◽  
Rising Incidence

Objective. Vulvar cancer is a rare disease mainly in older women. HPV and non-HPV induced vulvar cancer reflect two types of oncogenesis. Controversies exist on most recent developments in vulvar cancer incidence, patients, and disease characteristics. Changes in incidence, age of disease onset, and tumor site in women treated for primary vulvar cancer in a single German university hospital unit will be described.Methods. A retrospective analysis of patient records of women treated between 1994 and 2008 was performed. The fifteen-year-spanning period was divided into three five year-spanning cohorts. Descriptive and statistical analyses were performed.Results. 104 patients were identified: cohort-1 from 1994 to 1998 (11 patients); cohort-2 from 1999 to 2003 (21 patients); cohort-3 from 2004 to 2008 (72 patients). Mean age (years) was 73.18 (confidence interval (CI): 64.04; 82.33), 58.9 (CI: 52.24; 65.57), and 61.19 (CI: 57.27; 65.12), respectively. Vulvar cancer confined to the region between clitoris and urethra was seen more often in cohort-3 () compared to cohort-1 () or cohort-2 ().Conclusion. This analysis supports the notion of rising incidence of vulvar cancer and a changing pattern of anatomical local extension. Disease onset is not restricted to older women.

Sociodemographic determinants of disability in patients with schizophrenia (Bulgaria, 2018-2019)

2020 ◽  
Vol 30 (Supplement_5) ◽  
Author(s):  
I Veleva ◽  
S Yankulovska ◽  
K Velikova ◽  
M Valkova ◽  
G Grancharova
Keyword(s):  
Social Functioning ◽  
Protective Factor ◽  
Paranoid Schizophrenia ◽  
Disease Onset ◽  
Public Health Problem ◽  
Sociodemographic Factors ◽  
University Hospital ◽  
Psychiatric Clinic ◽  
Sociodemographic Determinants ◽  
The Impact

Abstract Background Schizophrenia is an increasing public health problem. Despite its low prevalence and mortality, it causes significant disability and has enormous health, social, and economic burden for patients, families, society and health systems worldwide. The aim was to assess the impact of some sociodemographic determinants on disability in patients with paranoid schizophrenia (PS). Methods The study includes 108 patients in Psychiatric Clinic at Pleven University Hospital: 66 males and 42 females, mean age 38.9±10.0, duration of disease 12.8±8.2 years; mean onset of disease - 24 years (Me = 25 and Mo = 22). The study was approved by Ethical Committee. The disease burden was assessed by Positive and Negative Syndrome Scale (РANSS), and level of disability - by 36-item WHO Disability Assessment Schedule 2.0. Four variables (gender, age, education, type of employment) were chosen and their impact on disability was assessed by one-way ANOVA (F-test), Tukey's NDS test, two-sided t-test, Pierson r. Data processing was performed by IBM SPSS v.24. Results The most severe deficits were observed in “getting along with people” (61.34±25.77),”life-activities-household” (55.74±23.45), “participation in society” (53.13±19.12), “understanding and communicating” (41.67±17.67). Age at disease onset and its duration showed weak positive correlation. In most domains, males had higher deficits (р=0.0001). Age had an impact only on “getting around” (r = 0.41;p=0.0001) and “self-care” (r = 0.260;p=0.007). Significant differences (F (2,104)=7.565, p < 0.01) were found between groups by education level and occupation (employed/students, unemployed and disabled) - F (3,103)=16.734; p<0.001. Conclusions Sociodemographic factors play a major role for different function domains deficits in PS patients. Most pronounced is the impact of education and occupation. Patients with higher education and those engaged in study or work demonstrate better social functioning than less educated and disabled people. Key messages Education is an important protective factor for disability. Lower educational level decreases competitiveness in workforce market, both in general population and in patients with schizophrenia. Sociodemographic determinants should be considered in planning appropriate preventive and rehabilitation activities to stimulate better social functioning and adaptation of PS patients.

scholarly journals Comparing Five Year Out-Come in Two Cohorts of Patients with Early Rheumatoid Arthritis – A BARFOT Study

2015 ◽  
Vol 9 (1) ◽  
pp. 8-15 ◽  
Author(s):  
Maria L.E Andersson ◽  
Kristina Forslind ◽  
Ingiäld Hafström
Keyword(s):  
Prospective Observational Study ◽  
Disease Activity Score ◽  
Disease Duration ◽  
Disease Onset ◽  
Health Assessment ◽  
Disease Characteristics ◽  
Group 2 ◽  
Assessment Questionnaire ◽  
Group 1

The objective of the study was to compare disease characteristics over the first 5 years of disease in patients with RA, with disease onset in 1990s and 2000s, respectively. Methods : All 2235 patients with early RA (disease duration ≤12 months) were recruited from the BARFOT prospective observational study. These patients were divided into group 1 included 1992 to 1999 (N=1084, 66% women) and group 2 included 2000 to 2006 (N=1151, 69% women). Disease Activity Score (DAS28), VAS pain and Health Assessment Questionnaire (HAQ) were assessed during 5 years. Remission was defined as DAS28 <2.6. Results : At inclusion, both women and men in group 2 had higher mean DAS28 (SD) than group 1, 5.42 (1.22) vs 5.26 (1.19), p=0.004 and 5.28 (1.22) vs 5.00 (1.27), p=0.004, respectively, mainly dependant on pain and not on inflammatory related measures. Over time DAS28 decreased and was in both genders, from 6 months to the 5-year follow-up, significantly lower in group 2. At 5-year, both women and men in group 2 had higher rate of remission than women and men in group 1. However, despite reduction of VAS pain and HAQ there were no differences in pain and HAQ between groups at any time point. Conclusion : Patients included in the 2000s achieved higher frequency of remission at the 5 year follow-up compared with those included in the 1990s, suggested to reflect the more active medical treatment. Interestingly, however, improvement in pain and HAQ did not differ between the two patient cohorts.

scholarly journals Multisystem proteinopathy due to a homozygous p.Arg159His VCP mutation

Neurology ◽  
2019 ◽  
Vol 94 (8) ◽  
pp. e785-e796 ◽  
Author(s):  
Willem De Ridder ◽  
Abdelkrim Azmi ◽  
Christoph S. Clemen ◽  
Ludwig Eichinger ◽  
Andreas Hofmann ◽  
...  
Keyword(s):  
Quantitative Proteomics ◽  
Disease Onset ◽  
Index Patient ◽  
Bioinformatic Analysis ◽  
Muscle Involvement ◽  
Proteomic Data ◽  
Disease Characteristics ◽  
Whole Exome ◽  
Disease Signatures ◽  
Muscle Biopsies

ObjectiveTo assess the clinical, radiologic, myopathologic, and proteomic findings in a patient manifesting a multisystem proteinopathy due to a homozygous valosin-containing protein gene (VCP) mutation previously reported to be pathogenic in the heterozygous state.MethodsWe studied a 36-year-old male index patient and his father, both presenting with progressive limb-girdle weakness. Muscle involvement was assessed by MRI and muscle biopsies. We performed whole-exome sequencing and Sanger sequencing for segregation analysis of the identified p.Arg159His VCP mutation. To dissect biological disease signatures, we applied state-of-the-art quantitative proteomics on muscle tissue of the index case, his father, 3 additional patients with VCP-related myopathy, and 3 control individuals.ResultsThe index patient, homozygous for the known p.Arg159His mutation in VCP, manifested a typical VCP-related myopathy phenotype, although with a markedly high creatine kinase value and a relatively early disease onset, and Paget disease of bone. The father exhibited a myopathy phenotype and discrete parkinsonism, and multiple deceased family members on the maternal side of the pedigree displayed a dementia, parkinsonism, or myopathy phenotype. Bioinformatic analysis of quantitative proteomic data revealed the degenerative nature of the disease, with evidence suggesting selective failure of muscle regeneration and stress granule dyshomeostasis.ConclusionWe report a patient showing a multisystem proteinopathy due to a homozygous VCP mutation. The patient manifests a severe phenotype, yet fundamental disease characteristics are preserved. Proteomic findings provide further insights into VCP-related pathomechanisms.

scholarly journals Moyamoya vasculopathy – Patient demographics and characteristics in the Finnish population

2016 ◽  
Vol 12 (1) ◽  
pp. 90-95 ◽  
Author(s):  
Marika Saarela ◽  
Satu Mustanoja ◽  
Johanna Pekkola ◽  
Tiina Tyni ◽  
Juha Hernesniemi ◽  
...  
Keyword(s):  
Disease Onset ◽  
Clinic Visit ◽  
University Hospital ◽  
Genetic Profile ◽  
German Population ◽  
Patient Demographics ◽  
Presenting Symptoms ◽  
Female Predominance ◽  
The Mean ◽  
Revascularization Surgery

Background and purpose Moyamoya vasculopathy, a rare steno-occlusive progressive cerebrovascular disorder, has not been thoroughly studied in Caucasian populations. We established a registry of Finnish patients treated at the Helsinki University Hospital, to collect and report demographic and clinical data. Methods We collected data both retrospectively and prospectively from all the patients with a moyamoya vasculopathy referred to our hospital between January 1987 and December 2014. All patients underwent a neurological outpatient clinic visit. Results We diagnosed 61 patients (50 females, 10 children) with moyamoya vasculopathy. The mean age at the disease-onset was 31.5 ± 17.9 years. The two most common presenting symptoms were ischemic stroke (n = 31) and hemorrhage (n = 8). Forty-four percent underwent revascularization surgery, and 70% were prescribed antithrombotic treatment. Conclusions The results support in part the Western phenotype of the disease considering the later presentation and larger female predominance compared to the Asian moyamoya vasculopathy reports. However, the proportion of ischemic strokes and hemorrhagic strokes is closer to Japanese population than German population. The absence of familial cases points to a different genetic profile in the Finnish patients.

scholarly journals The Possibility of Contralateral Non-Sentinel Groin Node’s Metastasis in Early Primary Vulvar Cancer Women After Unilateral Sentinel Node’s Metastasis: A Single Center Evaluation in University Hospital of Düsseldorf

2020 ◽  
Author(s):  
Andreas Suhartoyo Winarno ◽  
Anne Mondal ◽  
Franca Christina Martignoni ◽  
Tanja Natascha Fehm ◽  
Monika Hampl
Keyword(s):  
Vulvar Cancer ◽  
University Hospital ◽  
Morbidity Rate ◽  
Current Guideline ◽  
Single Center ◽  
Standard Management ◽  
Increased Risk ◽  
Early Primary ◽  
The University ◽  
Current Guidelines

Abstract Background: Sentinel node biopsy (SLNB) technique in unifocal vulvar cancer (diameter of < 4cm) and unsuspicious groin lymph nodes, the morbidity rate of patients has significantly decreased all over the world. In contrast to SLNB, bilateral inguinofemoral lymphadenectomy (IFL) has been associated with increased risk of common morbidities. Current guidelines (NCCN, ESGO, RCOG, and German) suggest that in cases of metastasis of unilateral SLNB, groin node dissection with IFL, should be performed bilaterally. However, a publication by Woelber et al. 0% (p=0/28) and Nica et al. 5.3% (p=1/19) contradicted the current guideline. Methods: A single-center analysis conducted in the University Hospital of Dusseldorf, evaluating vulvar cancer patients treated with SLNB retrospectively from 2002 to 2018. Result, discussion and conclusion: Current guideline for bilateral IFL should remain as the standard management because 22.2% women (n=4/18) had contralateral IFL groin metastasis after unilateral SLNB metastasis initially. The depth of tumor infiltrating cells was correlated significantly and positively with the incidence rate of groin metastasis (p=0.0038). Therefore, it is an indication for bilateral IFL.

scholarly journals Evaluation of the prognostic benefit of identifying the probable primary site in cancer of unknown primary

2015 ◽  
Vol 6 (3) ◽  
pp. 22-28
Author(s):  
Joyutpal Das ◽  
Shahid Gilani
Keyword(s):  
Median Survival ◽  
Cox Regression ◽  
Performance Status ◽  
Disease Onset ◽  
Significant Proportion ◽  
Poor Performance ◽  
Primary Site ◽  
University Hospital ◽  
Unknown Primary ◽  
Poor Performance Status

Abstract With the development of site-specific cancer therapy, identifying the primary origin allows the oncologist to personalise therapy for patients with the cancer of unknown primaries (CUPs). At present, immunohistochemistry (IHC) screening is the standard method used to postulate the primary site in CUP. In this retrospective study, we evaluated the prognostic benefit of identifying the primary site in CUP. All 84 patients who presented with suspected CUP to the Royal Stoke University Hospital between 2011 and 2012 were included in our study. Forty-eight percent (40/84) of these patients were unable to undergo necessary investigations to identify primary sites because of poor performance status. IHC screening was able to postulate the primary site in 59% (26/44) of the remaining patients with confirmed CUP. Therefore, the primary site was not identified in a significant proportion of patients with CUP. The median survival of confirmed CUP with probable primary site was 2.0 months (95% confidence interval (CI): 1.2 to 2.9 months), whereas the median survival of confirmed CUP with no probable primary site was 4.1 months (95% CI: 1.5 to 9.7 months). This difference in survival time was statistically significant. In addition, using the Cox regression model, we found that patients with confirmed CUP with primary sites had prognostically unfavourable diseases with a shorter median survival, regardless of the age of disease onset, gender, sites of metastases or number of metastases. One approach to improve the survival would be to start systemic therapy at the earliest possible opportunity rather than waiting for all investigation results, such as IHC.

External validation of a radiomic signature to predict HPV (p16) status from standard CT images of anal and vulvar cancer patients.

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. e15502-e15502
Author(s):  
Ralph T.H. Leijenaar ◽  
Sean Walsh ◽  
Akshayaa Vaidyanathan ◽  
Fadila Zerka ◽  
Mariaelena Occhipinti ◽  
...  
Keyword(s):  
Lung Cancer ◽  
Cancer Patients ◽  
Vulvar Cancer ◽  
External Validation ◽  
Ct Images ◽  
University Hospital ◽  
Control Cohort ◽  
Lung Cancer Patients ◽  
Computed Tomography Images ◽  
Hpv Status

e15502 Background: HPV status of anal and vulvar cancers cannot be predicted by visual inspection as well as for oropharyngeal cancers. Radiomics applied on computed tomography images can extract features that may better characterize the structure and the underlying biology of the tumor. Methods: In this multi-center study, we validated a CT based radiomic signature to predict HPV (p16) status, developed in head & neck cancer, in anal and vulvar cancer patients. The patients cohort was composed of 68 anal cancer patients and 7 vulvar cancer patients, with p16 status determined by immunohistochemistry, while a control cohort was composed of 422 lung cancer patients. The patient cohorts come from 4 different centers (Maastro Clinic - the Netherlands, CHU Liege – Belgium, St Luke’s Hospital – Ireland, Cork University Hospital - Ireland). The primary tumor volume was manually delineated for each patient on axial CT images. Prior to analysis, all images were resampled to isotropic voxels of 2 mm, using linear interpolation. A total of 37 radiomics features were calculated from five groups: tumor intensity, shape, texture, Wavelet and Laplacian of Gaussian. The signature was built using regularized logistic regression [1]. The signature was evaluated according to the Transparent Reporting of a multivariable prediction model for Individual Prognosis or Diagnosis (TRIPOD) and the Radiomics Quality Score (RQS). Results: The signature classified anal and vulvar cancers based on their HPV status (positive or negative), with an AUROC of 0.760 comparable to the performance of the original signature developed in oropharyngeal squamous cell carcinomas (AUROC of 0.764) [1]. The model, tested in the control cohort of lung cancer patients, predicted the HPV positive status of 1% of the patients which is in line with expected European prevalence (0 – 10%). This signature is TRIPOD level 4 (57%) with an RQS of 61%. Conclusions: This study supplies an additional insight into HPV imaging phenotype, providing a proof of concept that molecular information can be inferred from standard medical images by means of radiomics. These preliminary but encouraging results may pave the road for further generalization of CT image features of HPV-related tumors and aid in the optimization of future therapy developments [2]. Reference [1] Ralph TH Leijenaar et al., The British Journal of Radiology 2018 91:1086 [2] Immunotherapy Drug with Two Targets Shows Promise against HPV-Related Cancers - accessed on 12/02/2021 - https://www.cancer.gov/

P036 Juvenile Idiopathic Arthritis in adulthood: outcomes of oligo and polyarticular forms?

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
Miladi Saoussen ◽  
Makhlouf Yasmine ◽  
Fazaa Alia ◽  
Sellami Mariem ◽  
Ouenniche Kmar ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Transition To Adulthood ◽  
Disease Onset ◽  
Adult Life ◽  
Treatment Modalities ◽  
Disease Characteristics ◽  
Female Predominance ◽  
Tunnel Syndrome ◽  
The Mean ◽  
International League

Abstract Background Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory rheumatic disease of childhood that can cause major physical disability, impairing patients' ability to lead a normal adult life. The objective of this study was to assess the outcomes of oligo- and polyarticular JIA in adulthood as well as the progression to authentic rheumatoid arthritis (RA). Methods We conducted a retrospective study including adult patients with JIA (diagnosed according to the International League of Associations for Rheumatology [ILAR]). Data collected included age, gender, disease characteristics (JIA subtype, disease duration). The progression to an authentic RA was determined by checking the response to the ACR/EULAR 2010 classification criteria. Results Twenty-eight patients were included in this study. There was a female predominance with a sex ratio of 1.5. The mean age of disease onset was 6 years and 2 months [2–17]. The mean age at inclusion was 29.5 years [18–64]. The frequency of each JIA subtype was as follows: polyarticular with RF + (n = 14), polyarticular with RF- (n = 9), oligoarthritis (n = 5). The polyarticular forms: RF+ and RF- evolved into genuine seropositive and seronegative RA in 71.4% and 66.7% of cases, respectively. Among the oligoarticular JIA subtype, an extension of the disease to a seronegative RA was noted in one patient (20%). Hip involvement was noted in 20% of patients. Carpal tunnel syndrome and elbow arthritis were found in 28.5% and 14.3% of patients, respectively. Regarding treatment modalities and at the time of recruitment, 36.3% of RAs were treated with Methotrexate, and 33% were on biological treatment. Ten percent of the patients dropped out of school because of disease flares and deformities. Conclusion Our study showed oligo- and polyarticular JIA evolves in most of the cases towards an authentic RA causing deformities and handicaps. Earlier management is necessary to allow a better transition to adulthood.

scholarly journals Cross-sectional quantitative analysis of the natural history of TUBA1A and TUBB2B tubulinopathies

2020 ◽  
Author(s):  
Julian Schröter ◽  
Jan H. Döring ◽  
Sven F. Garbade ◽  
Georg F. Hoffmann ◽  
Stefan Kölker ◽  
...  
Keyword(s):  
Natural History ◽  
Disease Onset ◽  
Diagnostic Delay ◽  
Family Counseling ◽  
Cross Sectional ◽  
Clinical Endpoints ◽  
Therapeutic Trials ◽  
Disease Characteristics ◽  
Brain Malformations ◽  
History Of

Abstract Purpose TUBA1A and TUBB2B tubulinopathies are rare neurodevelopmental disorders characterized by cortical and extracortical malformations and heterogenic phenotypes. There is a need for quantitative clinical endpoints that will be beneficial for future diagnostic and therapeutic trials. Methods Quantitative natural history modeling of individuals with TUBA1A and TUBB2B tubulinopathies from clinical reports and database entries of DECIPHER and ClinVar. Main outcome measures were age at disease onset, survival, and diagnostic delay. Phenotypical, neuroradiological, and histopathological features were descriptively illustrated. Results Mean age at disease onset was 4 (TUBA1A) and 6 months (TUBB2B), respectively. Mortality was equally estimated with 7% at 3.2 (TUBA1A) and 8.0 years (TUBB2B). Diagnostic delay was significantly higher in TUBB2B (12.3 years) compared with TUBA1A tubulinopathy (4.2 years). We delineated the isotype-dependent clinical, neuroradiological, and histopathological phenotype of affected individuals and present brain malformations associated with epilepsy and an unfavorable course of disease. Conclusion The natural history of tubulinopathies is defined by the genotype and associated brain malformations. Defined data on estimated survival, diagnostic delay, and disease characteristics of TUBA1A and TUBB2B tubulinopathy will help to raise disease awareness and encourage future clinical trials to optimize genetic testing, family counseling, and supportive care.

scholarly journals Associated Lichen Sclerosis Increases the Risk of Lymph Node Metastases of Vulvar Cancer

2020 ◽  
Vol 9 (1) ◽  
pp. 250
Author(s):  
Yohann Dabi ◽  
Marie Gosset ◽  
Sylvie Bastuji-Garin ◽  
Rana Mitri-Frangieh ◽  
Sofiane Bendifallah ◽  
...  
Keyword(s):  
Lymph Node ◽  
Tumor Size ◽  
Vulvar Cancer ◽  
Univariate Analysis ◽  
Lymph Node Involvement ◽  
Lymph Node Status ◽  
Local Extension ◽  
Lichen Sclerosis ◽  
Node Involvement ◽  
The Impact

The most important prognostic factor in vulvar cancer is inguinal lymph node status at the time of diagnosis, even in locally advanced vulvar tumors. The aim of our study was to identify the risk factors of lymph node involvement in these women, especially the impact of lichen sclerosis (LS). We conducted a retrospective population-based cross-sectional study in two French referral gynecologic oncology institutions. We included all women diagnosed with a primary invasive vulvar cancer. Epithelial alteration adjacent to the invasive carcinoma was found in 96.8% (n = 395). The most frequently associated was LS in 27.7% (n = 113). In univariate analysis, LS (p = 0.009); usual type VIN (p = 0.04); tumor size >2 cm and/or local extension to vagina, urethra or anus (p < 0.01), positive margins (p < 0.01), thickness (p < 0.01) and lymphovascular space invasion (LVSI) (p < 0.01) were significantly associated with lymph node involvement. In multivariate analysis, only LS (OR 2.3, 95% CI [1.2–4.3]) and LVSI (OR 5.6, 95% CI [1.7–18.6]) remained significantly associated with positive lymph node. LS was significantly associated with older patients (p = 0.005), anterior localization (p = 0.017) and local extension (tumor size > 2 cm: p = 0.001). LS surrounding vulvar cancer is an independent factor of lymph node involvement, with local extension and LVSI.

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