scholarly journals Presentation and management of pheochromocytomas and paragangliomas: about 40 cases

2021 ◽  
Vol 27 (1) ◽  
Author(s):  
Faten Hadj kacem ◽  
Ameni Salah ◽  
Bilel Fathallah ◽  
Khouloud Boujelben ◽  
Nadia Charfi ◽  
...  
Keyword(s):  
Genetic Diseases ◽  
Adrenal Incidentaloma ◽  
Population Based ◽  
Clinical Aspects ◽  
Magnetic Resonance Imaging Scan ◽  
Spss Software ◽  
Plasma Free Metanephrines ◽  
Evolutionary Aspects ◽  
Chromaffin Tissue

Abstract Background Pheochromocytoma and paraganglioma are rare neuroendocrine tumors of the chromaffin tissue, which may produce catecholamines. The aim of our study was to analyze the clinical and para-clinical aspects as well as the therapeutic and evolutionary aspects of pheocromocytomas and paragangliomas based on a series of 40 cases. Methods Our retrospective population-based research study includes 40 patients. Then, a statistical analysis was carried out using the SPSS software (version21). Results Our study involves 40 patients, including 23 women (57, 5%) and 17 men (42,5%). The mean age at the time of the diagnosis was 43.8 ± 16.8 years. The circumstances of the discovery were mainly characterized by adrenal incidentaloma and hypertension. The biological diagnosis was based on the dosage of urinary metanephrines and plasma-free metanephrines in, respectively, 61.5% and 18% of cases. A computerized tomography scan and/or a magnetic resonance imaging scan could help to locate the tumor in 100% of cases. Our series includes 3 cases of bilateral pheochromocytoma, 3 cases of paragangliomas and 1 case of malignant pheochromocytoma, while a hereditary form was retained in 3 patients. In fact, thirty-two patients were operated; cure was clinically labeled in 100% and biologically in 87.5% of patients. Conclusions The main points for improvement that our study has revealed are; a patient follow-up after surgery, which was not always regular, and an insufficient screening for genetic diseases associated with pheochromocytomas and paragangliomas.

scholarly journals Asymptomatic and Biochemically Silent Pheochromocytoma with Characteristic Findings on Imaging

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Andrew Spiro ◽  
Aqueel Usman ◽  
Asif Ajmal ◽  
Thanh D. Hoang ◽  
Mohamed K. M. Shakir
Keyword(s):  
Laparoscopic Resection ◽  
Adrenal Incidentaloma ◽  
Surgical Removal ◽  
Laboratory Studies ◽  
Mri Findings ◽  
Tumor Surveillance ◽  
Asymptomatic Patients ◽  
Ct And Mri ◽  
Chromaffin Tissue

Pheochromocytomas are tumors that originate from the chromaffin tissue of the adrenal medulla and commonly produce catecholamines. The diagnosis is typically established by the measurement of catecholamines or their metabolites in urine or plasma, and tumors are localized with the use of radiographic and scintigraphic studies. Pheochromocytomas can occur in asymptomatic patients, and the preferred treatment is surgical removal of the tumor. We report a 48-year-old male with a left adrenal incidentaloma, which progressively increased in size from 1.1 cm to 2.6 cm over a 4-year period, as measured by an adrenal computed tomography (CT) scan. Throughout his entire course of treatment, he was asymptomatic with normal blood pressure readings. His biochemical screening was unremarkable for the first three years of tumor surveillance. Follow-up imaging, including CT and MRI, showed findings suspicious for pheochromocytoma, and the diagnosis was ultimately made with the combination of imaging and laboratory studies. He underwent laparoscopic resection of the adrenal mass with confirmation of pheochromocytoma on histology. This case illustrates how CT and MRI findings can alert providers to the presence of a pheochromocytoma, even in an asymptomatic, biochemically negative patient.

scholarly journals Clinical aspects of SDHx-related pheochromocytoma and paraganglioma

2009 ◽  
Vol 16 (2) ◽  
pp. 391-400 ◽  
Author(s):  
Henri J L M Timmers ◽  
Anne-Paule Gimenez-Roqueplo ◽  
Massimo Mannelli ◽  
Karel Pacak
Keyword(s):  
Head And Neck ◽  
Susceptibility Gene ◽  
Clinical Aspects ◽  
Apoptosis Resistance ◽  
Neuronal Precursor Cells ◽  
Positron Emission ◽  
Genes Encoding ◽  
Head And Neck Paragangliomas ◽  
Chromaffin Tissue

Paragangliomas (PGLs) derive from either sympathetic chromaffin tissue in adrenal and extra-adrenal abdominal or thoracic locations, or from parasympathetic tissue of the head and neck. Mutations of nuclear genes encoding subunits B, C, and D of the mitochondrial enzyme succinate dehydrogenase (SDHB 1p35-p36.1, SDHC 1q21, SDHD 11q23) give rise to hereditary PGL syndromes PGL4, PGL3, and PGL1 respectively. The susceptibility gene for PGL2 on 11q13.1 remains unidentified. Mitochondrial dysfunction due to SDHx mutations have been linked to tumorigenesis by upregulation of hypoxic and angiogenesis pathways, apoptosis resistance and developmental culling of neuronal precursor cells. SDHB-, SDHC-, and SDHD-associated PGLs give rise to more or less distinct clinical phenotypes. SDHB mutations mainly predispose to extra-adrenal, and to a lesser extent, adrenal PGLs, with a high malignant potential, but also head and neck paragangliomas (HNPGL). SDHD mutations are typically associated with multifocal HNPGL and usually benign adrenal and extra-adrenal PGLs. SDHC mutations are a rare cause of mainly HNPGL. Most abdominal and thoracic SDHB-PGLs hypersecrete either norepinephrine or norepinephrine and dopamine. However, only some hypersecrete dopamine, are biochemically silent. The biochemical phenotype of SDHD-PGL has not been systematically studied. For the localization of PGL, several positron emission tomography (PET) tracers are available. Metastatic SDHB-PGL is the best localized by [18F]-fluorodeoxyglucose PET. The identification of SDHx mutations in patients with PGL is warranted for a tailor-made approach to the biochemical diagnosis, imaging, treatment, follow-up, and family screening.

No relation between biomarkers at age 47–49 and aortic diameter after 14–19 years of follow-up – a population-based study

VASA ◽  
2017 ◽  
Vol 46 (4) ◽  
pp. 291-295 ◽  
Author(s):  
Soumia Taimour ◽  
Moncef Zarrouk ◽  
Jan Holst ◽  
Olle Melander ◽  
Gunar Engström ◽  
...  
Keyword(s):  
Population Based ◽  
Aortic Diameter ◽  
Aortic Dilatation ◽  
Population Based Study ◽  
Reactive Protein ◽  
Abdominal Aortic ◽  
Long Term Follow Up ◽  
Old Men

Abstract. Background: Biomarkers reflecting diverse pathophysiological pathways may play an important role in the pathogenesis of abdominal aortic aneurysm (aortic diameter ≥30 mm, AAA), levels of many biomarkers are elevated and correlated to aortic diameter among 65-year-old men undergoing ultrasound (US) screening for AAA. Probands and methods: To evaluate potential relationships between biomarkers and aortic dilatation after long-term follow-up, levels of C-reactive protein (CRP), proneurotensin (PNT), copeptin (CPT), lipoprotein-associated phospholipase 2 (Lp-PLA2), cystatin C (Cyst C), midregional proatrial natriuretic peptide (MR-proANP), and midregional proadrenomedullin (MR-proADM) were measured in 117 subjects (114 [97 %] men) aged 47–49 in a prospective population-based cohort study, and related to aortic diameter at US examination of the aorta after 14–19 years of follow-up. Results: Biomarker levels at baseline did not correlate with aortic diameter after 14–19 years of follow up (CRP [r = 0.153], PNT [r = 0.070], CPT [r = –.156], Lp-PLA2 [r = .024], Cyst C [r = –.015], MR-proANP [r = 0.014], MR-proADM [r = –.117]). Adjusting for age and smoking at baseline in a linear regression model did not reveal any significant correlations. Conclusions: Tested biomarker levels at age 47–49 were not associated with aortic diameter at ultrasound examination after 14–19 years of follow-up. If there are relationships between these biomarkers and aortic dilatation, they are not relevant until closer to AAA diagnosis.

Does Hospitalization Predict the Disease Course in Ulcerative Colitis? Prevalence and Predictors of Hospitalization and Re- Hospitalization in Ulcerative Colitis in a Population-based Inception Cohort (2000-2012)

2015 ◽  
Vol 24 (3) ◽  
pp. 287-292 ◽  
Author(s):  
Petra A. Golovics ◽  
Laszlo Lakatos ◽  
Michael D. Mandel ◽  
Barbara D. Lovasz ◽  
Zsuzsanna Vegh ◽  
...  
Keyword(s):  
Ulcerative Colitis ◽  
Cox Regression ◽  
Diagnostic Procedures ◽  
Population Based ◽  
Disease Course ◽  
Inception Cohort ◽  
Disease Extent ◽  
Cox Regression Analysis ◽  
Hospitalization Rates

Background & Aims: Limited data are available on the hospitalization rates in population-based studies. Since this is a very important outcome measure, the aim of this study was to analyze prospectively if early hospitalization is associated with the later disease course as well as to determine the prevalence and predictors of hospitalization and re-hospitalization in the population-based ulcerative colitis (UC) inception cohort in the Veszprem province database between 2000 and 2012. Methods: Data of 347 incident UC patients diagnosed between January 1, 2000 and December 31, 2010 were analyzed (M/F: 200/147, median age at diagnosis: 36, IQR: 26-50 years, follow-up duration: 7, IQR 4-10 years). Both in- and outpatient records were collected and comprehensively reviewed. Results: Probabilities of first UC-related hospitalization were 28.6%, 53.7% and 66.2% and of first re-hospitalization were 23.7%, 55.8% and 74.6% after 1-, 5- and 10- years of follow-up, respectively. Main UC-related causes for first hospitalization were diagnostic procedures (26.7%), disease activity (22.4%) or UC-related surgery (4.8%), but a significant percentage was unrelated to IBD (44.8%). In Kaplan-Meier and Cox-regression analysis disease extent at diagnosis (HR extensive: 1.79, p=0.02) or at last follow-up (HR: 1.56, p=0.001), need for steroids (HR: 1.98, p<0.001), azathioprine (HR: 1.55, p=0.038) and anti-TNF (HR: 2.28, p<0.001) were associated with the risk of UC-related hospitalization. Early hospitalization was not associated with a specific disease phenotype or outcome; however, 46.2% of all colectomies were performed in the year of diagnosis. Conclusion: Hospitalization and re-hospitalization rates were relatively high in this population-based UC cohort. Early hospitalization was not predictive for the later disease course.

scholarly journals Association of Tooth Scaling with Acute Myocardial Infarction and Analysis of the Corresponding Medical Expenditure: A Nationwide Population-Based Study

2021 ◽  
Vol 18 (14) ◽  
pp. 7613
Author(s):  
Yi-Wei Kao ◽  
Ben-Chang Shia ◽  
Huei-Chen Chiang ◽  
Mingchih Chen ◽  
Szu-Yuan Wu
Keyword(s):  
Myocardial Infarction ◽  
Acute Myocardial Infarction ◽  
Periodontal Diseases ◽  
Population Based ◽  
Medical Expenditure ◽  
Control Group ◽  
Proportional Hazard ◽  
Population Based Study ◽  
Scaling Group

Accumulating evidence has shown a significant correlation between periodontal diseases and systemic diseases. In this study, we investigated the association between the frequency of tooth scaling and acute myocardial infarction (AMI). Here, a group of 7164 participants who underwent tooth scaling was compared with another group of 7164 participants without tooth scaling through propensity score matching to assess AMI risk by Cox’s proportional hazard regression. The results show that the hazard ratio of AMI from the tooth scaling group was 0.543 (0.441, 0.670) and the average expenses of AMI in the follow up period was USD 265.76, while the average expenses of AMI in follow up period for control group was USD 292.47. The tooth scaling group was further divided into two subgroups, namely A and B, to check the influence of tooth scaling frequency on AMI risk. We observed that (1) the incidence rate of AMI in the group without any tooth scaling was 3.5%, which is significantly higher than the incidence of 1.9% in the group with tooth scaling; (2) the tooth scaling group had lower total medical expenditures than those of the other group because of the high medical expenditure associated with AMI; and (3) participants who underwent tooth scaling had a lower AMI risk than those who never underwent tooth scaling had. Therefore, the results of this study demonstrate the importance of preventive medicine.

scholarly journals Associations of Dietary Patterns with Incident Depression: The Maastricht Study

Nutrients ◽  
2021 ◽  
Vol 13 (3) ◽  
pp. 1034
Author(s):  
Vincenza Gianfredi ◽  
Annemarie Koster ◽  
Anna Odone ◽  
Andrea Amerio ◽  
Carlo Signorelli ◽  
...  
Keyword(s):  
Depressive Symptoms ◽  
Dietary Patterns ◽  
Proportional Hazards ◽  
A Priori ◽  
Preventive Measure ◽  
Population Based ◽  
Cox Proportional Hazards ◽  
Healthy Diet ◽  
Patient Health

Our aim was to assess the association between a priori defined dietary patterns and incident depressive symptoms. We used data from The Maastricht Study, a population-based cohort study (n = 2646, mean (SD) age 59.9 (8.0) years, 49.5% women; 15,188 person-years of follow-up). Level of adherence to the Dutch Healthy Diet (DHD), Mediterranean Diet, and Dietary Approaches To Stop Hypertension (DASH) were derived from a validated Food Frequency Questionnaire. Depressive symptoms were assessed at baseline and annually over seven-year-follow-up (using the 9-item Patient Health Questionnaire). We used Cox proportional hazards regression analyses to assess the association between dietary patterns and depressive symptoms. One standard deviation (SD) higher adherence in the DHD and DASH was associated with a lower hazard ratio (HR) of depressive symptoms with HRs (95%CI) of 0.78 (0.69–0.89) and 0.87 (0.77–0.98), respectively, after adjustment for sociodemographic and cardiovascular risk factors. After further adjustment for lifestyle factors, the HR per one SD higher DHD was 0.83 (0.73–0.96), whereas adherence to Mediterranean and DASH diets was not associated with incident depressive symptoms. Higher adherence to the DHD lowered risk of incident depressive symptoms. Adherence to healthy diet could be an effective non-pharmacological preventive measure to reduce the incidence of depression.

scholarly journals Lymphoproliferative malignancies in patients with neurofibromatosis 1

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Christina Bergqvist ◽  
François Hemery ◽  
Arnaud Jannic ◽  
Salah Ferkal ◽  
Pierre Wolkenstein
Keyword(s):  
General Population ◽  
Medical History ◽  
Hodgkin Lymphoma ◽  
Incidence Rate ◽  
Population Based ◽  
Neurofibromatosis 1 ◽  
Population Based Study ◽  
Non Hodgkin Lymphoma ◽  
History Of

AbstractNeurofibromatosis 1 (NF1) is an inherited, autosomal-dominant, tumor predisposition syndrome with a birth incidence as high as 1:2000. A patient with NF1 is four to five times more likely to develop a malignancy as compared to the general population. The number of epidemiologic studies on lymphoproliferative malignancies in patients with NF1 is limited. The aim of this study was to determine the incidence rate of lymphoproliferative malignancies (lymphoma and leukemia) in NF1 patients followed in our referral center for neurofibromatoses. We used the Informatics for Integrated Biology and the Bedside (i2b2) platform to extract information from the hospital’s electronic health records. We performed a keyword search on clinical notes generated between Jan/01/2014 and May/11/2020 for patients aged 18 years or older. A total of 1507 patients with confirmed NF1 patients aged 18 years and above were identified (mean age 39.2 years; 57% women). The total number of person-years in follow-up was 57,736 (men, 24,327 years; women, 33,409 years). Mean length of follow-up was 38.3 years (median, 36 years). A total of 13 patients had a medical history of either lymphoma or leukemia, yielding an overall incidence rate of 22.5 per 100,000 (0.000225, 95% confidence interval (CI) 0.000223–0.000227). This incidence is similar to that of the general population in France (standardized incidence ratio 1.07, 95% CI 0.60–1.79). Four patients had a medical history leukemia and 9 patients had a medical history of lymphoma of which 7 had non-Hodgkin lymphoma, and 2 had Hodgkin lymphoma. Our results show that adults with NF1 do not have an increased tendency to develop lymphoproliferative malignancies, in contrast to the general increased risk of malignancy. While our results are consistent with the recent population-based study in Finland, they are in contrast with the larger population-based study in England whereby NF1 individuals were found to be 3 times more likely to develop both non-Hodgkin lymphoma and lymphocytic leukemia. Large-scale epidemiological studies based on nationwide data sets are thus needed to confirm our findings.

Anorectal Malformations and the Risk of Colorectal Cancer—Is Early Routine Endoscopic Screening Indicated?

2020 ◽  
Author(s):  
Anna Svenningsson ◽  
Anna Gunnarsdottir ◽  
Tomas Wester
Keyword(s):  
Colorectal Cancer ◽  
Early Adulthood ◽  
Anorectal Malformations ◽  
Population Based ◽  
Observational Period ◽  
Endoscopic Screening ◽  
Medical Birth Register ◽  
National Patient ◽  
And Gender

Abstract Introduction Colorectal cancer (CRC) has been reported in early adulthood in patients with anorectal malformation (ARM), and therefore, the need of endoscopic controls has been discussed. The aim of this study was to assess the risk of CRC in patients with ARM. Materials and Methods This was a nationwide population-based study with data from Swedish national health care registers. All patients diagnosed with ARM born in Sweden between 1964 and 1999 were identified in the National Patient Register. The same group was followed up in the Swedish Cancer Register from birth to December 31, 2014, for occurrences of CRC. Five age- and gender-matched individuals randomly selected from the Medical Birth Register served as controls for each ARM patient born between 1973 and 1999. Results A total of 817 patients (474 males) with ARM were included and followed up from birth to the end of observational period. Time of follow-up ranged from 15 to 50 years (mean: 28 years). None of the patients was diagnosed with CRC during the observational period. One case of rectal cancer and one case of sigmoid cancer were detected among the 3,760 controls. Conclusion In our study, the risk of CRC in early adulthood in patients with ARM is low. Our result does not support routine endoscopic follow-up for patients with ARM during the first decade of life.

Sign in / Sign up

Export Citation Format

Share Document