Sarcomatous transformation of clival chordoma after charged-particle radiotherapy

2006 ◽  
Vol 105 (1) ◽  
pp. 136-141 ◽  
Author(s):  
Takayuki Hara ◽  
Nobutaka Kawahara ◽  
Koji Tsuboi ◽  
Junji Shibahara ◽  
Tetsuo Ushiku ◽  
...  
Keyword(s):  
Charged Particle ◽  
Recurrent Tumor ◽  
Sarcomatous Component ◽  
Imaging Studies ◽  
Histological Subtypes ◽  
Particle Radiotherapy ◽  
Long Term Prognosis ◽  
Skull Base Chordomas

✓ Skull base chordomas containing a sarcomatous component are extremely rare. Here the authors report two new cases in which a recurrent tumor with a sarcomatous component appeared after the patient had undergone charged-particle radiotherapy. Histological examinations performed in Case 1 revealed some retention of epithelial features in the sarcomatous component, whereas no such regions were observed in Case 2. Both patients had rapidly deteriorating clinical courses and died within 6 months after diagnosis of the recurrent tumor. The authors discuss the significance of the histological subtypes of these tumors for long-term prognosis and their pathogenetic mechanisms in relation to radiotherapy. Although these sarcomatous transformations are rare in conventional chordomas, a careful histological examination and thorough follow-up imaging studies are crucial when treating patients with such lesions.

scholarly journals Prevalence and Prognostic Significance of Left Ventricular Dysfunction in Patients Presenting Acutely with Atrial Fibrillation

2010 ◽  
Vol 4 ◽  
pp. CMC.S4106 ◽  
Author(s):  
Chin Lin ◽  
Colin Edwards ◽  
Guy P. Armstrong ◽  
Anthony Scott ◽  
Hitesh Patel ◽  
...  
Keyword(s):  
Systolic Dysfunction ◽  
Management Strategies ◽  
Newly Diagnosed ◽  
Imaging Studies ◽  
Long Term Outcomes ◽  
Prognostic Importance ◽  
Long Term Prognosis ◽  
Repeat Imaging

The prevalence and prognostic importance of CM occurring as a consequence of AF is poorly defined. This study investigated the incidence of CM in patients with AF, its clinical features and long-term outcomes. We demonstrated that CM is common in patients presenting acutely with newly diagnosed rapid AF, and carries a worse long-term prognosis. Systolic dysfunction was reversible in an important proportion of patients, suggesting a greater prevalence of rate-related CM in AF than has previously been postulated. This underscores the importance of appropriate rhythm management strategies and repeat imaging studies. Background Atrial fibrillation (AF) may precipitate LV dysfunction, potentially leading to cardiomyopathy (CM). The prevalence and prognostic importance of CM occurring as a consequence of AF is poorly defined. We investigated the incidence of CM in patients with AF, its clinical features and long-term outcomes. Methods We reviewed 292 consecutive patients (average age 72 ± 13yrs) presenting acutely with AF and tachycardia over a 3 year period from June 2004. Clinical details were obtained from medical records. CM was defined as ejection fraction (EF) ≤ 50% on index admission. Results Echo was performed 93% of patients at index admission, and 69 (24%) had CM (average EF% = 37 ± 11), 60 of which were newly diagnosed. Patients with CM had significantly higher presenting heart rate (141 ± 19 vs. 132 ± 23 bpm), larger end-diastolic (5.7 vs. 5.2 cm) and end-systolic (4.5 vs. 3.2 cm) dimensions, and larger left atrial size (4.6 vs. 4.3 cm) ( P < 0.05 for all). They were also statistically more likely ( P < 0.05) to be male, present with breathlessness, have a history of coronary disease, and be treated with digoxin and warfarin. Follow-up echo between 6 and 12 months was performed in 46% of patients with new CM, and average EF rose to 53 ± 12%. At an average follow-up of 2.5 years, there was a significant increase in mortality in CM patients (16% vs. 9.5%, P < 0.05). Conclusion CM is common in patients presenting acutely with newly diagnosed rapid AF, and carries a worse long-term prognosis. Systolic dysfunction was reversible in an important proportion of patients, suggesting a greater prevalence of rate-related CM in AF than has previously been postulated. This underscores the importance of appropriate rhythm management strategies and repeat imaging studies.

scholarly journals Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Jeong-Seon Lee ◽  
Joong-Gon Kim ◽  
Soyoung Lee
Keyword(s):  
Polyarteritis Nodosa ◽  
Clinical Manifestations ◽  
Long Term Outcomes ◽  
Childhood Onset ◽  
Laboratory Findings ◽  
Clinical Presentations ◽  
Long Term Prognosis ◽  
Digital Amputation

AbstractChildhood-onset polyarteritis nodosa (PAN) is a rare and systemic necrotising vasculitis in children affecting small- to medium-sized arteries. To date, there have been only a few reports because of its rarity. Thus, we aimed to investigate the clinical manifestations, laboratory findings, treatment, and long-term outcomes in patients with childhood-onset PAN and to evaluate the usefulness of the paediatric vasculitis activity score (PVAS). We retrospectively analysed the data of nine patients with childhood-onset PAN from March 2003 to February 2020. The median ages at symptom onset, diagnosis, and follow-up duration were 7.6 (3–17.5), 7.7 (3.5–17.6), and 7.0 (1.6–16.3) years, respectively. All patients had constitutional symptoms and skin manifestations, while five exhibited Raynaud’s phenomenon. Organ involvement was observed in one patient. The median PVAS at diagnosis was 7 (range: 2–32). Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived, but three patients with high PVAS at diagnosis experienced irreversible sequelae, including intracranial haemorrhage and digital amputation. In conclusion, early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. This study suggests that high PVAS score at diagnosis may be associated with poor prognosis.

scholarly journals Comparison of Short- and Long-Term Prognosis between ST-Elevation and Non-ST-Elevation Myocardial Infarction

2021 ◽  
Vol 10 (2) ◽  
pp. 180
Author(s):  
Frédéric Bouisset ◽  
Jean-Bernard Ruidavets ◽  
Jean Dallongeville ◽  
Marie Moitry ◽  
Michele Montaye ◽  
...  
Keyword(s):  
Myocardial Infarction ◽  
Mortality Rate ◽  
Confounding Factors ◽  
Coronary Syndrome ◽  
Short And Long Term ◽  
St Elevation ◽  
Probability Of Death ◽  
Long Term Prognosis

Background: Available data comparing long-term prognosis according to the type of acute coronary syndrome (ACS) are scarce, contradictory, and outdated. Our aim was to compare short- and long-term mortality in ST-elevated (STEMI) and non-ST-elevated myocardial infarction (non-STEMI) ACS patients. Methods: Patients presenting with an inaugural ACS during the year 2006 and living in one of the three areas in France covered by the Monitoring of Trends and Determinants in Cardiovascular Disease (MONICA) registry were included. Results: A total of 1822 patients with a first ACS—1121 (61.5%) STEMI and 701 (38.5%) non-STEMI—were included in the study. At the 28-day follow-up, the mortality rates were 6.7% and 4.7% (p = 0.09) for STEMI and non-STEMI patients, respectively, and after adjustment of potential confounding factors, the 28-day probability of death was significantly lower for non-STEMI ACS patients (Odds Ratio = 0.58 (0.36–0.94), p = 0.03). At the 10-year follow-up, the death rates were 19.6% and 22.8% (p = 0.11) for STEMI and non-STEMI patients, respectively, and after adjustment of potential confounding factors, the 10-year probability of death did not significantly differ between non-STEMI and STEMI events (OR = 1.07 (0.83–1.38), p = 0.59). Over the first year, the mortality rate was 7.2%; it then decreased and stabilized at 1.7% per year between the 2nd and 10th year following ACS. Conclusion: STEMI patients have a worse vital prognosis than non-STEMI patients within 28 days following ACS. However, at the 10-year follow-up, STEMI and non-STEMI patients have a similar vital prognosis. From the 2nd year onwards following the occurrence of a first ACS, the patients become stable coronary artery disease patients with an annual mortality rate in the 2% range, regardless of the type of ACS they initially present with.

scholarly journals Aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder with recurrent short partial transverse myelitis and favorable prognosis: Two new cases

2017 ◽  
Vol 23 (14) ◽  
pp. 1950-1954 ◽  
Author(s):  
Jinhua Zhang ◽  
Fang Liu ◽  
Yiqi Wang ◽  
Ying Yang ◽  
Yuehong Huang ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Acute Attack ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Attack Phase ◽  
Long Term Prognosis

Understanding the characteristics of neuromyelitis optica spectrum disorder (NMOSD) with recurrent short partial transverse myelitis (SPTM), which is very rare, contributes to the differential diagnosis of multiple sclerosis (MS). We present two Chinese aquaporin-4 immunoglobulin G (AQP4-IgG)-seropositive NMOSD cases who had at least twice SPTM during 4 and 6 years of follow-up, respectively. Their SPTMs have been mild and responded well to corticosteroids just like in the case of MS. The findings highlight the need of searching for serum AQP4-IgG (cell-based assay strongly recommended) in patients with recurrent SPTM and suggest that those patients may have a mild acute attack phase and favorable long-term prognosis.

scholarly journals Concomitant tricuspid regurgitation severity and its secondary reduction determine long-term prognosis after transcatheter mitral valve edge-to-edge repair

2021 ◽  
Author(s):  
Martin Geyer ◽  
Karsten Keller ◽  
Kevin Bachmann ◽  
Sonja Born ◽  
Alexander R. Tamm ◽  
...  
Keyword(s):  
Tricuspid Regurgitation ◽  
Survival Data ◽  
Common Finding ◽  
Prognostic Impact ◽  
Term Survival ◽  
Long Term Prognosis ◽  
The Impact ◽  
Symptomatic Benefit

Abstract Background Concomitant tricuspid regurgitation (TR) is a common finding in mitral regurgitation (MR). Transcatheter repair (TMVR) is a favorable treatment option in patients at elevated surgical risk. To date, evidence on long-term prognosis and the prognostic impact of TR after TMVR is limited. Methods Long-term survival data of patients undergoing isolated edge-to-edge repair from June 2010 to March 2018 (combinations with other forms of TMVR or tricuspid valve therapy excluded) were analyzed in a retrospective monocentric study. TR severity was categorized and the impact of TR on survival was analysed. Results Overall, 606 patients [46.5% female, 56.4% functional MR (FMR)] were enrolled in this study. TR at baseline was categorized severe/medium/mild/no or trace in 23.2/34.3/36.3/6.3% of the cases. At 30-day follow-up, improvement of at least one TR-grade was documented in 34.9%. Severe TR at baseline was identified as predictor of 1-year survival [65.2% vs. 77.0%, p = 0.030; HR for death 1.68 (95% CI 1.12–2.54), p = 0.013] and in FMR-patients also regarding long-term prognosis [adjusted HR for long-term mortality 1.57 (95% CI 1.00–2.45), p = 0.049]. Missing post-interventional reduction of TR severity was predictive for poor prognosis, especially in the FMR-subgroup [1-year survival: 92.9% vs. 78.3%, p = 0.025; HR for death at 1-year follow-up 3.31 (95% CI 1.15–9.58), p = 0.027]. While BNP levels decreased in both subgroups, TR reduction was associated with improved symptomatic benefit (NYHA-class-reduction 78.6 vs. 65.9%, p = 0.021). Conclusion In this large study, both, severe TR at baseline as well as missing secondary reduction were predictive for impaired long-term prognosis, especially in patients with FMR etiology. TR reduction was associated with increased symptomatic benefit. Graphic abstract

Are Prolonged Ventricular Pauses in Atrial Fibrillation a Marker of Poor Prognosis?

Cardiology ◽  
2021 ◽  
Author(s):  
Dorte Marie Stavnem ◽  
Rakin Hadad ◽  
Bjørn Strøier Larsen ◽  
Olav Wendelboe Nielsen ◽  
Mark Aplin Frederiksen ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Poor Prognosis ◽  
Pacemaker Implantation ◽  
Real Life ◽  
Increased Risk ◽  
All Cause Mortality ◽  
Long Term Prognosis ◽  
Rate Limiting

Background: In patients with atrial fibrillation (AF), the long-term prognosis of long electrocardiographic pauses in the ventricular action is not well-studied. Methods: Consecutive Holter recordings in patients with AF (n=200) between 2009-2011 were evaluated, focusing on pauses of at least 2.5 s. Outcomes of interest were all-cause mortality and pacemaker implantation. Results: Forty-three patients (21.5%) had pauses with a mean of 3.2 s and SD of 0.9 s. After a median follow-up of 99 months (ranging 89-111), 47% (20/43) of the patients with, and 45% (70/157) without pauses were deceased. Pauses of ≥ 2.5 s did not constitute a risk of increased mortality: HR = 0.75; (95% CI: 0.34 - 1.66); p = 0.48. Neither did pauses of ≥ 3.0 s: HR = 0.43; (95% CI: 0.06 - 3.20); p = 0.41. Sixteen percent of patients with pauses underwent pacemaker implantation during follow-up. Only pauses in patients referred to Holter due to syncope and/or dizzy spells were associated with an increased risk of pacemaker treatment: HR = 4.7 (95% CI: 1.4-15.9), p = 0.014, adjusted for age, sex and rate-limiting medication. Conclusion: In patients with AF, prolonged electrocardiographic pauses of ≥ 2.5 s or ≥ 3.0 s are not a marker for increased mortality in this real-life clinical study.

scholarly journals Long-Term Prognosis in Young Patients with Stable Coronary Disease: Ten-Year Follow-up of Percutaneous Coronary Intervention

2012 ◽  
Vol 20 (2) ◽  
pp. 161-165 ◽  
Author(s):  
Igor Ribeiro de Castro Bienert ◽  
Expedito E. Ribeiro ◽  
Luiz J. Kajita ◽  
Marco Antonio Perin ◽  
Carlos A.H. Campos ◽  
...  
Keyword(s):  
Percutaneous Coronary Intervention ◽  
Coronary Disease ◽  
Young Patients ◽  
Coronary Intervention ◽  
Percutaneous Coronary ◽  
Long Term Prognosis

scholarly journals Beyond Sector Retinitis Pigmentosa: Expanding the Phenotype and Natural History of the Rhodopsin Gene Codon 106 Mutation (Gly-to-Arg) in Autosomal Dominant Retinitis Pigmentosa

Genes ◽  
2021 ◽  
Vol 12 (12) ◽  
pp. 1853
Author(s):  
Brian G. Ballios ◽  
Emily M. Place ◽  
Luis Martinez-Velazquez ◽  
Eric A. Pierce ◽  
Jason I. Comander ◽  
...  
Keyword(s):  
Retinitis Pigmentosa ◽  
Autosomal Dominant ◽  
Peripheral Vision ◽  
Visual Fields ◽  
Macular Disease ◽  
History Of ◽  
Intrafamilial Variability ◽  
Long Term Prognosis

Sector and pericentral are two rare, regional forms of retinitis pigmentosa (RP). While usually defined as stable or only very slowly progressing, the available literature to support this claim is limited. Additionally, few studies have analyzed the spectrum of disease within a particular genotype. We identified all cases (9 patients) with an autosomal dominant Rhodopsin variant previously associated with sector RP (RHO c.316G > A, p.Gly106Arg) at our institution. Clinical histories were reviewed, and testing included visual fields, multimodal imaging, and electroretinography. Patients demonstrated a broad phenotypic spectrum that spanned regional phenotypes from sector-like to pericentral RP, as well as generalized disease. We also present evidence of significant intrafamilial variability in regional phenotypes. Finally, we present the longest-reported follow-up for a patient with RHO-associated sector-like RP, showing progression from sectoral to pericentral disease over three decades. In the absence of comorbid macular disease, the long-term prognosis for central visual acuity is good. However, we found that significant progression of RHO p.Gly106Arg disease can occur over protracted periods, with impact on peripheral vision. Longitudinal widefield imaging and periodic ERG reassessment are likely to aid in monitoring disease progression.

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