Suprasellar Rathke Cleft Cysts; Clinical Presentation and Treatment Outcomes

Neurosurgery ◽  
2011 ◽  
Vol 69 (5) ◽  
pp. 1058-1069 ◽  
Author(s):  
Matthew B. Potts ◽  
Arman Jahangiri ◽  
Kathleen R. Lamborn ◽  
Lewis S. Blevins ◽  
Sandeep Kunwar ◽  
...  

Abstract BACKGROUND Rathke cleft cysts (RCCs), benign remnants of the Rathke pouch typically arising in the sella, sometimes have suprasellar extension. Purely suprasellar RCCs are rarely reported. OBJECTIVE To compare the presentations, surgical outcomes, and pathology of purely suprasellar RCCs and sellar-based RCCs. METHODS We retrospectively reviewed records, magnetic resonance images, laboratory results, and pathology of 151 RCC patients surgically managed at our institution from 1989 to 2009. The RCCs were classified as purely sellar (type I, n = 76), sellar with suprasellar extension (type II, n = 56), or purely suprasellar (type III, n = 19). RESULTS The RCCs with a suprasellar component (types II and III) more commonly presented with visual dysfunction (P < .001). Complete cyst drainage occurred in 89%, 55%, and 38% of type I, II, and III RCCs, respectively (P < .001). Vision improved in 100%, 55%, and 33% and headache improved in 74%, 64%, and 29% of type I, II, and III patients, respectively (P = .02). Temporary or permanent postoperative diabetes insipidus occurred in 5%, 16%, and 21% of type I, II, and III patients, respectively. (P < .001). In a multivariate analysis, RCC type was the only factor predicting recurrence. Kaplan-Meier 3-year recurrence/progression rates were 0%, 16%, and 29% for type I, II, and III RCCs, respectively (P < .001, type I vs II, type I vs III; P = .5 type II vs III). CONCLUSION The RCCs with a suprasellar component are neurosurgically challenging because of their proximity to the optic chiasm and infundibulum. Compared with sellar-based RCCs, RCCs with a suprasellar component more frequently present with visual dysfunction, are more difficult to completely eliminate, recur more frequently, and are associated with higher postoperative endocrine morbidity, and their preoperative visual dysfunction and headache less frequently improve with surgery. These factors must be considered during the treatment of RCCs with a suprasellar component.

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii129-ii129
Author(s):  
Emilie Le Rhun ◽  
Patrick Devos ◽  
Johannes Weller ◽  
Katharina Seystahl ◽  
Francesca Mo ◽  
...  

Abstract BACKGROUND The EANO ESMO guidelines have proposed a classification of leptomeningeal metastases (LM) based on clinical (typical/atypical), cytological (positive/negative/equivocal) and MRI (A linear, B nodular, C linear and nodular, D normal or hydrocephalus only) presentation. Type I LM is defined by the presence of tumor cells in the cerebrospinal fluid (CSF) (confirmed LM) whereas type II LM is defined by typical clinical and MRI signs (probable or possible LM). Here we explored the clinical utility of these EANO ESMO LM subtypes for choice of treatment and outcome. PATIENTS AND METHODS We retrospectively assembled data from 254 patients with newly diagnosed LM from different solid tumors, including as main primary tumors breast cancer (n=98, 45%), lung cancer (n=65, 25.5%) and melanoma (n=51, 13.5%). Survival curves were estimated using the Kaplan-Meier method and compared by Log-rank test. RESULTS Median age at LM diagnosis was 56.5 years (range 20-82 years). Typical clinical LM symptoms or signs were noted in 225 patients (88.5%); only 13 patients (5%) were clinically asymptomatic. The most common MRI subtype was A seen in 117 patients (46%). Types B (n=33, 13%), C (n=54, 21%) and D (n=50, 19.5%) were less common. Tumor cells in the CSF were observed in 186 patients (73%) whereas the CSF was equivocal in 24 (9.5%) and negative in 44 (17.5%) patients. Patients with confirmed LM had inferior outcome than patients with probable or possible LM (p=0.0063). Type I patients had inferior outcome than type II patients (p=0.0019). Nodular disease was a negative prognostic factor in type II LM, but not in type I LM (p=0.0138). CONCLUSION The EANO ESMO LM subtypes are highly prognostic and should be considered for stratification and overall design of clinical trials.


2018 ◽  
Vol 28 (3) ◽  
pp. 586-593 ◽  
Author(s):  
Mette Calundann Noer ◽  
Sofie Leisby Antonsen ◽  
Bent Ottesen ◽  
Ib Jarle Christensen ◽  
Claus Høgdall

ObjectiveTwo distinct types of endometrial carcinoma (EC) with different etiology, tumor characteristics, and prognosis are recognized. We investigated if the prognostic impact of comorbidity varies between these 2 types of EC. Furthermore, we studied if the recently developed ovarian cancer comorbidity index (OCCI) is useful for prediction of survival in EC.Materials and MethodsThis nationwide register-based cohort study was based on data from 6487 EC patients diagnosed in Denmark between 2005 and 2015. Patients were assigned a comorbidity index score according to the Charlson comorbidity index (CCI) and the OCCI. Kaplan-Meier survival statistics and adjusted multivariate Cox regression analyses were used to investigate the differential association between comorbidity and overall survival in types I and II EC.ResultsThe distribution of comorbidities varied between the 2 EC types. A consistent association between increasing levels of comorbidity and poorer survival was observed for both types. Cox regression analyses revealed a significant interaction between cancer stage and comorbidity indicating that the impact of comorbidity varied with stage. In contrast, the interaction between comorbidity and EC type was not significant. Both the CCI and the OCCI were useful measurements of comorbidity, but the CCI was the strongest predictor in this patient population.ConclusionsComorbidity is an important prognostic factor in type I as well as in type II EC although the overall prognosis differs significantly between the 2 types of EC. The prognostic impact of comorbidity varies with stage but not with type of EC.


Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 2637-2637 ◽  
Author(s):  
Wenbin Xao ◽  
Wayne Chen ◽  
Lynn Sorbara ◽  
Theresa Davies-Hill ◽  
Stefania Pittaluga ◽  
...  

Abstract The classical Hodgkin lymphoma variant of Richter transformation (CHL-RT) occurs rarely in patients with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL). Two morphological patterns have been described: type I with Hodgkin/Reed-Sternberg (HRS) cells scattered in a CLL background, and type II with typical CHL morphology. HRS cells are frequently positive for EBV and can be clonally related or unrelated to CLL. The clinical significance of the different morphological patterns is unclear. What factors dictate the cellular derivation of the HRS cells remains elusive. We retrospectively reviewed 77 cases of CHL-RT submitted to our consultation service. Clinicopathological characteristics were summarized, EBV status was examined, and clonality was analyzed after microdissection of HRS-cells and CLL cells. Patients with the type I pattern (N=26) had a significantly shorter time to progression from CLL to CHL-RT than those with type II pattern (N=51, 15 vs. 49 months, p<0.0001, see Figure 1A). Consistent with these data, 27% of patients with the type I pattern had a prior CLL history as compared to 73% with the type II pattern. 12% (6/51) of type II cases had extranodal involvement (sites other than bone marrow) while none of type I cases did. Three patients with sequential biopsies progressed from type I to II and 2 had an aggressive clinical course. HRS cells were positive for EBV in 71% (55/77) of patients. Clonality analysis was performed in 33 cases: HRS cells were clonally related to the underlying CLL in 14 cases and unrelated in 19 cases. Among all the features examined, ZAP-70 expression of the CLL cells, but not EBV status or morphological pattern, was strongly correlated with clonal relationship: all 14 clonally related cases were negative for ZAP-70 while 74% (14/19) of the clonally unrelated cases were positive for ZAP-70. Overall median survival after the diagnosis of CHL-RT was 44 months. Advanced age was an adverse risk factor for survival (p<0.05, see Figure 1B). In conclusion, we provide evidence that type I morphology is more likely an early stage of CHL-RT and can progress to type II. The majority of CHL-RT cases are EBV positive. Clonal relationship in RT is determined by ZAP-70 and thus likely IGHV mutational status. Advanced age is associated with inferior survival. Figure 1. A, Time to progress from CLL to CHL-RT. B, Kaplan-Meier analysis of overall survival. Figure 1. A, Time to progress from CLL to CHL-RT. B, Kaplan-Meier analysis of overall survival. Disclosures No relevant conflicts of interest to declare.


2020 ◽  
Author(s):  
Emilie Le Rhun ◽  
Patrick Devos ◽  
Johannes Weller ◽  
Katharina Seystahl ◽  
Francesca Mo ◽  
...  

Abstract Background The EANO ESMO guidelines have proposed a classification of leptomeningeal metastases (LM) from solid cancers based on clinical, magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) cytology presentation. MRI patterns are classified as linear, nodular, both, or neither. Type I LM is defined by positive CSF cytology (confirmed LM) whereas type II LM is defined by typical clinical and MRI signs (probable or possible LM). Here we explored the clinical utility of these LM subtypes. Patients and methods We retrospectively assembled data from 254 patients with newly diagnosed LM from solid tumors. Survival curves were derived using the Kaplan-Meier method and compared by Log-rank test. Results Median age at LM diagnosis was 56 years. Typical clinical LM features were noted in 225 patients (89%); 13 patients (5%) were clinically asymptomatic. Tumor cells in the CSF were observed in 186 patients (73%) whereas the CSF was equivocal in 24 patients (9.5%) and negative in 44 patients (17.5%). Patients with confirmed LM had inferior outcome compared with patients with probable or possible LM (p=0.006). Type I patients had inferior outcome than type II patients (p=0.002). Nodular disease on MRI was a negative prognostic factor in type II LM (p=0.014), but not in type I LM. Administration of either intrathecal pharmacotherapy (p=0.020) or systemic pharmacotherapy (p=0.0004) was associated with improved outcome in type I LM, but not in type II LM. Conclusion The EANO ESMO LM subtypes are highly prognostic and should be considered for stratification and overall design of clinical trials.


2020 ◽  
Vol 2 (Supplement_2) ◽  
pp. ii7-ii8
Author(s):  
Emilie Le Rhun ◽  
Patrick Devos ◽  
Johannes Weller ◽  
Katharina Seystahl ◽  
Francesca Mo ◽  
...  

Abstract BACKGROUND The EANO ESMO guidelines have proposed a classification of leptomeningeal metastases (LM) based on clinical (typical/atypical), cytological (positive/negative/equivocal) and MRI (A linear, B nodular, C linear and nodular, D normal or hydrocephalus only) presentation. Type I LM is defined by the presence of tumor cells in the cerebrospinal fluid (CSF) (confirmed LM) whereas type II LM is defined by typical clinical and MRI signs (probable or possible LM). Here we explored the clinical utility of these EANO ESMO LM subtypes. PATIENTS AND METHODS We retrospectively assembled data from 254 patients with newly diagnosed LM from different solid tumors, including as main primary tumors breast cancer (n=98, 45%), lung cancer (n=65, 25.5%) and melanoma (n=51, 13.5%). Survival curves were estimated using the Kaplan-Meier method and compared by Log-rank test. RESULTS Median age at LM diagnosis was 56.5 years (range 20–82 years). Typical clinical LM symptoms or signs were noted in 225 patients (88.5%); only 13 patients (5%) were clinically asymptomatic. The most common MRI subtype was A seen in 117 patients (46%). Types B (n=33, 13%), C (n=54, 21%) and D (n=50, 19.5%) were less common. Tumor cells were observed in the CSF in 186 patients (73%) whereas the CSF was equivocal in 24 (9.5%) and negative in 44 (17.5%) patients. Patients with confirmed LM had inferior outcome than patients with probable or possible LM (p=0.0063). Type I patients had inferior outcome than type II patients (p=0.0019). Nodular disease was a negative prognostic factor in type II LM, but not in type I LM (p=0.0138). CONCLUSION The presence of tumor cells in the CSF appears to have a greater prognostic role than the neuroimaging presentation. EANO ESMO LM subtypes are highly prognostic and should be considered in the design of clinical trials.


Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 2061-2061
Author(s):  
Sami Malek ◽  
Sam Fossum ◽  
Kerby Shedden ◽  
Peter Ouillette

Abstract Unbiased quantitative assessments of genomic complexity in CLL using SNP arrays have identified high genomic complexity (≥3 subchromosomal lesions per genome) as a strong negative independent prognostic factor for short time to first therapy (TTFT) and short time to subsequent therapy (TTST) in CLL. In the present study, we have analyzed various variables as potential predictors for genomic complexity in CLL, including ZAP-70 status, CD38 status, IgVH mutation status, p53 aberrations (exon 5–9 sequence mutations or absent expression), CLL FISH categories (del17p, del11q, trisomy 12, and del13q14 type I or del13q14 type II, based on SNP arrays), MDM2 SNP309 allele status and the fraction of CLL cells alive 40 hours post-irradiation using univariate (N=178 cases) and multivariate (N=138 to 166 cases, depending on the included variables) analysis. Data on irradiation-induced ATM autophosphorylation are nearing completion and will be analyzed once available. SNP array-based genomic complexity was analyzed either as a dichotomous variable (&lt;3 versus ≥3 subchromosomal lesions per genome) or as a quantitative variable and was based on either losses only (~85% of all lesions in CLL) or as total complexity (losses, gains and UPD). Results: Using the dichotomous genomic complexity score based on losses, the percentage of cells alive post-irradiation (odds ratio [OR] 3.26, p&lt;0.0001), CD38 expression (OR=3.42, p=0.006), ZAP-70 expression (OR=4.06, p=0.005), IgVH-unmutated (OR=3.54, p=0.008), p53 aberrations (OR=13.2, p&lt;0.0001), del13q14 type II (OR=3.42, p=0.015), del17p (OR=21, p&lt;0.0001) and del11q (OR=6.6, p=0.002) all emerged as significant predictors of genomic complexity in univariate analysis. For multivariate analysis, various models were built incorporating factors with significant univariate associations and alternatively incorporating either del17p, p53 aberrations or the fraction of CLL cells alive post-irradiation (all three variables are p53 status-dependent) into the model. Using the dichotomous genomic complexity score based on losses, the fraction of cells alive post-irradiation, del17p, del11q, del13q14 type II, p53 aberrations and CD38 expression, emerged as significant independent predictors of genomic complexity in multivariate analysis. ZAP-70, IgVH status and del13q14 type I were not independent predictors. Using quantitative genomic complexity based on losses, the fraction of cells alive post-irradiation, del17p, del11q, del13q14 type I or II, and p53 aberrations emerged as significant predictors of genomic complexity in multivariate analysis. ZAP-70, CD38 expression and IgVH status were not independent predictors. In summary, multiple markers independently predict for genomic complexity in CLL, facilitating identification of genes/mutations (p53, ATM, Rb and others) involved in conferring unstable genomes in CLL. This analysis identifies SNP array-based genome interrogations as a single test uniquely able to capture genomic complexity in CLL.


2021 ◽  
pp. 1-10
Author(s):  
Li Chen ◽  
Weichen Zhang ◽  
Jinyun Tan ◽  
Min Hu ◽  
Weihao Shi ◽  
...  

<b><i>Background:</i></b> Neointimal hyperplasia (NIH) is believed to be the main reason for arteriovenous fistula (AVF) dysfunction, but other mechanisms are also recognized to be involved in the pathophysiological process. This study investigated whether different morphological types of AVF lesions are associated with the patency rate after percutaneous transluminal angioplasty (PTA). <b><i>Methods:</i></b> This retrospective study included 120 patients who underwent PTA for autogenous AVF dysfunction. All the cases were evaluated under Doppler ultrasound (DU) before intervention and divided into 3 types: Type I (NIH type), Type II (non-NIH type), and Type III (mixed type). Prognostic and clinical data were analyzed by Kaplan-Meier analysis and the Cox proportional hazards model. <b><i>Results:</i></b> There was no statistical difference in baseline variables among groups, except for lumen diameter. The primary patency rates in Type I, Type II, and Type III groups were 78.4, 93.2, and 83.2% at 6 months and 59.5, 84.7, and 75.5% at 1 year, respectively. The secondary patency rates in Type I, Type II, and Type III groups were 94.4, 97.1, and 100% at 6 months and 90.5, 97.1, and 94.7% at 1 year, respectively. The Kaplan-Meier curve showed that the primary and secondary patency rates of Type I group were lower than those of Type II group. Multivariable Cox regression analysis demonstrated that postoperative primary patency was correlated with end-to-end anastomosis (hazard ratio [HR] = 2.997, <i>p</i> = 0.008, 95% confidence interval [CI]: 1.328–6.764) and Type I lesion (HR = 5.395, <i>p</i> = 0.004, 95% CI: 1.730–16.824). <b><i>Conclusions:</i></b> NIH-dominant lesions of AVF evaluated by DU preoperatively were a risk factor for poor primary and secondary patency rate after PTA in hemodialysis patients.


2014 ◽  
Vol 4 ◽  
pp. 32 ◽  
Author(s):  
Gmaan A Alzhrani ◽  
Hosam M Al-Jehani ◽  
Denis Melançon

Split cord malformations (SCMs) are thought to be rare abnormalities representing 3.8-5% of all spinal cord anomalies. The prevalence is estimated to be 1 in 5499 live births (0.02%), with a slight female predominance (1.3:1). Although the estimates of prevalence vary, Type I SCM occurs more frequently than Type II SCM. In this paper, we are reporting the clinical presentation and imaging findings of multi-level SCM in a 27-year-old male. A literature review of the embryological background of SCM and pathological hypothesis for this entity is provided. A systematic review has been conducted to identify multi-level SCM cases reported in the literature, followed by proposing a new classification system to further our understanding and management of SCMs.


2021 ◽  
Vol 9 ◽  
pp. 2050313X2110118
Author(s):  
Phani Krishna Machiraju ◽  
Neetu Mariam Alex ◽  
Safinaaz ◽  
Nikita Mary Baby

Coronavirus disease-19 caused by severe acute respiratory syndrome Corona virus-2 is characterised by wide heterogeneity in clinical presentation. The typical radiographic findings in COVID-19 include bilateral ground-glass opacities and/or consolidations predominantly affecting the lower lobes and posterior segments of lungs. Other rare abnormal radiographic findings include pneumothorax, pneumomediastinum and pneumopericardium. There has been an increased incidence of pneumomediastinum, a rare but potentially life-threatening complication during this pandemic. It may be spontaneous or secondary. Pneumomediastinum may be due to barotrauma, cytokine storm induced diffuse alveolar injury or direct viral infection of type I and type II pneumocytes. The presence of pneumomediastinum in COVID-19 patients may indicate extensive alveolar membrane destruction and those patients need close monitoring. There are no consensus guidelines in managing COVID-19 patients with pneumomediastinum. Higher mortality rates (70.58%) are reported in intubated COVID-19 patients with pneumomediastinum. The development of pneumomediastinum in COVID-19 should be considered as a poor prognostic factor.


2017 ◽  
Vol 9 (1) ◽  
pp. 11-17 ◽  
Author(s):  
Abduelmenem Alashkham ◽  
Abdulrahman Alraddadi ◽  
Roger Soames

A notch is often observed on the anterosuperior aspect of the glenoid fossa, however its association with gender remains unexplored. The aims of this study were to: (i) investigate the incidence and type of glenoid notch, and (ii) its association with gender, age and side. A total of 140 shoulders from 30 male and 40 female cadavers were examined. All muscles and blood vessels surrounding the glenohumeral joint, as well as the fibrous capsule, were removed to expose the glenoid fossa: the presence of a notch was classified as type I (mild), type II (moderate) or type III (severe). The mean age of specimens was 81.5 years (±9.8 years). A type III notch was the most commonly observed (32 male, 21 female specimens), followed by type I (14 male, 34 female specimens) and finally type II (14 male, 25 female specimens). Multivariate analysis showed that the type of glenoid notch was significantly associated with gender (?2 (2, n=140) = 11.088, p = 0.004). Females are significantly more likely to have a type I or II glenoid notch, while males are significantly more likely to have a type III notch. This difference could explain the higher incidence of shoulder dislocation in males compared to females.  A menudo se observa una muesca en el lado anterosuperior de la fosa glenoidea, sin embargo su relación con el sexo sigue siendo inexplorada. Los objetivos de este estudio fueron: (i) investigar la incidencia y el tipo de muesca glenoidea, y (ii) su relación con el sexo, la edad y el lado en el que se observa. Se examinaron un total de 140 hombros de entre 30 cadáveres masculinos y 40 femeninos. Todos los músculos y vasos sanguíneos que rodean la articulación glenohumeral, así como la cápsula fibrosa, fueron retirados para permitir el acceso a la fosa glenoidea: la presencia de la muesca fue clasificada como tipo I (leve), tipo II (moderado) o tipo III (grave). La edad media de los especímenes examinados fue de 81,5 años (± 9,8 años). La muesca de tipo III fue la más comúnmente observada (32 varones, 21 hembras), seguida por la muesca de tipo I (14 varones, 34 hembras) y finalmente seguida de la de tipo II (14 varones, 25 hembras). El análisis multivariado mostró que el tipo de muesca glenoidea está significativamente relacionado con el sexo (?2 (2, n = 140) = 11.088, p = 0.004). Las mujeres son significativamente más propensas a presentar una muesca glenoidea de tipo I o II, mientras que los varones son significativamente más propensos a presentar una muesca de tipo III. Esta diferencia podría explicar la mayor incidencia de luxación de hombro que se produce en los varones en comparación con la que se produce en las mujeres. 


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