Clinical prognostic factors for pediatric extra-abdominal desmoid tumor: analyses of 66 patients at a single institution

Background and purpose

Pediatric desmoid tumor (PDT) is rare and has a high local recurrence rate. The purpose of the present study was to analyze clinical risk factors of local recurrence in PDT patients.

Materials and methods

We reviewed clinical data of 66 PDT patients from 2004 to 2015. All patients underwent macroscopically complete resection, and some recurrent tumors were prescribed radiotherapy. Factors such as sex, age at presentation, location, and proximity to nerves or vasculature were analyzed. The local recurrence rate and recurrence-free survival were analyzed with these factors.

Results

All patients in the present study were children and had extra-abdominal tumors. The median follow-up time was 6.6?years. Thirty-six (55%) patients had local recurrence. Age, sex, tumor site, tumor size, and proximity to nerves/vasculature had a significant impact on prognosis in univariate analysis. Radiotherapy decreased the local recurrence rate. In multivariate analysis, younger age, tumor location in buttocks, larger tumor, and proximity to important nerves/vasculature were independent risk factors for poor prognosis.

Conclusions

Favorable therapeutic strategies could be selected according to the preoperative prognostic risk factors. Radiotherapy should be considered for local recurrence of PDT.

     

Prognostic significance of microvascular invasion in tumor stage for hepatocellular carcinoma

Background

The presence of microvascular invasion (McVI) in hepatocellular carcinoma (HCC) has been proposed as a cause of recurrence and poor survival, although this has not been officially emphasized in staging systems. Thus, we conducted a retrospective study to investigate the prognostic importance of McVI in tumor staging in patients with HCC who underwent hepatic resection.

Methods

A retrospective analysis was performed of patients who underwent hepatic resection for HCC at our center from 1994 to 2012. Patients with HCC were classified into four groups based on the presence of McVI and extent of gross vascular invasion (VI).

Results

The 5-year overall and recurrence-free survival rates of 676 patients were 63.3 and 42.6%, respectively. There was no difference in tumor recurrence or survival rate between patients with HCC and McVI without gross VI and those with gross VI confined to segmental/sectional branches. Multivariate analysis revealed that the extent of VI based on the presence of McVI and gross VI was independently associated with tumor recurrence and overall survival.

Conclusions

McVI was revealed to be an important risk factor similar to gross VI confined to a segmental/sectional branch in patients with HCC who underwent hepatic resection. This finding should be considered when estimating the stage for prognosis.

     

The importance of margins status after breast conservative surgery and radiotherapy in node positive patients: a follow-up of 10–15 years

Background

Margin status is the main factor determining local recurrence (LR) after wide excision and radiotherapy for breast cancer. The aim of the study is to evaluate if positive margins are as great a risk factor for LR in node-positive as in node-negative patients, since the major risk in the former group is dissemination and whether there is a correlation between nodal status and margins in relation to prognosis.

Methods

773 patients underwent WLE and radiotherapy between 1988 and 1992 and were followed-up (> 10 years) to determine LR rates according to margin and nodal status. Margins were assessed by cavity-shave biopsies and the axilla was staged by sampling or clearance.

Results

461 patients were node negative and 312 node positive. In the node-negative group 415 patients had negative margins and 46 positive: LR after > 10 years was 12 % and 28 % respectively. Among the 312 patients in the node positive group, 267 were margin negative and 45 positive; the LR rate was 12 % and 18 % respectively. In the node negative-group there was a statistically significant difference between the positive and the negative margins with higher relapse rate and lower overall survival (p < 0.001), whereas in the node-positive group the equivalent comparison didn't show any statistical difference.

Conclusion

Although re-excision should be always recommended, in node-negative patients positive margins are associated with a statistically higher LR rate and lower overall survival while in node-positive disease margins might be of less importance in determining prognosis as dissemination is more likely to occur.

     

Medium-term outcomes of 78,808 patients after heart valve surgery in a middle-income country: a nationwide population-based study

Background

Heart valve surgery outcomes are unknown in middle-income countries and thus cannot be used in health system decision making processes. This study estimated in-hospital mortality and medium and long-term survival.

Methods

This was a retrospective study of 78,806 patients who underwent heart valve surgery between 2001 and 2007 in Brazil. Two national databases were used, the Hospital Information System and the Mortality Information System. Kaplan-Meier survival analysis and log-rank tests were performed. Maximum and median follow-up was 7.7 and 2.8 years, respectively (0.002–7.707).

Results

Valve replacement accounted for 69.1% of procedures performed. Mitral stenosis, the most common valve injury, represented 38.9% of the total. In 94.7% of mitral stenosis patients, aetiology was rheumatic heart disease. In-hospital mortality was 7.6% and was higher for women, for patients who had undergone concomitant coronary artery bypass grafting (CABG) and for the elderly. Overall survival was 69.9% at the end of follow-up. Survival was worst among elderly, male and concomitant CABG patients (P<0.001).

Conclusions

Rheumatic heart disease is still a major public health problem in Brazil. In-hospital mortality and global survival rates of patients who have undergone heart valve surgery were less satisfactory than those reported in high-income countries. The findings of this study can contribute to guiding decision making processes in middle-income countries similar to Brazil and others concerned with improving the quality of care.

     

Metallic small y stent placement at primary right carina for bronchial disease

Background

Metallic large Y stent placement has been used mainly for airway disease around the main carina. However, few studies have reported this treatment for bronchial disease around the primary right carina.

Methods

Twenty-eight patients were treated by small y stent. All stents were custom-designed and placed under fluoroscopic guidance. Clinical and imaging data were analyzed retrospectively.

Results

Thirty-one stents were successfully inserted in 28 patients. Twenty-five patients succeed at the first attempt (89.3%), and 3 patients needed a second attempt. Twelve complications occurred in 10 patients (35.7%). Stent restenosis and sputum retention were the most common complications. Five patients underwent successful stent removal due to complications or cure efficacy. During follow up, 17 patients died of tumors and one died of myocardial infarction. The 1-, 3-, and 5-year survival rates were 49.3, 19.6 and 19.6%, respectively.

Conclusions

Metallic small y stent placement is technically feasible, effective and safe for bronchial disease around the primary right carina.

     

Physician attitudes and treatment patterns for pancreatic cancer

Background

Surgery appears to be an underutilized treatment option for pancreatic cancer. Nihilistic physician attitudes may be partly responsible. The study objectives were to analyze physician attitudes towards this disease and determine treatment patterns and outcomes including rates of surgical referral.

Methods

A survey was administered to 420 physicians in Manitoba to document general knowledge and attitudes. Population based administrative data was accessed for all patients diagnosed with pancreatic cancer between 2004 and 2006 to examine treatment patterns and outcomes.

Results

181 physicians responded to the survey. Most (73%) believed that surgical resection was worthwhile. Of the 413 Manitobans diagnosed with pancreatic cancer, only 11% underwent an attempt at surgical resection. There were 124 patients with stage I or II disease (i.e. potentially resectable), 85 of these patients received no treatment and 39% were not referred to a surgeon. These patients were older than those referred, but did not have more comorbidities.

Conclusion

Most physicians were insightfully aware of both the survival benefit and potential risks of surgical resection. However, some did overestimate the surgical mortality and underestimate the associated survival benefit. Although advanced age may justly account for some of the patients not receiving a referral, it is reasonable to assume that nihilistic physician attitudes is contributing to the apparent underutilization of surgery for pancreatic cancer. Efforts should be made to ensure that eligible patients are at least offered surgery as a potential treatment option.

     

Recurrent Nicolau syndrome associated with subcutaneous glatiramer acetate injection—a case report

Background

Glatiramer acetate is worldwide used as first line treatment in relapsing remitting multiple sclerosis. Local skin reactions associated with glatiramer acetate are common, however, only isolated cases of severe local injection site reactions known as Nicolau Syndrome have been reported so far.

Case presentation

We describe the case of a recurrent Nicolau Syndrome occurred during longstanding glatiramer acetate treatment in a woman with multiple sclerosis. The haemorrhagic patch necrotized and was treated locally as a deep second degree burn with excision of dead skin tissue and was healed. Treatment with glatiramer acetate was definitely suspended.

Conclusions

GA injections can be complicated by isolated or recurrent Nicolau Syndrome, a potentially life-threatening condition of which neurologists should be aware.

     

Is preoperative core biopsy accurate in determining the hormone receptor status in women with invasive breast cancer?

Background

The objective of this study was to determine the concordance rate between core needle biopsy (CNB) and surgical excision of invasive breast cancer regarding the oestrogen receptor (ER) and Progesterone receptor (PgR) status as determined by Immunohistochemistry (IHC).

Methods

Hormone receptor status was established using IHC (using quickscore system 0–8) on preoperative CNB and subsequent surgical excision in 93 patients with invasive breast cancer. Results were compared taking into account tumour's size, grade, and patient's age.

Results

The ER concordance rate between CNB and surgical excisions was 95%. The PgR concordance rate was 89%. This shows that CNB has a sensitivity of 97% for ER and 95% for PgR.There is a positive correlation of ER and PgR between CNB and surgical excision (p < 0.000001). There was no significant difference in the number of core biopsies between concordant and discordant cases.

Conclusion

Preoperative core biopsy is highly sensitive for the IHC detection of ER and PgR in invasive breast cancer. The concordance rate is higher for ER than PgR, which could be due to the fact that ER is more homogeneously distributed.

     

Phylloides tumor of the breast: a rare neoplasm,though not that innocent

Background

Cystosarcoma phylloides (CP) is an extremely rare form of breast cancer with an unpredictable clinical course. The histological characteristics of this neoplasm have not proved to offer much in the estimation of prognosis of these patients.

Patients and methods

In our clinics, in a time period of 38 years, 22 patients with cystosarcoma phylloides were treated. There were 5 cases of malignancy, 15 cases with benign tumors, and two cases histologically characterized as borderline neoplasia. Metastases were manifested in one patient. All patients were on a 5-year follow-up, except in five cases, one operated three years ago and four operated within the last two years.

Results

16 of 22 patients did not present any signs of local recurrence or metastases. There were three patients that manifested local recurrence and underwent supplementary ongectomy or mastectomy and are free of recurrence ever since. One patient with metastatic CP died.

Conclusion

Independently of its histopathological behavior, CP is a tumor difficult to be treated. Meticulous follow-up is mandatory in order to manage possible recurrence of the neoplasm.

     

Cardiac allograft vasculopathy in Dutch heart transplant recipients

Background

Cardiac allograft vasculopathy (CAV) is a multifactorial disease and a major cause of graft failure after heart transplantation. However, the impact of CAV may vary according to the definition and the regional differences in transplantation settings.

Objectives

We sought to assess CAV prevalence, predictors and prognosis in Dutch heart transplant recipients based on coronary angiography, following the 2010 standard nomenclature of the International Society for Heart and Lung Transplantation.

Methods

Patients ≥18 years who underwent heart transplantation at our centre with at least one coronary angiography during follow-up were included in the analysis. Clinical variables were collected prospectively.

Results

Among 495 analysed recipients, there were 238 (48?%) with CAV. The prevalence of CAV was 18, 47 and 70?% at 4, 12 and 20 years, respectively. In the multivariable proportional hazards regression analysis, only male donor gender and increasing donor age were significantly associated with the risk of CAV. The long-term prognosis of the patients with CAV at fourth-year angiography was significantly worse as compared with that of CAV-free patients, independently of the severity of CAV (p < 0.001).

Conclusion

The prevalence of CAV increased gradually over time, with a similar trend as in other registries. Post-transplant survival is decreased in patients with any degree of early CAV, indicating that management strategies should start with donor selection and preventive measures immediately after transplantation.

     

Hypocapnia is a biological marker for orthostatic intolerance in some patients with chronic fatigue syndrome

Context

Patients with chronic fatigue syndrome and those with orthostatic intolerance share many symptoms, yet questions exist as to whether CFS patients have physiological evidence of orthostatic intolerance.

Objective

To determine if some CFS patients have increased rates of orthostatic hypotension, hypertension, tachycardia, or hypocapnia relative to age-matched controls.

Design

Assess blood pressure, heart rate, respiratory rate, end tidal CO2 and visual analog scales for orthostatic symptoms when supine and when standing for 8 minutes without moving legs.

Setting

Referral practice and research center.

Participants

60 women and 15 men with CFS and 36 women and 4 men serving as age matched controls with analyses confined to 62 patients and 35 controls showing either normal orthostatic testing or a physiological abnormal test.

Main outcome measures

Orthostatic tachycardia; orthostatic hypotension; orthostatic hypertension; orthostatic hypocapnia or combinations thereof.

Results

CFS patients had higher rates of abnormal tests than controls (53% vs 20%, p < .002), but rates of orthostatic tachycardia, orthostatic hypotension, and orthostatic hypertension did not differ significantly between patients and controls (11.3% vs 5.7%, 6.5% vs 2.9%, 19.4% vs 11.4%, respectively). In contrast, rates of orthostatic hypocapnia were significantly higher in CFS than in controls (20.6% vs 2.9%, p < .02). This CFS group reported significantly more feelings of illness and shortness of breath than either controls or CFS patients with normal physiological tests.

Conclusion

A substantial number of CFS patients have orthostatic intolerance in the form of orthostatic hypocapnia. This allows subgrouping of patients with CFS and thus reduces patient pool heterogeneity engendered by use of a clinical case definition.

     

Thirty years of heart transplantation at the University Medical Centre Utrecht

Purpose

To analyse patient demographics, indications, survival and donor characteristics for heart transplantation (HTx) during the past 30 years at the University Medical Centre Utrecht (UMCU).

Methods

Data have been prospectively collected for all patients who underwent HTx at the UMCU from 1985 until 2015. Patients who were included underwent orthotopic HTx at an age >14 years.

Results

In total, 489 hearts have been transplanted since 1985; 120 patients (25%) had left ventricular assist device (LVAD) implantation prior to HTx. A shift from ischaemic heart disease to dilated cardiomyopathy has been seen as the leading indication for HTx since the year 2000. Median age at HTx was 49 years (range 16–68). Median waiting time and donor age have also increased from 40 to 513 days and from 27 to 44 years respectively (range 11–65). Donor cause of death is now primarily stroke, in contrast to head and brain injury in earlier years. Estimated median survival is 15.4 years (95% confidence interval 14.2–16.6) There is better survival throughout these years.

Conclusion

Over the past 30 years, patient and donor demographics and underlying diseases have shifted substantially. Furthermore, the increase in waiting time due to lack of available donor hearts has led to a rise in the use of LVADs as bridge to transplant. Importantly, an improvement in survival rates is found over time which could be explained by better immunosuppressive therapy and improvements in follow-up care.

     

Off-site primary percutaneous coronary intervention in a new centre is safe: comparing clinical outcomes with a hospital with surgical backup

Objectives

To evaluate the procedural and clinical outcomes of a new primary percutaneous coronary intervention (PPCI) centre without surgical back-up (off-site PCI) and to investigate whether these results are comparable with a high volume on-site PCI centre in the Netherlands.

Background

Controversy remains about the safety and efficacy of PPCI in off-site PCI centres.

Methods

We retrospectively analysed clinical and procedural data as well as 6?month follow-up of 226 patients diagnosed with ST-elevated myocardial infarction (STEMI) who underwent PPCI at VieCuri Medical Centre Venlo and 115 STEMI patients who underwent PPCI at Catharina Hospital Eindhoven.

Results

PPCI patients in VieCuri Medical Centre had similar procedural and clinical outcomes to those in Catharina Hospital. Overall there were no significant differences. The occurrence of procedural complications was low in both groups (8.4?% VieCuri vs. 12.3?% Catharina Hospital). In the VieCuri group there was one procedural-related death. No patients in either group needed emergency surgery. At 30 days, 17 (7.9?%) patients in the VieCuri group and 9 (8.1?%) in the Catharina Hospital group had a major adverse cardiac event.

Conclusion

Performing PPCI in an off-site PCI centre is safe and effective. The study results show that the procedural and clinical outcomes of an off-site PPCI centre are comparable with an on-site high-volume PPCI centre.

     

Metabolomics approach for predicting response to neoadjuvant chemotherapy for colorectal cancer

Introduction

Colorectal cancer (CRC) is a clinically heterogeneous disease, which necessitates a variety of treatments and leads to different outcomes. Only some CRC patients will benefit from neoadjuvant chemotherapy (NACT).

Objectives

An accurate prediction of response to NACT in CRC patients would greatly facilitate optimal personalized management, which could improve their long-term survival and clinical outcomes.

Methods

In this study, plasma metabolite profiling was performed to identify potential biomarker candidates that can predict response to NACT for CRC. Metabolic profiles of plasma from non-response (n?=?30) and response (n?=?27) patients to NACT were studied using UHPLC–quadruple time-of-flight)/mass spectrometry analyses and statistical analysis methods.

Results

The concentrations of nine metabolites were significantly different when comparing response to NACT. The area under the receiver operating characteristic curve value of the potential biomarkers was up to 0.83 discriminating the non-response and response group to NACT, superior to the clinical parameters (carcinoembryonic antigen and carbohydrate antigen 199).

Conclusion

These results show promise for larger studies that could result in more personalized treatment protocols for CRC patients.

     

HLA-matched sibling transplantation with G-CSF mobilized PBSCs and BM decreases GVHD in adult patients with severe aplastic anemia

Background

Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is an effective treatment for severe aplastic anemia (SAA). However, graft failure and graft-versus-host disease (GVHD) are major causes of the early morbidity in Allo-HSCT.

Methods

To reduce graft failure and GVHD, we treated fifteen patients with SAA using high- dose of HSCT with both G-CSF mobilized PB and BMSCs from HLA-identical siblings to treat patients with SAA.

Results

All patients had successful bone marrow engraftment. Only one patient had late rejection. Median time to ANC greater than 0.5 × 10⁹/L and platelet counts greater than 20 × 10⁹/L was 12 and 16.5 days, respectively. No acute GVHD was observed. The incidence of chronic GVHD was 6.67%. The total three-year probability of disease-free survival was 79.8%.

Conclusion

HSCT with both G-CSF mobilized PB and BMSCs is a promising approach for heavily transfused and/or allo-immunized patients with SAA.

     

Three stage axillary lymphatic massage optimizes sentinel lymph node localisation using blue dye

Aims

This paper describes a simple technique of axillary and breast massage which improves the successful identification of blue sentinel nodes using patent blue dye alone.

Methods

Patent blue dye was injected in the subdermal part of the retroaroelar area in 167 patients having surgical treatment for invasive breast cancer. Three stage axillary lymphatic massage was performed prior to making the axillary incision for sentinel lymph node biopsy. All patients had completion axillary sampling or clearance.

Results

A blue lymphatic duct leading to lymph nodes of the first drainage was identified in 163 (97%) of the patients. Results are compared with 168 patients who had sentinel lymph node biopsy using blue dye without axillary massage. Allergic reactions were observed in four patients (1.2%).

Conclusion

Three stage axillary lymphatic massage improves the successful identification of a blue sentinel lymph node in breast cancer patients.

     

Factor XIII Val34Leu polymorphism and risk of recurrent pregnancy loss in Iranian population: a case control study

BACKGROUND

Recurrent pregnancy loss (RPL) is a heterogeneous condition and thrombophilias have been considered as a probable cause.

OBJECTIVE

The aim of this study was to investigate the prevalence of the coagulation factor XIII Val34Leu polymorphism among women with unexplained RPL.

METHODS

A total of 140 women with a history of unexplained RPL and 100 age-matched healthy fertile women were recruited. The presence of FXIII Val34Leu polymorphism among the cases and controls was investigated using PCR-RFLP method.

RESULTS

Genotype analyses of the subjects revealed that the patients had a significantly higher prevalence of V/L and L/L than the controls (P<0.05): 33.5% vs. 15%, and 9.2% vs. 2%, respectively.

CONCLUSION

These results indicate a significant association between FXIII Val34Leu polymorphism and unexplained RPL in the Iranian patient.

     

Extrahepatic cholangiocarcinoma with prolonged survival: a case report

Background

Cholangiocarcinoma has poor prognosis and short term-survival. Here, we report the case of a patient with unusually prolonged survival.

Case presentation

Our patient was a 56-year-old Arab man with a 6-month history of obstructive jaundice. A computed tomography scan of his abdomen revealed a mass at the confluence of the hepatic ducts with suspected malignant strictures on endoscopy. A positive tissue diagnosis was achieved more than 18 months after commencement of his symptoms. He remained functional throughout this period despite recurrent episodes of cholangitis.

Conclusions

Cholangiocarcinoma is a presumably fatal disease, especially because patients tend to present late with unresectable disease. Many patient-related and disease-related factors may alter survival.

     

KniMet: a pipeline for the processing of chromatography–mass spectrometry metabolomics data

Introduction

Data processing is one of the biggest problems in metabolomics, given the high number of samples analyzed and the need of multiple software packages for each step of the processing workflow.

Objectives

Merge in the same platform the steps required for metabolomics data processing.

Methods

KniMet is a workflow for the processing of mass spectrometry-metabolomics data based on the KNIME Analytics platform.

Results

The approach includes key steps to follow in metabolomics data processing: feature filtering, missing value imputation, normalization, batch correction and annotation.

Conclusion

KniMet provides the user with a local, modular and customizable workflow for the processing of both GC–MS and LC–MS open profiling data.

     

Three dimensional rotational angiography for assessment of coronary arteries during melody valve implantation: introducing a technique that may improve outcomes

Background

Adverse events from Melody valve implantation may be catastrophic. To date a role for three dimensional rotational angiography of the aortic root (3DRAA) during Melody valve implantation has not been established.

Objectives

To describe the role of 3DRAA in the assessment of Melody valve candidacy and to demonstrate that it may improve outcomes.

Methods

All patients who underwent cardiac catheterisation for Melody valve implantation and 3DRAA between August 2013 and February 2015 were reviewed.

Results

31 patients had 3DRAA with balloon sizing. Ten were deemed not Melody candidates (5 coronary compression, 2 aortic root distortion with cusp flattening, 2 RVOT was too large, and 1 had complex branch stenosis and a short landing zone). Of the 21 patients who were Melody candidates, 12 had conduits, 6 prosthetic valves and 3 native RVOTs. In patients with conduits, the technique of stenting the conduit prior to dilation was used after measuring the distance between the conduit and the coronary arteries on 3DRAA. In the Melody patients, we had 100% procedural success and no serious adverse events (coronary compression, tears, stent fracture or endocarditis).

Conclusion

As a tool for case selection, 3DRAA may facilitate higher procedural success and decreased risk of serious adverse events. Furthermore, 3D rotational angiography allows stenting of the conduit prior to dilation, which may prevent tears and possibly endocarditis.