NHÂN CA PSEUDOMYXOMA PERITONEI ở MEDIC

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Pseudomyxoma peritonei (PMP) usually begins as a slow-growing tumour in the appendix, called a Low-Grade Mucinous Appendiceal Neoplasm (LAMN). Rarely, PMP starts in other parts of the bowel, ovary or bladder.

Over time, the tumour produces a jelly-like substance called mucin. This can cause the appendix to swell up like a balloon. The tumour can then break through the wall of the appendix and spread tumour cells into the lining of the tummy (the peritoneum).

The tumour cells and mucin build up in the lining of the tummy, putting pressure on the bowel and causing symptoms. It can be many years before symptoms become obvious. Unlike other cancers, PMP rarely spreads via the lymphatic system or the bloodstream. It usually remains inside the tummy, spreading along its internal surfaces.

Causes of pseudomyxoma peritonei

The cause of PMP is unknown.

Signs and symptoms of pseudomyxoma peritonei

Most people don't have any symptoms for a long time. When symptoms occur they may include any of the following:

• slow increase in waist size
• hernia (a swelling on the abdomen)
• loss of appetite
• unexplained weight gain
• abdominal or pelvic pain
• changes in bowel habits
• appendicitis.

Most people with these symptoms won't have PMP, but it's important to have any symptoms checked by your doctor.

How pseudomyxoma peritonei is diagnosed

PMP can be difficult to diagnose. It may be found during investigations into abdominal symptoms, or it may be discovered during an operation for another problem.

CT (computerised tomography) scan

A CT scan takes a series of x-rays that build up a three-dimensional picture of the inside of the body. The scan is painless. It can help to find where the tumour started and check whether it has spread within the abdomen. It usually takes 10-30 minutes. CT scans use a small amount of radiation, which is very unlikely to harm you and won't harm anyone you come into contact with. You will be asked not to eat or drink for at least four hours before the scan.

You may be given a drink or injection of a dye, which allows particular areas to be seen more clearly. For a few minutes, this may make you feel hot all over. If you are allergic to iodine or have asthma you could have a more serious reaction to the injection, so it's important to let your doctor know beforehand.

Sometimes the pictures from the CT scan are enough to make the diagnosis, but sometimes biopsies or an operation are needed to be sure of the diagnosis of PMP.

Treatment

The treatment of PMP depends on a number of factors. These include how far the tumour has spread and your general health. Some of the standard cancer treatments, such as radiotherapy, aren't suitable for treating PMP. This is because PMP cells aren't sensitive to radiotherapy and they are often spread over too large an area for this treatment.

Surgery

You may be offered surgery| to treat this kind of cancer. There are two types of surgery:

• Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC).
• Debulking surgery.

Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC)

This may be an option for some people. It‘s an intensive treatment that aims to remove the tumour to try to cure PMP. It is also known as the Sugarbaker technique (named after the surgeon who first developed it). It involves removing the lining of the abdomen or organs such as the bowel, omentum (fatty tissue in the tummy) and gallbladder. In women, the womb (uterus) and ovaries may also be removed. About half (50%) of people who have a Sugarbaker operation will need a stoma (colostomy). Most of the stomas are temporary and will be reversed after about six months.

Once the surgeon has removed all or most of the tumour, a heated chemotherapy drug is put in the tummy (hyperthermic intraperitoneal chemotherapy) for 90 minutes during the operation. The combination of the chemotherapy drug and heat aims to kill any tumour cells that are left behind.

This is a major operation and may take up to 10 hours. Afterwards, you’ll be nursed in a critical care unit for several days and will stay in hospital for about 2 weeks. This operation has potentially serious complications and the surgeon will discuss these with you.

The National Institute for Health and Clinical Excellence (NICE)| is an organisation that currently advises doctors on treatments for all types of illness. It has produced guidelines about this type of surgery with intraperitoneal chemotherapy. You can read the guidelines on the NICE website.

It's very important to discuss this operation with specialist doctors, as the Sugarbaker technique is a very complicated procedure and isn't suitable for everyone. It should only be carried out at a specialist centre. There are two in the UK:

• The Basingstoke and North Hampshire Hospital
• The Christie Hospital – Manchester.

Debulking surgery

This is done when it’s not possible to have cytoreductive surgery. It aims to remove as much of the tumour as possible to reduce the symptoms of the cancer. This may involve removing the omentum (fatty tissue in the tummy) and part of the bowel. In women, the womb (uterus) and ovaries may also be removed.

Unfortunately, this surgery will not take away all the tumour cells and the PMP is likely to grow back. Further debulking operations may be needed. However, each operation becomes more difficult to do, with less benefit and more risks of complications each time.

Sometimes, a permanent stoma is needed after debulking surgery. It can help to prevent the bowel from becoming blocked (obstructed). Your specialist nurse can give you more information about looking after a stoma.

Chemotherapy

Chemotherapy| can be used to treat PMP. Some people who can’t have surgery may benefit from chemotherapy. It does not cure the cancer but can be used to slow it down. Research into other treatments for PMP is ongoing and advances are being made. Cancer specialists use clinical trials| to assess new treatments. You may be asked to take part in a clinical trial. Your doctor must discuss the treatment with you so that you have a full understanding of the trial and what it means to take part.

Watchful waiting

For some people, the risks of treatment may outweigh the potential benefits, especially as this can be a slow-growing cancer. If you're in this situation, your specialist may suggest watchful waiting. This involves being monitored closely with regular check-ups. Only if the PMP begins to cause you problems will your specialist discuss starting treatment.

Reviewing information is just one of the ways you could help when you join our Cancer Voices network|.

Content last reviewed: 1 February 2013

Ultrasound Helps Diagnose Lung Congestion in Dialysis Patients

 

 

Asymptomatic lung congestion has been shown to increase dialysis patients’ risks of dying prematurely or experiencing myocardial infarctions or other cardiac events, according to recent research.

The findings also revealed that utilizing lung ultrasound to identify this congestion aids in diagnosing patients at risk. Italian investigators recently measured the degree of lung congestion in 392 dialysis patients by using a very simple and inexpensive technique – lung ultrasound.

Lung congestion due to fluid accumulation is very common among kidney failure patients on dialysis, but it frequently does not cause any symptoms. To see whether such asymptomatic congestion affects dialysis patients’ health, Carmine Zoccali, MD, from Ospedali Riuniti (Reggio Calabria, Italy; www.rc.ibim.cnr.it) and his colleagues published their study findings February 28, 2013, in the  Journal of the American Society of Nephrology (JASN).

Among the major findings (1) Lung ultrasound revealed very severe congestion in 14% of patients and moderate-to-severe lung congestion in 45% of patients. (2) Among those with moderate-to-severe lung congestion, 71% were asymptomatic. (3) Compared with those having slight or no congestion, those with very severe congestion had a 4.2-fold increased risk of dying and a 3.2-fold increased risk of experiencing heart attacks or other cardiac events over a two-year follow-up period. (4) Lastly, asymptomatic lung congestion identified by lung ultrasound was a better predictor of patients’ risk of dying prematurely or experiencing cardiac events than symptoms of heart failure.

By evaluating subclinical pulmonary edema can help better establish dialysis patients’ prognoses, according to the findings.The researchers will soon initiate a clinical trial that will integrate lung fluid measurements by ultrasound and will examine whether dialysis intensification in patients with asymptomatic lung congestion can reduce the risk of heart failure and cardiac events and prevent premature death.

From MII, June 2013

 Background

Advancement in dialysis technology and new drug therapies of uremic complications are major achievements of modern nephrology. As a result of progress in the care of ESRD, a continuous increase in survival of dialysis 4 patients has been documented over the last 13 years in the European Renal Association-European Dialysis Transplant Association (ERA-EDTA) registry (1). Adequate control of fluid balance is a primary goal of dialysis treatment and experience in centres applying strict volume control policies documented a remarkable reduction in mortality in comparison with average mortality rate in well matched cohorts in the USRDS and in the ERA-EDTA Registry (2). Even though specific recommendations in past and current guidelines emphasise the risk of volume overload, the problem still remains pervasive in the dialysis population (3). Unsatisfactory control of volume expansion depends on various reasons encompassing both medical and non-medical factors such as reimbursement of the cost of extra or longer dialyses and other organizational and logistic factors. As to the medical factors, it is widely agreed that the high prevalence of patients with LV dysfunction and heart failure and the lack of simple, non-expensive, bedside techniques that may serve to estimate and monitor parameters of central hemodynamics for guiding the prescription of ultrafiltration (UF) and drug treatment  is a factor of major clinical relevance.

Extra-vascular lung water (LW), a fundamental component of body fluids volume, represents the water content of the lung interstitium which is strictly dependent on the filling pressure of the left ventricle (4; 5). Chest ultrasound (US) has recently emerged as a reliable technique for detecting LW in intensive care patients (6) and in patients with heart failure (7). The basic principle of this technique is that in the presence of excessive LW, the ultrasound beam is efflected by subpleural thickened interlobular septa, a low impedance structure surrounded by air with a high acoustic mismatch. US reflection generates hyperechoic reverberation artefacts between thickened septa and the overlying pleura which are defined “lung comets” (8). These artefacts are easily detected with standard US probes and chest US has been formally validated as a reliable technique to estimate LW in patients with heart diseases (9). This method captures changes in LW which occur across dialysis and the feasibility and repeatability of chest US studies in hemodialysis patients has been recently described (10). However the clinical usefulness of this technique in the everyday care in ESRD patients is still untested and it remains unknown whether systematic application of chest US may translate into better clinical outcomes in these patients. With this background in mind the European Renal and Cardiovascular Medicine (EURECA-m) working group of the ERA-EDTA designed a randomised, multicenter, clinical trial investigating whether a treatment policy based on LW monitoring in haemodialysis patients by chest US is more effective than standard clinical monitoring for reducing death, decompensated heart failure and myocardial infarction and prevent the evolution of LVH and LV dysfunction in patients with myocardial ischemia or heart failure over a 2-year follow-up.

 

This trial will be the first which formally tests a biomarker as a guide the optimize volume control and drug treatment in high risk dialysis patients. Other promising indicators of fluid volume in dialysis patients - such as body impedance analysis (BIA) or cardiac natriuretic peptides - have never been tested into a clinical trial, which is a basic requirement for recommending systematic use of biomarkers in clinical practice.