Neuroblastoma is a disease in which malignant (cancer) cells form in nerve tissue of the adrenal gland.

Definition

Neuroblastoma is a disease in which malignant (cancer) cells form in nerve tissue of the adrenal gland, neck, chest, or spinal cord. Neuroblastoma often begins in the nerve tissue of the adrenal glands. There are two adrenal glands, one on top of each kidney in the back of the upper abdomen.

The adrenal glands produce important hormones that help control heart rate, blood pressure, blood sugar, and the way the body reacts to stress. Neuroblastoma may also begin in the chest, in nerve tissue near the spine in the neck, or in the spinal cord.

Neuroblastoma most often begins during early childhood, usually in children younger than 5 years. It sometimes forms before birth but is usually found later, when the tumor begins to grow and cause symptoms. In rare cases, neuroblastoma may be found before birth by fetal ultrasound. By the time neuroblastoma is diagnosed, cancer has usually metastasized (spread), most often to the lymph nodes, bones, bone marrow, liver, and skin.

Epidemiology

The incidence in other industrialized nations appears to be similar to that observed in the United States. International reports have shown that the incidence rates of neuroblastoma are highest among high-income countries in Europe and North America, and lower in low-income countries in Africa, Asia, and Latin America. No published data are available on the incidence in the emerging high-income countries of Asia.

Stages of Neuroblastoma          

The stage of a cancer describes how far it has grown or spread. The stage is important because it helps determine the best treatment. There are four main stages of neuroblastoma cancers:

Stage 1- The tumour is in one area of the body and hasn’t spread. It can be completely removed with surgery.

Stage 2- is divided into 2A and 2B. In 2A, the tumour can’t be completely removed with surgery because of its size or position, but there are no cancer cells in any lymph nodes. In stage 2B, it may or may not be possible to completely remove the tumour, but it has spread to nearby lymph nodes.

Stage 3- The tumour can’t be completely removed with surgery and there is either tumour on both sides of the body (either side of the spine) or there is tumour on one side of the body and lymph nodes containing cancer cells on the other.

Stage 4- The cancer has spread to parts of the body that are some distance from where it started.

Stage 4S- is a special case, as it has a better outlook than other stages. Stage 4S means the child is younger than one year at diagnosis. The tumour may have spread to the liver or skin, but not to the bones. And no more than 10% of cells in the bone marrow are neuroblastoma cells.

Children with stage 4S disease almost always get better with very little treatment or none at all. These tumours either regress spontaneously or after chemotherapy success, which is only given if the tumour is causing symptoms. They disappear completely or develop into a non-cancerous (benign) tumour, called a ganglioneuroma. Many of these children, after their initial diagnostic tests and staging investigations, will just need careful monitoring for some years.

A newer staging system has been developed by the International Neuroblastoma Risk Group (INRG). This system looks at whether or not certain ‘image-defined risk factors’ are present in a neuroblastoma tumour before treatment. These risk factors can be detected by scans and help doctors understand the extent of the disease.

Stage L1– The tumour is localised and has not spread into vital structures nearby. It can be removed by surgery

Stage L2– The tumour is localised but has ‘image-defined risk factors’ and can’t be safely removed by surgery

 Stage M– The tumour has spread to other parts of the body

Stage MS- The tumour has spread to the skin, liver and/or the bone marrow in children younger than 18 months old. If the cancer has spread to distant parts of the body, this is known as secondary or metastatic cancer

Neuroblastoma risk factors

A “risk factor” is something that may impact your child’s chances of developing cancer. For example, some types of adult cancers may be linked to lifestyle choices, such as smoking, or environmental factors. However, there are no known risk factors linked to lifestyle or environment for most forms of childhood cancer, including neuroblastoma.

The two biggest risk factors for neuroblastoma are age and heredity:

Age: Most causes of neuroblastoma are diagnosed in children between the ages of one and two, and 90% are diagnosed before the age of 5.

Heredity: 1% to 2% of neuroblastoma cases seem to be the result of a gene inherited from a parent. Children with familial neuroblastoma usually have one or more family members who also had neuroblastoma as infants.

However, it is important to remember that neuroblastoma is an extremely rare disease, with only about 700 cases diagnosed each year.

Scientists also believe that having birth defects, or congenital anomalies, may also be a risk factor for neuroblastoma. Although the link is not yet clearly understood, scientists believe that genetic mutations in the DNA that cause birth defects may also increase the likelihood of abnormal cell development leading to the development of childhood cancer. Again, however, it is important to note that neuroblastoma is extremely rare and most children with a congenital abnormality will not develop neuroblastoma.

Causes

The exact cause of the disease is not fully understood. Various studies on neuroblastoma in children focus on factors that prevent neuroblasts from maturing properly during gestation and conception. Previous research has ruled out environmental factors such as smoking.

Use of medicinal drugs during pregnancy, and exposure to specific chemicals as possible causes of the condition. Other studies that examined its possible link with the use of fertility drugs and exposure to infection early in life are inconclusive.

However, scientists agree that gene mutations and congenital syndromes may play a part in the development of the disease.

About 2% of patients have close family members with neuroblastoma. Familial neuroblastoma can be caused by mutations in the anaplastic lymphoma kinase,

KIF1B, and PHOX2A genes. Also, a number of neuroblastoma patients have been diagnosed with congenital central hypoventilation syndrome, a breathing control disorder.

Neuroblastoma symptoms

Many symptoms of neuroblastoma are caused by pressure from the tumor or bone pain if the cancer has spread to the bones. Bone pain may cause the child to limp, refuse to walk, or become unable to walk. Other symptoms may include:

A lump or mass in the abdomen, chest, neck, or pelvis

Skin lesions or nodules under the skin with blue or purple patches

Eyes that bulge out and dark circles under the eyes, if cancer has spread behind the eyes

Changes in the eyes, such as black eyes, a droopy eyelid, a pupil that is constricted, vision problems, or changes in the color of the iris

Pain in the chest, difficulty breathing, or a persistent cough

Pain in the arms, legs, or other bones

Pain in the back, or weakness, numbness, or paralysis of the legs if the tumor has spread to the spinal cord

Fever and anemia, which is a low level of red blood cells

Constant diarrhea or high blood pressure caused by hormones released by the tumor

Rotating movements of the eyes and sudden muscle jerks, likely from immune system problems caused by the disease

Sometimes, children with neuroblastoma do not have any of these changes. Or, the cause of a symptom may be a different medical condition that is not cancer. If you are concerned about any changes your child experiences, please talk with your child’s doctor.

Complications of Neuroblastoma

Complications of neuroblastoma may include:

Spread of the cancer (metastasis): Neuroblastoma may spread (metastasize) to other parts of the body, such as the lymph nodes, bone marrow, liver, skin and bones.

Spinal cord compression: Tumors may grow and press on the spinal cord, causing spinal cord compression. Spinal cord compression may cause pain and paralysis.

Signs and symptoms caused by tumor secretions: Neuroblastoma cells may secrete certain chemicals that irritate other normal tissues, causing signs and symptoms called paraneoplastic syndromes. One paraneoplastic syndrome that occurs rarely in people with neuroblastoma causes rapid eye movements and difficulty with coordination. Another rare syndrome causes abdominal swelling and diarrhea.

Diagnosis and test

Your child will need to have a series of tests to help doctors understand the extent of the disease. Some neuroblastoma tests include:

123I-MIBG scan: A small amount of radioactive iodine (123I), linked to the very specific neuroblastoma chemical MIBG, is injected and an area of the body is viewed through pictures taken 24 hours later.

CT scan (computerized tomography) or MRI scan (magnetic resonance imaging): These scans are done to get pictures of the head, chest, abdomen and/or pelvis to measure the size of the tumor and to look for spread of the disease.

Bone marrow aspirate and biopsy: Bone marrow is removed from the bone (usually the hip bone) either by aspiration (suctioning a small amount through a hollow needle) or by biopsy (cutting out a small piece of bone marrow). This test is generally done under anesthesia.

PET (positron emission tomography) scan: Like an MIBG scan, this scan detects neuroblastoma throughout the body after the injection of a small amount of radiation tagged to a sugar-like compound. This is especially useful in the 10 to 15 percent of cases where MIBG is not a useful diagnostic tool.

Urine VMA/HVA test: This test will determine the amount of a substance called catecholamines that is found in the urine, and is produced and secreted by the tumor.

Tissue biopsy: This test allows us to collect a small sample of the tumor. It is done in the operating room or in the interventional radiology suite. This test can confirm the diagnosis of relapsed neuroblastoma, and can be used to perform genetic testing for specific mutations that might be targets for new drugs. The rationale for and logistics of a biopsy would be discussed with you in detail.

Treatment 

Treatment depends on the patient’s age and how much the disease has spread:

Surgery

Surgery is usually performed if the tumor has not spread to other parts of the body. Surgical removal usually means the patient will remain free of neuroblastoma.

If the tumor affects the spinal cord, chemotherapy is used right away instead of surgery, which could cause paralysis.

Chemotherapy and other medicines may also be used instead of surgery when neuroblastoma has already spread by the time it is diagnosed.

Chemotherapy (“chemo”)

It uses powerful medicines to kill cancer cells or stop them from growing (dividing) and making more cancer cells:

Chemo may be injected into the bloodstream, so that it can travel throughout the body.

Some chemo may be given by mouth.

Combination therapy uses more than one type of chemo at a time.

Radiation therapy may be combined with chemotherapy.

Radiation therapy

It uses high-energy X-rays or other types of radiation to kill cancer cells or stop them from growing:

External radiation uses machines outside the body to deliver the X-ray dose.

Internal radiation uses needles, seeds, wires or catheters (tubes) to deliver the radiation directly into or close to the cancer.

High-risk patients are given more intense treatments. Patients with neuroblastoma are considered high-risk when the tumor cannot be surgically removed and has spread:

To lymph nodes near the tumor;

To other areas near the tumor, but not to other parts of the body; or

To distant lymph nodes in other parts of the body such as bones, bone marrow, liver, skin or other organs.

For high-risk patients, higher-dose chemotherapy and immune therapy combined with a stem cell transplant may be used.

Stem cell transplant

Includes replacing blood-forming cells in the bone marrow that have been killed by chemo and/or radiation therapy:

A stem cell transplant gives the patient new immature blood cells from a donor’s blood or bone marrow. These cells grow into healthy blood cells to replace the ones the patient lost

Some types of stem cell transplants may be called “bone marrow transplants” because the cells come from the donor’s bone marrow

Immune therapy with an antibody called GD2 along with GM-CSF and Il-2 is given at the end of therapy

Prevention of Neuroblastoma

The risk of many adult cancers can be reduced with certain lifestyle changes (such as staying at a healthy weight or quitting smoking), but at this time there are no known ways to prevent most cancers in children.

The only known risk factors for neuroblastoma (age and heredity) cannot be changed. There are no known lifestyle-related or environmental causes of neuroblastomas at this time.

Some studies suggest that having mothers take prenatal multi-vitamins or folic acid might lower the risk of neuroblastoma, but further research is needed to confirm this. Getting care from a doctor during pregnancy is always an important thing to do for the health of your baby.

If there is a history of neuroblastoma in your family, you may want to talk with a genetic counselor about your children’s risks of developing the disease. It is important to remember, though, that familial neuroblastoma is very rare.

Excessive, uncontrollable sweating of the hands or palms, is called palmar hyperhidrosis.

Description

Excessive, uncontrollable sweating of the hands or palms, is called palmar hyperhidrosis.

This medical condition is an extremely stressful, embarrassing, and confidence-wrecking problem. From ruined paperwork to slippery handshakes, sweaty palms can negatively impact your social life, education, and career.

It’s proven and published that palmar hyperhidrosis has the most significant negative impact on patients’ lives of any dermatologic condition. But there are treatments available, the treatments work, and you have a number of choices.

Pathophysiology

Generalized hyperhidrosis may be the consequence of autonomic dysregulation, or it may develop secondary to a metabolic disorder, febrile illness, or malignancy.

In its localized form, hyperhidrosis may result from a disruption followed by abnormal regeneration of sympathetic nerves or a localized abnormality in the number or distribution of the eccrine glands, or it may be associated with other (usually vascular) abnormalities.

Essential hyperhidrosis, a disorder of the eccrine sweat glands, is associated with sympathetic overactivity. Essential hyperhidrosis does not appear to be a generalized disorder involving vascular endothelium. Palmoplantar hyperhidrosis may be inherited in an autosomal dominant manner.

Causes and risk factors

Sweaty palms occurs due to overactive sweat glands, and there are many reasons for this to occur.

It can run in families, and it can be associated with other forms of hyperhidrosis or with certain medical conditions.

Associated conditions include:

Anxiety

Infections

Prescription medications

Substance abuse

Heart disease,

Hyperthyroidism

Lung disease

Acromegaly (a condition in which the pituitary gland produces too much growth hormone)

Menopause

Parkinson’s disease

Glucose disorders

Tuberculosis

Stroke

Pheochromocytoma (a tumor in the adrenal gland)

Carcinoid syndrome (a disease that can occur when a carcinoid tumor found in the small intestines, pancreas, liver, or stomach)

The condition affects both sexes equally, but females may be more likely to seek treatment for sweaty palms.

Symptoms of Palmar Hyperhidrosis

Symptoms of hyperhidrosis of the hands may include:

Excessively clammy and sweaty hands

Hands that are cold to the touch

Pale or blush coloring of the hands

Sweating in the hands from everyday tasks such as writing with a pencil or holding hands with someone

Abrupt onset of sweating episodes

Fear of situations that require hand contact

Swelling of the fingers

What are the complications of Palmar Hyperhidrosis?

Over time, excessive sweating could put you at an increased risk of developing a skin infection. Hyperhidrosis can also impact your mental health.

You may find yourself changing how you act to hide your symptoms from others. Constant sweating may be so severe that you avoid routine actions (such as lifting your arms or shaking hands). You may even give up activities you enjoy to avoid problems or embarrassment from excessive sweating.

In some cases, extreme sweating may be due to a severe and life-threatening problem. See a healthcare provider right away if you ever have chest pain or feel queasy or dizzy along with sweating symptoms.

Diagnosis of Palmar Hyperhidrosis

Your doctor will ask you where you experience sweating on your body, the pattern, timing, and whether you have other symptoms such as weight loss, fever, appetite, and hormone levels.

If you don’t have a medical condition as the cause of your sweaty palms, your doctor may do a diagnostic test for you.

Tests may include:

A starch-iodine test: An iodine solution is applied to the palm and, once dried, sprinkled with starch. In areas of excess sweat, the iodine and starch solution will turn the palms a dark blue color.

A paper test: A doctor places a special type of paper on the palms to absorb the sweat. The paper is then weighed to see how much sweat has accumulated on the palms.2

A diagnosis of primary sweaty palms requires that the sweat must be excessive and lasting six months or longer without a known cause.

Other factors that contribute to the diagnosis include the frequency of sweating (having at least one episode of sweating a week), age (it is most prominent under the age of 25), family history, having sweat occur on both palms, and not experiencing any sweating during sleep (which could be a different condition all together called sleep hyperhidrosis).

How is it palmer hyperhidrosis treated?

Treatment will depend on how severe the sweating is and which previous treatments have been successful.

Topical treatments

Some mild to moderate cases can respond to topical treatments such as aluminium chloride hexahydrate (Driclor).

Driclor should always be applied to skin that is as dry as possible in order to maximise the benefit and minimise potential side effects. Ideally, it should be applied just after a shower prior to bedtime. Dry the area off with a hairdryer on the cool setting then apply Driclor.

Wash your hands first thing in the morning with plain water (no soap). If irritation develops, applying a corticosteroid cream intermittently can be useful (this needs to used be under the guidance of a health professional).

Anticholinergic creams such as glycopyrolate (0.5-3%) can also be effective. A compounding pharmacist can make up these creams. Side effects are uncommon. How often the cream is applied will depend on the individual.

Iontophoresis

Iontophoresis may be considered for cases which do not respond to topical treatments. Treatments are individualised. The affected area is immersed in tap water, salty water or glycopyrrolate solution. Then a gentle electric current is passed across the skin surface for 10 to 20 minutes.

Glycopyrrolate iontophoresis has the highest success rate – up to 80% of affected people respond well to this treatment. The time between treatments will vary. Treatments will need to be repeated every 2 to14 days.

Botulinum toxin type A (botox) injections

Botox injections can treat sweaty hands safely and effectively. However, the treatment is not subsidised under Medicare-PBS and the cost limits its wider use.

Most treatment is performed under a nerve block. Approximately 100 units of botox will be needed in each hand. The treatment is usually effective for 3 to 5 months. Side effects include temporary muscle weakness.

Oral medications

Anticholinergic tablets (such as oxybutynin and propantheline bromide) can be useful in treating palmar hyperhidrosis. However, side effects such as constipation, dry mouth and drowsiness are common.

Other medications reported to be useful include oral glycopyrrolate (not available in Australia), propranolol, clonazepam and gabapentin.

Medications can be a viable short-term option for several days to weeks and give people a “break” from their sweating.

Surgery

Endoscopic thoracic sympathectomy (ETS) is a surgical treatment option for various forms of hyperhidrosis. It is conducted by a vascular or neurosurgeon under general anaesthesia. It has a very high success rate for treating palmar hyperhidrosis but carries a significant risk of compensatory or “rebound” sweating. Compensatory sweating occurs in areas such as the back or lower limbs weeks to months after ETS surgery. Compensatory hyperhidrosis can be difficult to treat and usually persists for life.

An in-depth discussion with your vascular surgeon or neurologist is needed prior to considering ETS surgery.

Natural Remedies of Palmar Hyperhidrosis:

Some changes in the lifestyle and daily activities may help in improving the Palmoplantar hyperhidrosis symptoms like:

Antiperspirants: Deodorants may not stop sweating; however, antiperspirants sprays may help people suffering from Palmoplantar hyperhidrosis to deal with it naturally. Some of the prescribed antiperspirants are aluminum chloride that may plug out the sweat glands.

Armpit Shields: Pads can be worn in armpits for protecting the garment from getting wet.

Shoes: Synthetic material shoes can worsen the symptoms and so the natural materials like leather are normally recommended.

Clothing: The synthetic fibers in cloth like nylon can worsen the symptoms and so loose cotton clothing is advised.

Socks can absorb the moisture in a better way if made up of natural fibers like cotton.

Coping

Dealing with sweaty palms can be stressful for some people. If you’re feeling anxious in everyday situations—such as holding or shaking hands, filing papers, or writing, it can cause social distress and embarrassment. Stress and anxiety can cause you to sweat, which may exacerbate the problem.

In addition to medical treatments, it’s often recommended to consider psychotherapy to help learn techniques and tools to cope.

Psychotherapy and cognitive behavioral therapy may help you learn how to successfully reduce the release of cortisol in your body (a hormone that often increases in times of stress). You may also adopt social skills to help you cope in your day-to-day life as you continue to get treatment for sweaty palms.

Pericarditis is an inflammation of the pericardium.

Definition

Pericarditis is an inflammation of the pericardium. Pericarditis is usually acute it develops suddenly and may last up to several months.

The condition usually clears up after 3 months, but sometimes attacks can come and go for years.

When you have pericarditis, the membrane around your heart is red and swollen, like the skin around a cut that becomes inflamed. Sometimes there is extra fluid in the space between the pericardial layers, which is called pericardial effusion. Pericarditis can affect anyone, but it is most common in men aged 16 to 65.

Types

There are several types of pericarditis, and each one of them is identified by different symptoms.

Acute pericarditis: This type of pericarditis starts suddenly, but patients are likely to observe pain and other signs for two to three weeks. However, the possibility of it occurring again is likely. It can be difficult to evaluate the difference between the pain because of a heart attack and acute pericarditis.

Recurrent pericarditis: After you have faced an episode of acute pericarditis, there is a possibility of recurrent pericarditis. It occurs about four to six weeks after you have suffered from acute pericarditis, and you are likely to observe any symptoms in between the time frame.

Incessant pericarditis: This type lasts from four to six weeks and does not  last more than three months. You can observe continuous symptoms like chest pain.

Chronic constrictive pericarditis: This is a type as it develops slowly and has a longer duration than incessant pericarditis, like more than three months.

Causes

Causes of acute pericarditis include

Infection (viral, bacterial, parasitic, or fungal and, in people with AIDS, tuberculosis, or aspergillosis)

Heart attack

Heart surgery (postpericardiotomy syndrome)

Systemic lupus erythematosus (lupus)

Rheumatoid arthritis

Kidney failure

Chest injury

Cancer (such as leukemia, breast cancer, or lung cancer, or, in people with AIDS or Kaposi sarcoma)

Rheumatic fever

Radiation therapy

Drugs, including warfarin and heparin (anticoagulants), penicillin, procainamide (a drug used to treat abnormal heart rhythms), and phenytoin (an antiseizure drug)

Unknown (idiopathic or nonspecific pericarditis)

In people who have AIDS, a number of infections, including tuberculosis and aspergillosis, may result in pericarditis. Pericarditis due to tuberculosis (tuberculous pericarditis) accounts for less than 5% of cases of acute pericarditis in the United States but accounts for the majority of cases in some areas of India and Africa.

After a heart attack, acute pericarditis develops during the first day or two in 10 to 15% of people and after about 10 days to 2 months in 1 to 3% (subacute pericarditis). Subacute pericarditis is caused by the same disorders that cause acute pericarditis.

Symptoms

The following are the most common symptoms of pericarditis. However, each child may experience symptoms differently. Symptoms may include chest pain that:

Can be felt especially behind the breastbone, and sometimes beneath the clavicle (collarbone), neck, and left shoulder

Is a sharp, piercing pain over the center or left side of the chest that increases as the child takes a deep breath

A low-grade fever

Irritability

Fatigue

Loss of appetite

Irregular heartbeat

Children may not be able to describe that they have “chest pain” or be able to explain how they feel. Sometimes, non-specific symptoms such as irritability, loss of appetite, or fatigue will be all that the child is able to express.

The symptoms of pericarditis may resemble other medical conditions or heart problems. Always consult your child’s physician for a diagnosis.

Complications

Some of the dangerous complications of pericarditis include:

Cardiac tamponade – fluid builds up between the two layers of the pericardium. The heart is compressed and can’t function properly. This requires immediate attention and can be fatal.

Abscess A build-up of pus either within the heart or in the pericardium

Spread of infection Aas with any infection, the infection can spread to other areas

Constrictive pericarditis The pericardium is scarred by the inflammation. Scar tissue doesn’t stretch, so the heart can’t function properly.

Diagnosis and test

If your healthcare provider suspects pericarditis, he or she will listen to your heart very carefully. A common sign of pericarditis is a pericardial rub. This is the sound of the pericardium rubbing against the outer layer of your heart. Other chest sounds that are signs of fluid in the pericardium (pericardial effusion) or the lungs (pleural effusion) may also be heard.

Your healthcare provider may also check for pulsus paradoxus. This is when your blood pressure drops when you take a deep breath. The changes in the pressure in your chest keep blood from returning from your body and entering your heart.

Along with a health history and physical exam, you may also need certain tests. These may include:

Echocardiogram (echo): This test uses sound waves to check your heart’s size and shape. The echo sound waves create a picture on a screen as an ultrasound transducer is passed over the skin over the heart. Echo can show how well your heart is working and whether fluid has built up around your heart.

Electrocardiogram (ECG): This test records the strength and timing of the electrical activity of the heart. It shows abnormal rhythms and can sometimes detect heart muscle damage. Small sensors are taped to your skin to pick up the electrical activity.

Chest X-ray: An X-ray may be done to check your lungs and see if your heart is enlarged.

Cardiac MRI: This is an imaging test that takes detailed pictures of the heart. It may be used to look for thickening or other changes in the pericardium.

Cardiac CT: This type of X-ray takes a clear, detailed picture of your heart and pericardium. It may be used to help rule out other causes of chest pain.

Blood tests: Certain blood tests can help rule out other heart problems, such as heart attack, and can tell the doctor how much inflammation there is in your body.