A sarcoma is a rare kind of cancer. Sarcomas are different from the much more common carcinomas because they happen in a different kind of tissue. Sarcomas grow in connective tissue — cells that connect or support other kinds of tissue in your body. These tumors are most common in the bones, muscles, tendons, cartilage, nerves, fat, and blood vessels of your arms and legs, but they can happen anywhere.

Sarcoma Symptoms:

Soft tissue sarcomas are hard to spot, because they can grow anywhere in your body. Most often, the first sign is a painless lump. As the lump gets bigger, it might press against nerves or muscles and make you uncomfortable or give you trouble breathing, or both. There are no tests that can find these tumors before they cause symptoms that you notice.

Osteosarcoma can show obvious early symptoms, including:

• Pain off and on in the affected bone, which may be worse at night
• Swelling, which often starts weeks after the pain
• A limp, if the sarcoma is in your leg

Children and young adults get osteosarcoma more often than adults. And because healthy, active children and teens often have pain and swelling in their arms and legs, osteosarcoma might be mistaken for growing pains or a sports injury. If your child’s pain doesn’t get better, gets worse at night, and is in one arm or leg rather than both, talk to a doctor.

Sarcoma Treatments:

How your sarcoma is treated depends on what type you have, where in your body it is, how developed it is, and whether or not it has spread to other parts of your body, or metastasized.

Surgery takes the tumor out of your body. In most cases of osteosarcoma, the doctor can remove just the cancer cells, and you won’t need your arm or leg removed, too.

Radiation can shrink the tumor before surgery or kill cancer cells that are left after surgery. It could be the main treatment, if surgery isn’t an option.

Chemotherapy drugs can also be used with or instead of surgery. Chemo is often the first treatment when the cancer has spread.

Targeted therapies are newer treatments that use drugs or man made versions of antibodies from the immune system to block the growth of cancer cells while leaving normal cells undamaged.

Surviving Sarcoma:
Most people diagnosed with a soft tissue sarcoma are cured by surgery alone, if the tumor is low-grade; that means it is not likely to spread to other parts of the body. More aggressive sarcomas are harder to treat successfully.

The survival rate for osteosarcoma is between 60% and 80% if the cancer has not spread. It is more likely to be cured if all of the cancer can be removed by surgery.

Schwannomas are benign tumors that arise from the nerve sheath (covering) of cranial nerves along-side the cerebellum and brainstem.

The optimal treatment for the majority of symptomatic schwannomas is maximal surgical removal and/or focused radiation therapy (radiosurgery). Fortunately, for patients requiring schwannoma surgery, most large vestibular (acoustic) schwannomas can be removed through a retromastoid keyhole craniotomy while most trigeminal schwannomas can be removed through either a retromastoid approach or an endonasal endoscopic approach.

These tumors are typically benign and arise from the nerve sheath (covering) of cranial nerves along-side the cerebellum and brainstem. The most common schwannoma arises from the 8th cranial nerve (the vestibulo-cochlear nerve) or the 5th cranial nerve (the trigeminal nerve). In some instances, schwannomas are related to a genetic syndrome called Neurofibromatosis. Bilateral vestibular schwannomas are associated with NF-2.

 

What Are Gallstones?

They aren’t really stones. They’re pieces of solid material that form in the gallbladder, a small organ located under the liver.

You might not even know you have them until they block a bile duct, causing pain that you need to get treated right away.

Types:
The two main kinds are:Cholesterol stones. These are usually yellow-green in color. They’re the most common kind, accounting for 80% of gallstones.

Pigment stones. These stones are smaller and darker. They’re made up of bilirubin, which comes from bile, a fluid your liver makes and your gallbladder stores.

 

What Causes Gallstones?

There may be several reasons, including:

• Your genes
• Your weight
• Problems with your gallbladder
• Diet

Bile can be part of the problem. Your body needs bile, but if it has too much cholesterol in it, that makes gallstones more likely.

It can also happen if your gallbladder can’t empty properly.

Pigment stones are more common in people with certain medical conditions, such as cirrhosis (a liver disease) or blood diseases such as sickle cell anemia.

What are  Risk factors?

You’re more likely to get gallstones if:

You’re obese. This is one of the biggest risk factors. Obesity can raise your cholesterol level and also make it harder for the gallbladder to empty completely.

You take birth control pills, hormone replacement therapy for menopause symptoms, or are pregnant. The extra estrogen is the problem. It can increase cholesterol and make it harder for the gallbladder to empty.

You have diabetes. People with this condition tend to have higher levels of triglycerides (a type of blood fat), which is a risk factor for gallstones.

You take medicine to lower your cholesterol. Some of these drugs boost the amount of cholesterol in bile, which may increase your chances of getting cholesterol stones.

You lost weight too quickly. Your liver makes extra cholesterol, which may lead to gallstones.

You’re fasting. Your gallbladder may not squeeze as much.

What Are the Symptoms?

You might not notice anything, or even know you have gallstones, unless your doctor tells you. But if you do get symptoms, they usually include:

• Pain in your upper belly and upper back that can last for several hours
• Nausea
• Vomiting
• Other digestive problems, including bloating, indigestion and heartburn, and gas

What’s the Treatment?

Many people with gallstones get surgery to take out the gallbladder. There are two different kinds of operations.

Laparoscopic cholecystectomy. This is the more common procedure. The surgeon passes instruments, a light, and a camera through several small cuts in the belly. He views the inside of the body on a video monitor. Afterward, you spend the night in the hospital.

Open cholecystectomy. The surgeon makes bigger cuts in the belly to remove the gallbladder. You stay in the hospital for a few days after the operation.

If gallstones are in your bile ducts, the doctor may use ERCP to find and remove them before or during gallbladder surgery.

Ischaemic myelopathy is a condition where the blood supply to part of the spinal cord (the bundle of nerves that run inside the spine) is suddenly interrupted by a ‘clot’ that blocks a small artery (blood vessel). This is similar to a stroke or heart attack in people, except that it is the spinal cord that is affected rather than the brain or heart.

‘Ischaemia’ is a loss of blood supply, and ‘myelopathy’ is a form of damage to the nerves of the spinal cord. The cause of the blockage of the spinal cord blood vessels is poorly understood. In the majority of cases the ‘clot’ which blocks the artery is similar in structure to the material that forms the discs (the cushions between the bones of the spine). This disc material is called ‘fibro-cartilage’. It is assumed that some of this disc material somehow moves from its normal location to become lodged in the blood vessels of the spinal cord. This is the reason why the condition is commonly referred to as‘fibrocartilaginous embolism’ (shortened to ‘FCE’).

Other less common reasons why the blood supply to the spinal cord can be interrupted include either an abscess or a tumour, either of which can press on important blood vessels and prevent blood flow to the nerves.

 

What are the common signs of ischaemic myelopathy?

Ischaemic myelopathy most commonly occurs in large breed dogs, although it is also encountered in smaller breeds and, very occasionally, in cats. The onset of neurological signs is always extremely rapid and often occurs during vigorous exercise. Neurological abnormalities may vary from mild weakness or incoordination, through to an inability to walk. Paralysis, incontinence and inability to feel pain are possible in severe cases. Ischaemic myelopathy is a non-painful condition, although at the initial onset some patients may cry out or yelp. Neurological signs do not tend to progress after the first 24 hours.

 

How is ischaemic myelopathy diagnosed?

Ischaemic myelopathy is diagnosed by ruling out other causes of sudden onset neurological signs, such as a ‘slipped disc’ (see information sheets on cervical disc disease and thoracolumbar disc disease), traumatic disc extrusion or a spinal fracture.

Advanced diagnostic imaging investigations are necessary in order to diagnose ischaemic myelopathy and to rule-out other conditions – an MRI scan is the imaging technique of choice in these circumstances. MRI scanning uses high powered magnets and a computer to generate images of the spine (this is the same technique and the same equipment which is used for body scanning in human patients). It provides detailed information on the location and extent of any blood vessel-related injury to the spinal cord. Myelography is an alternative imaging technique which can also be used for investigating spinal injuries. This involves injecting a dye (contrast agent) around the spinal cord and obtaining multiple X-rays to assess the flow of the dye. Injecting around the spinal cord is not without risk of causing further damage to already compromised nerve tissue, however. Unlike an MRI scan, myelography will not show damage to the spinal cord caused by ischaemic myelopathy, although it will enable some other conditions to be ruled out as the cause of the spinal cord injury. MRI scanning is less invasive than myelography, and with less risk of side-effects, and for most patients MRI provides the best option for investigation. Both MRI and myelography require the dog to have a general anaesthetic.

 

MRI scan showing an ischaemic myelopathy (FCE) in the neck (arrow)

 

MRI scan showing an ischaemic myelopathy (FCE) in the back (arrow)

 

How can ischaemic myelopathy be treated?

There is no specific treatment for ischaemic myelopathy. Supportive care and nursing are essential in order to aid recovery. Bedding needs to be well padded in order to prevent bed sores. Regular physiotherapy and, in some cases, swimming at a hydrotherapy unit can help function to be regained and assist in building strength. Some patients may require a catheter to be placed in the bladder temporarily to aid urination.

What is the outcome for patients with ischaemic myelopathy?

The outcome in dogs with ischaemic myelopathy depends on two key factors – a) the location and b) the severity of the spinal cord injury. The findings on the neurological examination and MRI investigations can help to predict the chances of recovery. Dogs that are paralysed and also cannot feel pain in their limbs are unlikely to recover, unfortunately. However, the majority of less severely affected dogs will gradually improve over a period of weeks to months, to a point where they can freely exercise and have a good quality of life. A degree of weakness in one or more limbs may persist in some dogs. Recurrence of ischaemic myelopathy is very uncommon.

 

• Hyperglycemia is an abnormally high blood glucose (blood sugar) level.
• Hyperglycemia is a hallmark sign of diabetes (bothtype 1 diabetes and type 2 diabetes) and prediabetes.
• Diabetes is the most common cause of hyperglycemia.
• Other conditions that can cause hyperglycemia arepancreatitis, Cushing’s syndrome, unusual hormone-secreting tumors, pancreatic cancer, certain medications, and severe illnesses.
• The main symptoms of hyperglycemia are increased thirst and a frequent need to urinate.
• Severely elevated glucose levels can result in a medical emergency like diabetic ketoacidosis (DKA) or hyperglycemic hyperosmolar nonketotic syndrome (HHNS, also referred to as hyperglycemic hyperosmolar state).
• Insulin is the treatment of choice for people with type 1 diabetes and for life-threatening increases in glucose levels.
• People with type 2 diabetes may be managed with a combination of different oral and injectable medications.
• Hyperglycemia due to medical conditions other than diabetes is generally treated by treating the underlying condition responsible for the elevated glucose

 

Diabetic Ketoacidosis Symptoms

People with diabetes don’t have the luxury of that auto-sensing. Not enough insulin and the glucose levels in the blood stream start to rise; too much insulin, and they plummet.

The consequences of hypoglycemia are easy to understand. No energy source, no function – and the first organ to go is the brain. It needs glucose to function and without it, the brain shuts down quickly. Confusion, lethargy, and coma occur quickly. Blood sugar is one of the first things checked on scene of a comatose patient, because it’s so easy to fix and very embarrassing for an EMT to miss.

A number of medical conditions can cause hyperglycemia, but the most common by far is diabetes mellitus. Diabetes affects over 8% of the total U.S. population. In diabetes, blood glucose levels rise either because there is an insufficient amount of insulin in the body or the body cannot use insulin well. Normally, the pancreas releases insulin after a meal so that the cells of the body can utilize glucose for fuel. This keeps blood glucose levels in the normal range.

Type 1 diabetes is responsible for about 5% of all cases of diabetes and results from damage to the insulin-secreting cells of the pancreas. Type 2 diabetes is far more common and is related to the body’s inability to effectively use insulin. In addition to type 1 and type 2, gestational diabetes is a form of diabetes that develops in pregnant women. Studies show that between 2% to 10% of all pregnant women get gestational diabetes.

Sometimes, hyperglycemia is not the result of diabetes. Other medical conditions that can cause hyperglycemia include:

• Pancreatitis (inflammation of the pancreas)
• Pancreatic cancer
• Hyperthyroidism (overactive thyroid gland)
• Cushing’s syndrome (elevated blood cortisol level)
• Unusual tumors that secrete hormones, including glucagonoma,pheochromocytoma, or growth hormone-secreting tumors
• Severe stresses on the body, such as heart attack, stroke, trauma, or severe illnesses, can temporarily lead to hyperglycemia
• Taking certain medications, including prednisone, estrogens, beta-blockers, glucagon, oral contraceptives, phenothiazines, and others, can elevate blood glucose levels

Mild or transient hyperglycemia may not need medical treatment, depending upon the cause. People with mildly elevated glucose or prediabetes can often lower their glucose levels by incorporating diet and lifestyle changes. To assure that you chose the right dietary and lifestyle changes , you should speak with your health care professional or use reliable resources such as the American Diabetes Association.

Insulin is the treatment of choice for people with type 1 diabetes and for life-threatening increases in glucose levels. People with type 2 diabetes may be managed with a combination of different oral and injectable medications. Some people with type 2 diabetes also take insulin.

Hyperglycemia due to medical conditions other than diabetes is generally treated by addressing the underlying condition responsible for the elevated glucose. In some cases, insulin may be needed to stabilize glucose levels during this treatment.

Types of Dementia

Dementia is a general term for loss of memory and other mental abilities severe enough to interfere with daily life. It is caused by physical changes in the brain.

 

• Alzheimer’s disease
• Vascular dementia
• Dementia with Lewy bodies (DLB)
• Mixed dementia
• Parkinson’s disease
• Frontotemporal dementia
• Creutzfeldt-Jakob disease
• Normal pressure hydrocephalus
• Huntington’s disease
• Wernicke-Korsakoff Syndrome

 

Alzheimer’s disease

Most common type of dementia; accounts for an estimated 60 to 80 percent of cases.

Symptoms: Difficulty remembering recent conversations, names or events is often an early clinical symptom; apathy and depression are also often early symptoms. Later symptoms include impaired communication, poor judgment, disorientation, confusion, behavior changes and difficulty speaking, swallowing and walking.

Revised criteria and guidelines for diagnosing Alzheimer’s were published in 2011 recommending that Alzheimer’s be considered a slowly progressive brain disease that begins well before symptoms emerge.

Brain changes: Hallmark abnormalities are deposits of the protein fragment beta-amyloid (plaques) and twisted strands of the protein tau (tangles) as well as evidence of nerve cell damage and death in the brain.

 

Vascular dementia:

Previously known as multi-infarct or post-stroke dementia, vascular dementia is less common as a sole cause of dementia than Alzheimer’s, accounting for about 10 percent of dementia cases.

Symptoms: Impaired judgment or ability to make decisions, plan or organize is more likely to be the initial symptom, as opposed to the memory loss often associated with the initial symptoms of Alzheimer’s. Occurs from blood vessel blockage or damage leading to infarcts (strokes) or bleeding in the brain. The location, number and size of the brain injury determines how the individual’s thinking and physical functioning are affected.

Brain changes: Brain imaging can often detect blood vessel problems implicated in vascular dementia. In the past, evidence for vascular dementia was used to exclude a diagnosis of Alzheimer’s disease (and vice versa). That practice is no longer considered consistent with pathologic evidence, which shows that the brain changes of several types of dementia can be present simultaneously. When any two or more types of dementia are present at the same time, the individual is considered to have “mixed dementia” (see entry below).

 

Dementia with Lewy bodies (DLB):

Symptoms: People with dementia with Lewy bodies often have memory loss and thinking problems common in Alzheimer’s, but are more likely than people with Alzheimer’s to have initial or early symptoms such as sleep disturbances, well-formed visual hallucinations, and slowness, gait imbalance or other parkinsonian movement features.

Brain changes: Lewy bodies are abnormal aggregations (or clumps) of the protein alpha-synuclein. When they develop in a part of the brain called the cortex, dementia can result. Alpha-synuclein also aggregates in the brains of people with Parkinson’s disease, but the aggregates may appear in a pattern that is different from dementia with Lewy bodies.

The brain changes of dementia with Lewy bodies alone can cause dementia, or they can be present at the same time as the brain changes of Alzheimer’s disease and/or vascular dementia, with each abnormality contributing to the development of dementia. When this happens, the individual is said to have “mixed dementia.”

 

Mixed dementia:

In mixed dementia abnormalities linked to more than one cause of dementia occur simultaneously in the brain. Recent studies suggest that mixed dementia is more common than previously thought.

Brain changes: Characterized by the hallmark abnormalities of more than one cause of dementia —most commonly, Alzheimer’s and vascular dementia, but also other types, such as dementia with Lewy bodies.

 

Parkinson’s disease:

As Parkinson’s disease progresses, it often results in a progressive dementia similar to dementia with Lewy bodies or Alzheimer’s.

Symptoms: Problems with movement are common symptoms of the disease. If dementia develops, symptoms are often similar to dementia with Lewy bodies.

Brain changes: Alpha-synuclein clumps are likely to begin in an area deep in the brain called the substantia nigra. These clumps are thought to cause degeneration of the nerve cells that produce dopamine.

 

Frontotemporal dementia:

Includes dementias such as behavioral variant FTD (bvFTD), primary progressive aphasia, Pick’s disease, corticobasal degeneration and progressive supranuclear palsy.

Symptoms: Typical symptoms include changes in personality and behavior and difficulty with language. Nerve cells in the front and side regions of the brain are especially affected.

Brain changes: No distinguishing microscopic abnormality is linked to all cases. People with FTD generally develop symptoms at a younger age (at about age 60) and survive for fewer years than those with Alzheimer’s.

 

Creutzfeldt-Jakob disease:

CJD is the most common human form of a group of rare, fatal brain disorders affecting people and certain other mammals. Variant CJD (“mad cow disease”) occurs in cattle, and has been transmitted to people under certain circumstances.

Symptoms: Rapidly fatal disorder that impairs memory and coordination and causes behavior changes.

Brain changes: Results from misfolded prion protein that causes a “domino effect” in which prion protein throughout the brain misfolds and thus malfunctions.

 

Normal pressure hydrocephalus:

Symptoms: Symptoms include difficulty walking, memory loss and inability to control urination.

Brain changes: Caused by the buildup of fluid in the brain. Can sometimes be corrected with surgical installation of a shunt in the brain to drain excess fluid.

 

Huntington’s Disease:

Huntington’s disease is a progressive brain disorder caused by a single defective gene on chromosome 4.

Symptoms: Include abnormal involuntary movements, a severe decline in thinking and reasoning skills, and irritability, depression and other mood changes.

Brain changes: The gene defect causes abnormalities in a brain protein that, over time, lead to worsening symptoms.

 

Wernicke-Korsakoff Syndrome:

Korsakoff syndrome is a chronic memory disorder caused by severe deficiency of thiamine (vitamin B-1). The most common cause is alcohol misuse.

Symptoms: Memory problems may be strikingly severe while other thinking and social skills seem relatively unaffected.

Brain changes: Thiamine helps brain cells produce energy from sugar. When thiamine levels fall too low, brain cells cannot generate enough energy to function properly.

During a routine Scale and Polish at Smiles Dental, your hygienist will thoroughly clean all deposits and stains from teeth. They will scale away tartar build-up from tooth surfaces and polish your teeth using special dental instruments. You can feel tartar build up on the backs of your teeth with your tongue.

Ultrasonic scalers are used to remove calculus rapidly from the tooth surface. The scaling tip vibrates in the ultrasonic range of 20-45 kHz (i.e. 20,000 to 45,000 times per second), with an optimum frequency between 18 kHz and 32kHz.

Treatment for iron-deficiency anemia will depend on its cause and severity. Treatments may include dietary changes and supplements, medicines, and surgery.

Brain stem tumors are perhaps the most dreaded cancers in pediatric oncology, owing to their historically poor prognosis, yet they remain an area of intense research.  Brain stem tumors account for about 10 to 15% of childhood brain tumors.  Peak incidence for these tumors occurs around age 6 to 9 years.  The term brain stem glioma is often used interchangeably with brain stem tumor.  More precisely, glioma encompasses tumor pathology types such as ganglioglioma, pilcytic astrocytoma, diffuse astrocytoma, anaplastic astrocytoma, and glioblastoma multiforme.

Rarely, other tumor pathologies such as atypical teratoid/rhabdoid tumor (ATRT), primitive neuroectodermal tumor (PNET)/embryonal tumor, and hemangioblastoma occur at the brain stem. These entities are quite different from brain stem gliomas, and the following comments do not apply.

Classification:   Brain stem gliomas have been grouped in the past according to their pathology and location within the brain stem. Terms found in the medical literature include diffuse intrinsic gliomas, midbrain tumors, tectal gliomas, pencil gliomas, dorsal exophytic brain stem tumors, cervicomedullary tumors, focal gliomas, and cystic tumors.  A simpler way to classify these tumors is by two categories: diffuse intrinsic pontine glioma and focal brain stem glioma.
Symptoms:  Children with DIPG present with ataxia (clumsiness or wobbliness), weakness of a leg and/or arm, double vision, and sometimes headaches, vomiting, tilting of the head, or facial weakness. Double vision (diplopia) is the most common presenting symptom for these tumors.  Symptoms are usually present for 6 months or less at time of diagnosis.  Patients with focal brain stem gliomas may display some of the same symptoms, although not the usual combination of ataxia, weakness, and double vision. Duration of symptoms is often greater than 6 months before the focal brain stem tumor is diagnosed.

Diagnosis:  Throughout the United States, brain magnetic resonance imaging (MRI), with and without gadolinium contrast, remains the “gold standard” for diagnosis of brain stem gliomas.  Biopsy is seldom performed outside specialized biomedical research protocols for DIPG, unless the diagnosis of this tumor is in doubt.  Biopsy may be indicated for brain stem tumors that are focal or atypical, especially when the tumor is progressive or when surgical excision may be possible.

Diffuse intrinsic pontine gliomas (DIPG) insinuate diffusely throughout the normal structures of the pons (the middle portion of the brain stem), sometimes spreading to the midbrain (the upper portion of the brain stem) or the medulla (the bottom portion of the brain stem).  The term diffuse intrinsic glioma is synonymous.  By pathology, these tumors are most often a diffuse (sometimes referred to as fibrillary) astrocytoma (World Health Organization [WHO] grade II) or its higher-grade counterparts, anaplastic astrocytoma (WHO III) and glioblastoma multiforme (WHO IV).  Very rarely these tumors start in the medulla or midbrain.

Focal brain stem gliomas–perhaps 20% or more of brain stem gliomas–include tumors that are more circumscribed, focal, or contained at the brain stem.  These tumors may have cysts or grow out from the brain stem (i.e., exophytic).  These tumors more often arise in the midbrain or medulla, rather than the pons. Pathology for these tumors is frequently pilocytic astrocytoma (WHO I) or ganglioglioma (WHO I), although rarely diffuse astrocytoma (WHO II).

Treatment:  Since brain stem gliomas are relatively uncommon and require complex management, children with such tumors deserve evaluation in a comprehensive cancer center where the coordinated services of dedicated pediatric neurosurgeons, child neurologists, pediatric oncologists, radiation oncologists, neuropathologists, and neuroradiologists are available. In particular, for DIPG, because of its rarity and poor prognosis, children and their families should be encouraged to participate in clinical trials attempting to improve survival with innovative therapy.

Neurosurgery:  Surgery to attempt tumor removal is usually not possible or advisable for DIPG. By their very nature, these tumors invade diffusely throughout the brain stem, growing between normal nerve cells. Aggressive surgery would cause severe damage to neural structures vital for arm and leg movement, eye movement, swallowing, breathing, and even consciousness.

Surgery with less than total removal can be performed for many focal brain stem gliomas.  Such surgery often results in quality long-term survival, without administering chemotherapy or radiotherapy immediately after surgery, even when a child has residual tumor.  Surgery is particularly useful for tumors that grow out (exophytic) from the brain stem.

Focal brain stem tumors that arise at the top back of the midbrain (tectal gliomas) should be managed conservatively, without surgical removal. Nevertheless, shunt placement or ventriculostomy for hydrocephalus (see below) is frequently necessary. These tumors have been described to be stable for many years or decades without any intervention other than shunting.

Radiotherapy:  Conventional radiotherapy, limited to the involved area of tumor, is the mainstay of treatment for DIPG.  A total radiation dosage ranging from 5400 to 6000 cGy, administered in daily fractions of 150 to 200 cGy over 6 weeks, is standard.  Hyperfractionated (twice-daily) radiotherapy was used previously to deliver higher irradiation dosages, but such did not lead to improved survival.  Radiosurgery (e.g., gamma knife, Cyberknife) has no role in the treatment of DIPG.

Chemotherapy and other drug therapies:  The role of chemotherapy in DIPG remains unclear.  Studies to date with chemotherapy have shown little improvement in survival, although efforts (see below) through the Children’s Oncology Group (COG), Pediatric Brain Tumor Consortium (PBTC), and others are underway to explore further the use of chemotherapy and other drugs.  Drugs utilized to increase the effect of radiotherapy (radiosensitizers) have thus far shown no added benefit, but promising new agents are under investigation.  Immunotherapy with beta-interferon and other drugs to modify biologic response have shown disappointing results.  Intensive or high-dose chemotherapy with autologous bone marrow transplant or peripheral blood stem cell rescue has not demonstrated any effectiveness in brain stem gliomas and is not recommended.  Future clinical trials may incorporate medicines to interfere with cellular pathways (signal transfer inhibitors) or other approaches that alter the tumor or its environment.  For more information and a listing of the most up-to date trials, the reader is encouraged to check the websites of the National Institutes of Health clinical trials registry , the National Childhood Cancer Foundation/COG, and the PBTC .

In focal brain stem gliomas, chemotherapy, such as carboplatin/vincristine,  procarbazine/CCNU/vincristine, or temozolomide, may be useful in children whose tumors are progressive and not surgically accessible.  In children younger than age 3 years, chemotherapy may be preferable to radiotherapy because of the effects of irradiation on the developing brain.

Recurrent or Progressive Brain Stem Gliomas: Regrettably, DIPG has a high rate of recurrence or progression. At relapse, a variety of Phase I and Phase II drug trials are available through the national research consortiums COG and PBTC, as well as through individual pediatric institutions. Oral etoposide, temozolomide, and cyclophosphamide are drug options sometimes utilized outside a study.

Prognosis:  DIPG often follows an inexorable course of progression, despite therapy. A large majority of children die within a year of diagnosis. Focal brain stem glioma, however, can carry an exceptional prognosis, with long-term survivals frequently reported.

Other Management Issues:  Shunts: Less than half of children with brain stem tumors will develop obstructive hydrocephalus, requiring a shunt or ventriculostomy, at some time during the course of their illness.  Shunts are simple mechanical tubing devices that divert cerebrospinal fluid trapped in the brain’s ventricles above the tumor to another location in the body, typically the abdomen (peritoneum), as in a ventriculoperitoneal shunt.  A ventriculostomy is the surgical creation of an internal channel, often from the third ventricle to a lower portion of the brain, to allow cerebrospinal fluid to drain beyond the tumor.

Steroids:  Dexamethasone (brand name Decadron) is a steroid drug frequently administered to brain stem tumor patients for the swelling and “tightness” of their tumor at the base of their skull. Dexamethasone must be used sparingly!  Dexamethasone should never be prescribed prophylactically or “just in case.” That is, this steroid is an extremely effective medicine for symptomatic swelling associated with treatment of a brain stem glioma, particularly with radiotherapy.  However, dexamethasone is not necessary unless a child has symptomatic swelling.  Dexamethasone has a number of side effects which include mood changes, insomnia, weight gain, fluid retention, glucose instability, high blood pressure, and increased susceptibility to infection.