A Rare Cancer in Children

It is always heartbreaking when children have cancer. Usually cancer in children affects the blood cells or brain. A very rare cancer in children is rhabdomyosarcoma. Where is this cancer located in a child's body?

Fewer than 60 children are diagnosed with rhabdomyosarcoma in the UK each year. About the same number in the United States. Most of them are younger than 10 years old. It's more common in boys than girls.
Rhabdomyosarcoma is the most common of the soft tissue sarcomas in children. These tumors develop from muscle or fibrous tissue and can grow in any part of the body.
The most common areas of the body to be affected are around the head and neck, the bladder or the testes. Sometimes tumours are also found in a muscle or a limb, in the chest or in the abdominal wall. If the tumour is in the head or neck region, it can occasionally spread into the brain or the fluid around the spinal cord.

What causes this disease is unknown. Children who have rare genetic disorders are more prone to have rhabdommyosarcoma.

                               Image of rhabdomyosarcoma that has been removed from a child's body.

The images of the children with this cancer are very disturbing so they will not be displayed.

The signs and symptoms will depend on the part of the body that's affected by the rhabdomyosarcoma. The most common sign is a swelling or lump.

• If the tumor is in the head area, it can sometimes cause a blockage (obstruction) and a discharge from the nose or throat. Occasionally, an eye may appear swollen and protruding.
• If the tumor is in the abdomen (tummy), your child may have discomfort in the abdomen and difficulty going to the toilet.
• If the tumor is in the bladder, your child may have blood in the urine and difficulty passing urine.  

A variety of tests and investigations may be needed to diagnose a rhabdomyosarcoma. A small operation may be needed to remove a sample from the tumour to be looked at under a microscope. This called a biopsy. It's usually done under a general anaesthetic.
Various tests may be done to check the exact size of the tumour and whether it has spread to any other part of the body. These may include:

• a chest x-ray to check the lungs
• an ultrasound
• CT or MRI scans
• blood and bone marrow tests.

Any tests and investigations that your child needs will be explained to you. The booklet A parent's guide to children's cancer gives details of what the tests and scans involve.

Rhabdomyosarcomas are rare tumours and should be treated at specialist centres. 
Treatment depends upon the size of the tumour, its position within the body, and whether it has spread. Treatment of rhabdomyosarcoma usually includes surgery, radiotherapy or chemotherapy, or a combination of these treatments.

Surgery

If at all possible, surgery will be used to remove the tumour. Chemotherapy, using a combination of drugs, is often given before surgery to shrink the tumour. Radiotherapy may also be given to the area of the tumour, particularly if it cannot be completely removed by surgery.

Chemotherapy

If the tumour cannot be removed with surgery, treatment will usually involve a combination of chemotherapy and radiotherapy. Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells and is usually given every three weeks. It may be given to shrink the tumour before surgery or with radiotherapy when the tumour can't be removed by surgery. The drugs used and the length of treatment depends on the type and stage of the rhabdomyosarcoma.

Radiotherapy

Radiotherapy treats cancer by using high-energy rays, which destroy the cancer cells while doing as little harm as possible to normal cells. It's given to the area where the rhabdomyosarcoma occurs.

Side effects of treatment 

Treatment for rhabdomyosarcoma often causes side effects, and your child’s doctor will discuss this with you before treatment starts. Any possible side effects will depend on the particular treatment being given and the part of the body that's being treated.
Chemotherapy can make your child feel better by relieving the symptoms of the cancer, but it can sometimes have side effects such as feeling sick (nausea) and being sick (vomiting), hair loss, an increased risk of infection, bruising and bleeding, tiredness and diarrhoea.

Late side effects

A small number of children may develop side effects many years after their treatment for a rhabdomyosarcoma. Long-term side effects depend on the type of treatment used, and may include a possible reduction in bone growth, infertility, a change in the way the heart and the kidneys work, and a slight increase in the risk of developing another cancer in later life.
Your child’s doctor or nurse will talk to you about any possible late side effects. There is more detailed information about these late side effects in the booklet A parent’s guide to children’s cancer.

                                        Mayo Clinic Oncologist Explains Rhabdomyosarcoma

                                       Holly's Journey Dealing With This Rare Cancer

http://www.macmillan.org.uk/Cancerinformation/Cancertypes/Childrenscancers/Typesofchildrenscancers/Rhabdomyosarcoma.aspx

Tumors in the Heart

Have you ever known of anyone that had tumors removed from their heart? If you haven't it is because it is not very common.

Human heart cancer health care medicine concept with the inner human organ and red cancer cells forming tumors spreading in the body as a malignant disease that needs chemotherapy or heart surgery. 

Classification and external resources
Heart cancer is an extremely rare form of cancer that is divided into primary tumors of the heart and secondary tumors of the heart.

Most heart tumors are benign myxomas, fibromas, rhabdomyomas, and hamartomas, although malignant sarcomas (such as angiosarcoma or cardiac sarcoma) have been known to occur. In a study of 12,487 autopsies performed in Hong Kong seven cardiac tumors were found, most of which were benign. According to Mayo Clinic: "At Mayo Clinic, on average only one case of heart cancer is seen each year."

                                             Video of a removal of a cardiac tumor.

For more information go to www.mayoclinic.com or
http://en.wikipedia.org/wiki/Heart_cancer

Waldenstrom Macroglobulinemia, A Rare and Complicated Disease

WM is a rare disorder with an incidence of approximately three per million people per year with 1400 new cases diagnosed in the United States each year . The median age at diagnosis is 64 years; less than 1 percent of patients are under 40 years of age, and approximately 60 percent are males. WM is much more common in Caucasians than in other ethnic groups . Specifically, it is uncommon in Blacks and those of Mexican descent who make up approximately 5 percent of cases.
The majority of patients with the histopathologic finding of lymphoplasmacytic lymphoma (LPL) have a circulating monoclonal IgM consistent with the diagnosis of WM. In the past, LPL and WM have been arbitrarily differentiated from each other based on the level of the monoclonal IgM protein. Currently, the preferred terminology in cases of LPL with circulating monoclonal IgM is WM, rather than lymphoplasmacytic lymphoma, regardless of the size of the monoclonal IgM protein.

Waldenström macroglobulinemia, one of the malignant monoclonal gammopathies, is a chronic, indolent, lymphoproliferative disorder.It is characterized by the presence of a high level of a macroglobulin (immunoglobulin M [IgM]), elevated serum viscosity, and the presence of a lymphoplasmacytic infiltrate in the bone marrow. (See Pathophysiology, Etiology, and Workup.)
A clonal disease of B lymphocytes, Waldenström macroglobulinemia is considered to be a lymphoplasmacytic lymphoma, as defined by the Revised European American Lymphoma Classification (REAL) and World Health Organization (WHO) classification.
The clinical manifestations of Waldenström macroglobulinemia result from the presence of the IgM paraprotein and malignant lymphoplasmacytic cell infiltration of the bone marrow and other tissue sites. The clinical presentation is similar to that of multiple myeloma except that organomegaly is common in Waldenström macroglobulinemia and is uncommon in multiple myeloma and  lytic bony disease and renal disease are uncommon in Waldenström macroglobulinemia but are common in multiple myeloma. (See Pathophysiology, Presentation, and Workup.)

Complications

Complications of Waldenström macroglobulinemia include the following:

• Hyperviscosity syndrome
• Visual disturbances secondary to hyperviscosity syndrome
• Diarrhea and malabsorption secondary to gastrointestinal (GI) involvement
• Renal disease (less common)
• Amyloidosis of the heart, kidney, liver, lungs, and joints
• Bleeding manifestations secondary to platelet dysfunction and coagulation factor and fibrinogen abnormalities due to interaction with  plasma IgM
• Raynaud phenomenon secondary to cryoglobulinemia
• Increased predisposition to infection due to B-cell dysfunction (disease related) or T-cell dysfunction (therapy related, particularly after nucleoside analogues)
• Cardiac failure
• Increased incidence of lymphomas, myelodysplasia, and leukemias
•  
   
  Initially this disease may look like Multiple Myeloma, but one can see that extensive testing is required for diagnosis. Of course, a bone marrow biopsy is important so the pathologist can look for abnormalities in the bone marrow tissue.
   

  Blood counts

  The complete blood count (CBC) is a test that measures the levels of red cells, white cells, and platelets in the blood. If the lymphoma cells occupy too much of the bone marrow, these levels will be low.
  

  Quantitative immunoglobulins

  This test measures the blood levels of the different antibodies. There are several different types of antibodies in the blood: IgA, IgE, IgG, and IgM. The levels of these immunoglobulins are measured to see if any are abnormally high or low. In WM the level of IgM is high but the IgG level is often low.
  

  Electrophoresis

  The immunoglobulin produced in WM (IgM) is abnormal because it is monoclonal -- meaning that it is just many copies of the exact same antibody. Serum protein electrophoresis (SPEP) is a test that measures the total amount of immunoglobulins in the blood and finds any abnormal (monoclonal) immunoglobulin. Then, another test, such as immunofixation or immunoelectrophoresis, is used to determine the type of antibody that is abnormal (IgM or some other type). Finding a monoclonal IgM immunoglobulin in the blood is necessary to make a diagnosis of WM. The abnormal protein in WM is known by several different names, including monoclonal immunoglobulin M, IgM protein, IgM spike, IgM paraprotein, and M-spike. Other types of monoclonal immunoglobulins, like IgA or IgG, are seen in different disorders (like multiple myeloma and some lymphomas).
  Sometimes pieces of the IgM protein are excreted by the kidneys into the urine. The procedure used for finding a monoclonal immunoglobulin in the urine is called urine protein electrophoresis (UPEP).
  

  Viscosity

  Viscosity measures how thick the blood is. If the IgM level is too high, it will cause the blood to become thick (viscous) so that it can't flow freely. Think about pouring honey compared to pouring water. If the blood becomes too thick, the brain doesn't get enough blood and oxygen. This can be treated with plasmapheresis (see below).
  

  Cryocrit

  This tests the blood for a cryoglobulin (a protein that causes the blood to clump together in cool temperatures).
  

  Beta-2-microglobulin

  This is another protein produced by the malignant lymphoplasmacytoid cells. This protein itself doesn't cause any problems, but it is a useful indicator of a patient’s prognosis (outlook). High levels mean a poor outlook.
  
  WM is a rare disorder with an incidence of approximately three per million people per year with 1400 new cases diagnosed in the United States each year. The median age at diagnosis is 64 years; less than 1 percent of patients are under 40 years of age, and approximately 60 percent are males . WM is much more common in Caucasians than in other ethnic groups . Specifically, it is uncommon in Blacks and those of Mexican descent who make up approximately 5 percent of cases .
  The majority of patients with the histopathologic finding of lymphoplasmacytic lymphoma (LPL) have a circulating monoclonal IgM consistent with the diagnosis of WM. In the past, LPL and WM have been arbitrarily differentiated from each other based on the level of the monoclonal IgM protein. Currently, the preferred terminology in cases of LPL with circulating monoclonal IgM is WM, rather than lymphoplasmacytic lymphoma, regardless of the size of the monoclonal IgM protein.
  
  
  
• For more information:  www.medscape.com, www.uptodate.com, www.cancer.org