Melanoma is a type of skin cancer that primarily occurs in adults but also is found occasionally in children. In the United States, approximately 500 children are diagnosed with pediatric melanoma a year according to the National Cancer Institute, and there is evidence that incidence is on the rise.
Melanoma may grow faster in children than in adults. Children may fare better than adults, and if caught early, most do not require treatment beyond surgery. Accurate diagnosis and surgery by an experienced surgeon are vital for better outcomes in children. Among children, sun exposure plays less of a role in the development of melanoma. Researchers believe it is a combination of genetic predisposition and other unknown triggers.
Children with fair skin, freckles or red or blond hair have a higher risk of melanoma. Previous studies have shown that children who have been treated for melanoma are at an increased risk of recurrence later in life
Remember the ABCD’s when studying moles:
If a spot looks suspicious, parents should go to a specialist such as a dermatologist to have the place checked. Most melanomas are diagnosed with a biopsy. Once the biopsy is done, surgeons will perform another surgery to ensure the melanoma is completely removed. Based on the first biopsy sentinel node mapping may be required, and depending on pathology from that biopsy, a lymph node resection may be performed. Misdiagnosis or mistreatment may lead to a higher staging or worse prognosis.
Finding an experienced team that diagnoses and treats children with melanoma is important. A qualified team should include a pediatric oncologist, an experienced melanoma dermatopathologist who knows how to do sentinel node analysis and a surgical oncologist who is experienced in sentinel node mapping and biopsy.
Surgery is the primary form of treatment for pediatric melanoma. Low-stage melanomas just need observation after surgery. Thicker, ulcerated or melanomas that have spread to the lymph nodes may necessitate additional medical treatment including biologic therapy, chemotherapy or both.
Once a child has been diagnosed with melanoma, he/she needs to have lifelong skin checks and follow-up with an experienced melanoma oncologist. Follow-up is important since children with melanoma have a higher risk of getting a second melanoma later in life.
Multiple Endocrine Neoplasia
Multiple endocrine neoplasia (MEN) syndromes received their name because they predispose people to develop tumors of the endocrine glands. The endocrine system is comprised of glands that secrete hormones into the bloodstream that control numerous processes within the body. The endocrine system is instrumental in regulating mood, growth and development, metabolism, as well as sexual function and reproductive processes.
The major glands of the endocrine system affected by the MEN syndromes are the pituitary, thyroid, parathyroids, adrenals and pancreas. Currently, there are two distinct MEN syndromes, MEN1 and MEN2. In some ways, the two syndromes are similar, but there are important differences.
Multiple myeloma is a type of cancer that affects the bone marrow, the body's blood-forming system. The plasma cells (a type of white blood cell) become abnormal and multiply rapidly, interfering with the production of normal blood cells. The overgrowth of malignant cells in the bone marrow can also weaken the bones, especially in the back and ribs, causing pain and bone fractures.
Multiple myeloma is uncommon, affecting about 20,000 people annually in the United States. Although the exact cause is unknown, multiple myeloma can be controlled in most patients, sometimes for many years. The development of new drugs has helped control multiple myeloma in a larger number of patients, and has resulted in longer survival.
People diagnosed with multiple myeloma often don't have any symptoms in the early stages of disease. Signs to look out for include:
Fractures: Bone damage is the most serious concern at the time myeloma is diagnosed. Bone damage occurs because myeloma cells produce substances called cytokines, which can trigger bone cells (osteoclasts) to destroy surrounding bone. When more than 30% of the bone has been destroyed, X-rays will show either a thinning of the bone (osteoporosis) or the presence of dark holes (lytic lesions). The weakened area of bone can break, which is called a pathological fracture. To prevent bone destruction, your doctor may recommend giving you drugs that prevent osteoclasts from destroying bone (e.g., Zometa® or Aredia®).
Bone Pain: This is especially common in the middle and/or lower back, rib cage or hips. The pain can be mild or severe depending on the size of the lesion, the speed with which it has developed and whether or not a fracture or nerve compression has occurred. Typically, movement makes the pain much worse.
Fatigue: Because the myeloma cells crowd out the other blood cells in the bone marrow, the number of red cells in the body decreases. This leads to symptoms of tiredness or fatigue.
Infection: Because the myeloma cells crowd out normal white blood cells, which fight infection, there is a risk of infection. Symptoms of infection depend upon where the infection is located. Pneumonia, bladder or kidney infections, sinusitis and skin infections are common.
Hypercalcemia: A high amount of calcium in the blood stream. When the bone is destroyed, calcium is released into the bloodstream. As the amount increases, the kidneys are unable to get rid of the calcium in the urine. Symptoms of hypercalcemia include thirst, nausea, constipation and mental confusion.
Having one or more of the symptoms listed above does not necessarily mean you have multiple myeloma. However, it is important to discuss any symptoms with your doctor, since they may indicate other health problems.
Since the exact cause is unknown and there are no avoidable risk factors, it is not possible to prevent multiple myeloma at this time. There are no screening tests currently available.
Blood & Urine Tests
Blood and urine tests are needed to determine calcium levels and changes in the level of different abnormal proteins that myeloma produces. In the blood, these proteins are called “paraproteins” and in the urine, they are called “Bence-Jones proteins.” These paraproteins are measured in the blood by a test called serum protein electrophoresis (or SPEP). The Bence-Jones protein is measured in the urine by collecting a 24-hour urine sample and running a urine protein electrophoresis (or UPEP).
If these proteins cannot be detected by serum electrophoresis or by urine electrophoresis, an additional test, called immunofixation (or IFE), is performed to detect even small traces of these abnormal proteins.
Bone Marrow Aspiration
A bone marrow aspiration is used to determine the number of plasma cells present in the bone marrow. Normal bone marrow contains less than 5% plasma cells, whereas in myeloma, plasma cells account for more than 30% of cells being produced. However, it is important to know that multiple myeloma is considered a “spotty” disease, meaning that you can find a spot in your marrow that is packed with myeloma cells and move a few centimeters away and find a spot that is relative clean and free of myeloma cells.
Myeloma can be present as single or multiple tumors in the bone or soft tissue around the bone. These tumors are called plasmacytomas. Direct biopsy of one of these plasmacytomas usually shows 90-100% myeloma cells.
Bone X-rays or surveys will be done to look for lytic lesions or osteoporosis. Other more detailed tests may be required, such as a bone density scan, magnetic resonance imaging (MRI) or positron emission tomography (PET) scan. These tests may find very early or small lytic lesions missed by bone x-rays.
Once multiple myeloma is diagnosed, your doctor will determine the stage of the disease. Staging is a way of determining how much disease is in the body and where it is. The doctor needs this information to decide the best way to treat the cancer. Once staging is determined, it does not change regardless of response to chemotherapy or disease activity.
Multiple myeloma is staged using a system called Durie-Salmon Staging System. The system is based upon the number of bone lesions, the level of protein in the blood and/or urine and the blood calcium level.
In addition, each stage is classified as “A” or "B" depending on kidney function. "A" means there is normal kidney function and “B” means abnormal kidney function. As you would expect, stage IIIB would require more urgent treatment to protect and/or recover kidney function.
Treatment generally depends on the stage of the disease, any previous treatments, and how well the patient has responded to previous treatment. Multiple myeloma is usually treated with chemotherapy, although radiation therapy may also be used to kill myeloma cells or to relieve pain in the spine and other areas. Some patients may require high-dose chemotherapy or radiation followed by a stem cell transplant.
Chemotherapy destroys the myeloma cells directly. Chemotherapy may be given over a period of months. Most often chemotherapy may be received outside the hospital, but at times it may be necessary to receive it in the hospital. The drugs are given in cycles, giving the patient’s immune system and normal cells time to recover. By destroying the cancer cells, chemotherapy can also relieve many of the symptoms of the disease.
This is usually used to treat a specific area where there is bone destruction and pain. Radiation can kill cancer cells more quickly than chemotherapy and has fewer side effects. For this reason, it is often used to get quicker pain relief and control severe bone loss.
Bone Marrow & Stem Cell Transplantation
There are two types of transplants: autologous and allogeneic. These procedures restore the supply of normal blood cells when they have been destroyed by high-dose chemotherapy. In an autologous transplant, patients receive their own stem cells. In an allogeneic transplant, patients receive stem cells from a donor. The donor may be related (usually a matched sibling) or unrelated (non-family) individual.
Stem cells can be collected in two ways:
Therefore, a bone marrow transplant is a transplant where stem cells are collected/harvested directly from the patient or donor. A peripheral blood stem cell transplantation (PBSCT) is a transplant where stem cells are collected through the peripheral bloodstream by pheresis.
Often, myeloma patients who undergo autologous transplants will have their stem cells collected by pheresis because it is easier on the patient and requires a shorter recovery time. Patients will receive two daily injections over a few days, to stimulate the bone marrow and “squeeze” the stem cells from the bone marrow into the peripheral blood. During this process, patients are connected to a machine that will remove their blood and selectively collect their stem cells. Once the stem cells are collected, the blood is returned to the patient. Each pheresis session takes approximately four hours. Depending on how many stem cells are collected with each session, the entire process may take one to three days or more. Collected stem cells are frozen and stored until the patient is ready to receive them.
Chemotherapy destroys the cancer cells, but it will also destroy “good” cells. By collecting the stem cells in advance, intense or high-dose chemotherapy can be given safely. Once the patient receives the high-dose chemotherapy, they receive their collected stem cells back. By infusing (returning) these stem cells, patients are able to recover their blood counts and “bounce back” more quickly.
The most common side effects of high-dose chemotherapy include:
When patients have low blood counts, they are more susceptible to getting infections, suffering from fatigue and bleeding. Although patients may have some side effects during administration of the chemotherapy, they usually feel worse a few days after receiving chemotherapy, when their blood counts drop. During this time, they are monitored closely for any fevers (infections), the need for any blood or platelet transfusions, and the need for fluid and electrolyte replacement.
Allogeneic transplantation for myeloma is not as common because it is associated with a higher risk of death. It is mostly for patients who relapse after an autologous transplant or for those with very aggressive myeloma. When patients receive stem cells from a donor, they are essentially receiving a new immune system that may help fight the myeloma. The high risk of allogeneic transplantation is largely from graft-versus-host disease (GVHD). GVHD occurs when the new bone marrow (the graft) recognizes the tissues of the patient’s body as foreign and reacts against the body. Graft-versus-host disease can vary from mild and temporary, to serious and chronic, or even life threatening. Signs and symptoms include: a rash, dry eyes, dry mouth, nausea, vomiting, diarrhea or liver enzymes abnormalities.
Cancer is a journey that no one needs to take alone. There are many forms of support to help you through every stage: diagnosis, treatment and survivorship. Whether you meet with other cancer survivors like yourself, use complementary therapies or individual coping mechanisms, help is available in many forms. Listed below are just some of the ways to find help...and hope.
Getting together with other cancer patients in a support group is a valuable coping tool. Support groups are usually focused on a single disease or topic, such as breast cancer survivors or people coping with life-changing side effects from their cancer or cancer therapy. These groups allow participants to meet others like themselves and seek strength from each other. Most major cities and cancer hospitals offer support groups that meet weekly or monthly. There are also dozens of online support web sites or message boards for those who may not have access to a traditional meeting.
Complementary therapies are used in conjunction with cancer treatment, in an effort to reduce treatment side effects, ease depression and anxiety and help cancer patients take their mind off the negative aspects of their situation. Complementary therapies may include mind-body exercises like yoga, tai chi and Qi gong; visualization or guided imagery; using art or music as therapy and self-expression and traditional Eastern medicine such as acupuncture.
Staying physically active as much as possible during cancer treatment has many positive benefits. Physical activity stimulates the release of endorphins, a hormone that helps elevate mood, as well as decreasing feelings of fatigue.
Exercises for cancer patients can range from simple stretches done in the bed or chair, to more active pursuits such as walking or light gardening work. However, it’s important not to push yourself too hard. Check with your doctor before attempting any physical activity to make sure you are up to it.
Many people find it helpful to keep a journal of their cancer treatment experience. It may be as simple as recording symptoms and side effects into a notebook, or may include personal emotions and opinions about what they may be going through. Journals can be private, like a diary, or shared with loved ones and even strangers.
Increasingly, people are turning to the Internet to share their cancer journey with the world at large and to seek out others with similar experiences. Many cancer patients have begun their own web log, or “blog” to publicize their battle with cancer. Twitter, a mini-blogging technology that limits posts to 140 characters, has also proven to be a helpful tool for cancer patients to keep friends updated and reach out to others.