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Hereditary retinoblastoma and soft tissue sarcomas

Patients diagnosed with retinoblastoma have a greater chance than the general public of developing soft-tissue sarcomas over their lifetime, says an article recently published in the Journal of the National Cancer Institute.

Retinoblastoma is a rare cancer that originates in the eye. Treatment for retinoblastoma often includes radiation therapy and may also include chemotherapy and surgical removal of the eye. There has been some concern that radiation therapy increases the risk of developing cancer in tissues within the radiation field.

The National Cancer Institute recently conducted a study to evaluate potential associates between patients diagnosed with retinoblastoma and the rate of soft-tissue sarcomas following treatment.

The study included 963 patients treated and diagnosed with hereditary retinoblastoma from 1914-1984. Data on these patients regarding the rates of soft-tissues sarcomas were compared to the general population.

The researchers concluded that patients diagnosed with hereditary retinoblastoma appear to have a significantly increased risk of developing soft-tissue sarcomas. This risk was increased in these patients whether or not they received radiation therapy and remained increased outside the field of radiation therapy; this indicates a potential genetic susceptibility for soft tissue sarcomas among patients with hereditary retinoblastoma.

Eye cancer and risk of metastasis

Researchers have pioneered the first technique to biopsy tissue from the living eye in order to predict what tumors possess a high chance of spreading to other parts of the body.

If the eye cancer metastasizes the patient will usually not survive the disease. Ocular melanoma attacks the pigment cells in the retina. Earlier studies discovered that patients who are missing one copy of chromosome 3 in their tumor tissue are more likely to have highly aggressive cancers.

This new procedure could offer huge medical and psychological benefits to the patients. Dr. Tara Young, assistant professor of ophthalmology at UCLA's Jules Stein Eye Institute and a Jonsson Comprehensive Cancer Center researcher, said "Identifying patients at high risk for metastasis is an important first step toward reducing the death rate of this cancer, which kills nearly half of its patients."

The technique of fine needle aspiration for collecting cancer cells from the living eye has been the standard of care at the Jules Stein Eye Institute since 2004, but adopted by only a handful of other ophthalmic centers in the nation.

Ocular melanoma is the most common eye cancer to strike adults. Some 2,000 cases are newly diagnosed every year in the United States and Canada.

Patients want to know about their prognosis. The patients that have a low chance of metastasis can breathe a sigh of relief and the high risk group can plan arrangements for their family and finances. The high risk group might also want to have more aggressive treatment and join a clinical trial to find better treatments to treat metastatic eye cancer.

Promising new treatment for eye cancer in children

St. Jude Children's Research Hospital scientists have demonstrated a new locally applied treatment for the eye cancer retinoblastoma. The new treatment, tested on mice, reduced the size of the tumor and also did not cause the side effects common with chemotherapy.

This targeted therapy uses a customized drug to disable a specific molecule inside a growing cancer cell. It is delivered locally to the site of the disease, rather than using systematic treatment. Dr. Dyer, Ph. D., associate member of the St. Jude Department of Developmental Neurobiology said "The findings suggest that this treatment not only could offer children with retinoblastoma more effective and less toxic treatment, it could also increase the chance that their vision can be preserved by eliminating the tumor and preventing its spread from the eye to the rest of the body".

Retinoblastoma occurs in about 5,000 young children worldwide each year, arising from the immature retina, which is the part of the eye responsible for detecting light and color. A report on this work appears in the November 2nd issue of the journal Nature.

Searching for Shnnoogles

Five years ago, when Michael Dunn's seven-year-old son Steven woke one morning with a swollen left eye, he took him to the emergency room thinking they would come home with eye drops for Steven. Instead, Steven became the 33rd known case of rhabdomyosarcoma, a cancer striking soft body tissue, that had no long-term survivors over the age of 20.

Enter artist Connie Douglas. While doing some work in the Dunn home, she was in awe of Steven's spirit as he battled cancer that would most likely steal his life before he reached adulthood. Inspired by Steven, she contacted Phoenix Children's Hospital, with a proposal to create and donate artwork for the children who visited or stayed at the hospital.

The art project turned into nine wall story panels, and within each painted panel are hidden Shnnoogles. A Shnnoogle, which stands for caring and sharing, hugging and snuggling, giving in goodness and action, are furry snowball creatures with big round eyes. As children look at the panels, they search for the hidden Shnnoogles.

While Douglas was painting the panels, she was diagnosed with MS. To be equally inspired by Arizona Republic Kathleen Quilligan's feature Artist hopes Shnnoogles bring comfort to hospitalized kids, to find out how Steven and Connie are doing, and the book she plans to write, go here.

Message in the Bottle: inspiration from a cancer patient

The Northwest Arkansas Morning News is featuring a story about Nicole Young, and her new non-profit Message In A Bottle project, that is providing inspiration to cancer patients and their families with hand-written messages delivered in a bottle.

Young, who is now 33, was first diagnosed with ocular melanoma, a rare eye cancer, three years ago while she was seven months pregnant. The diagnosis of cancer was made after she experienced blindness. Last fall, the doctors found tumors in her liver. She is currently undergoing chemotherapy.

Young says she knows all about the emotional and physical pain that cancer patients and their families go through, and she came up with the idea of writing messages of inspiration and delivering them to patients and their families facing cancer as a way to bring happiness and hope. To date, Young has been delivering her messages in a bottle to local cancer patients but she hopes to be able to do this on a worldwide basis.

To read more about Young, who at the age of 8, wrote, published and delivered the Lakeside News to neighbors featuring interviews, gossip and a comedy column, read Cancer Patient Bottles Inspiration. She needs help to make her messages in a bottle dream a worldwide reality.

Eye cancer most often a secondary cancer

Eye cancer in most adults is a secondary cancer spread from tumors of the breast, lung, kidneys and prostate glands via the bloodstream or the lymphatic system. Yearly eye exams are the best way to screen for eye cancer or Intraocular lymphoma.

Often melanomas of the eye are found during a routine eye exam. When the doctor looks through the pupil at the back of the eye, they may be able to see a dark spot that may indicate an early melanoma. Because they are rare, there is no screening test for them other than routine eye exams. People who find a dark spot on their iris (colored part of the eye) that is enlarging should have a doctor look at it. The first symptom of this disease is usually trouble with vision. Pain is rare. Intraocular lymphoma most often affects both eyes.

Examination of the eye by an ophthalmologist is the most important step in diagnosing melanoma of the eye. The doctor will look for enlarged blood vessels on the outside of the eye, which can indicate a tumor inside the eye. Using an ophthalmoscope, the ophthalmologist can get a very good look inside the eye and detect a tumor or other abnormality. Most of the time, this examination alone can make the diagnosis. To confirm the diagnosis, imaging tests such as ultrasound or angiography may be required. Angiography is a procedure done with a fluorescent dye that is injected into the bloodstream through a vein in the arm and will travel to the veins in the eye for examination. Very rarely will a biopsy also be needed.

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