Spotlight on rare cancers for Rare Disease Day
Rare Disease Day 2016 falls on the similarly rare 29th February. Here we explore some of the less common types of cancer and what we are doing to understand them better.
What is a rare cancer?
A rare cancer is one that affects very few people. But the actual definition is not as simple as you might think. You can read our previous blog on what makes a rare cancer to find out why this is the case.
Do we fund research into rare cancers?
Worldwide Cancer Research funds research into all cancer types, regardless of how many people it affects. We are currently funding a range of rare cancers. Some of which are highlighted below.
Hope for children with Ewing’s Sarcoma
Dr Maria Paola Paronetto, Fondazione Santa Lucia, Rome, Italy is investigating the rare bone cancer, Ewing’s Sarcoma. Cancer usually affects older people but Ewing’s Sarcoma (ES) is mainly found in teenagers and young adults. Ewing's Sarcoma can develop anywhere in the body, although it most often starts in the bone. If caught before it has spread, 70% of patients survive for at least 5 years after diagnosis. But that means 30% of patients die less than 5 years after diagnosis. The survival rate also decreases the more advanced the disease is when diagnosed.
Dr Paronetto told us “I work on the rare cancer Ewing’s sarcoma because it is a cruel tumour, which nestles into bone and soft tissue of children. We need a better solution than amputation and multidrug chemotherapy. If successful, I hope my research will help affected children have a greater chance of a normal life.”
Beating T-acute lymphoblastic leukaemia
Dr Anna Bigas (pictured above) at the Fundacio IMIM in Barcelona, Spain is studying T-acute lymphoblastic leukaemia (T-ALL) development. She wants to know what happens to the communication pathways in cells as T-ALL begins.
Dr Bigas explained “I work on the rare cancer T-ALL because it is a devastating disease, affecting both children and adults. Although current therapies have greatly improved the survival for children, a minority of patients are still not cured. In addition, adult patients still have a very poor rate of disease-free survival (the length of time a patient lives without the cancer coming back).”
She concluded “If successful, I hope my research into T-ALL will help us understand how this disease develops, and identify new targets for therapy.”
Better treatments for enteropathy-associated T-cell lymphoma (EATL)
EATL is a very rare type of T-cell lymphoma. It usually occurs in the small intestine, and is associated with celiac disease. Patients having a massive increase in faulty, premalignant, white blood cells in their small intestine are at high risk for developing EATL. It is fast growing and the future for these patients is not good, with an average five year survival of only 10-20%.
Together with his colleagues, Dr Jeroen van Bergen at the Leiden University Medical Center, The Netherlands has discovered a small group of cells in the intestines which he believes could be key to the initiation of EATL. Dr van Bergen is therefore using his grant to study how these begin to grow and divide rapidly, whilst avoiding death.
He told us “I hope my research into the rare cancer enteropathy-associated T-cell lymphoma will help to develop new therapies for this deadly disease in the future.”
Oncocytomas – how is the cell’s power house involved?
Dr Giuseppe Gasparre at the University of Bologna, Italy is investigating oncocytomas and in particular, how cellular power houses in our cells can become mutated. These mutations stop the cells from growing and force them into a sleep-like state.
Dr Gasparre told us “In some families, the accumulation of DNA mutations in the powerplants (mitochondria) in tumors is a recurrent event, happening in parents, children and grandchildren etc. These mutations lead to so-called ‘oncocytic tumors’, which are generally benign (non-cancerous) but can be precancerous or indeed cancerous.”
When asked about the aim of his work he told us “If we understand how these oncocytic tumour cells enter the slow growing sleep-like state it could be exploited with the use of drugs to make other, more dangerous cancer cells do the same.”
Although all of these cancer types may not affect large numbers of people, they have a big impact on those diagnosed and their families. Worldwide Cancer Research is dedicated to working towards the day when no life is cut short by cancer, regardless of how common the cancer type is.