Desmoplastic
Small Round Cell
Tumor Charity
We are passionate about making sure that the stories shared on our page are as diverse as those diagnosed by Desmoplastic Small Round Cell Tumour and the paths that they follow. If you would like your story or that of a loved one to be shared here please do get in touch. We want to both celebrate those who have survived or are still battling this disease and remember those who are sadly no longer with us. In the words of Terry Pratchet “No one is finally dead until the ripples the cause in the world die away.” And we are determined to make ripples…
Liz was just 16 when she was diagnosed with Desmoplastic Small Round Cell Tumour. Having just started her A-Levels, she’d spent the autumn term looking around universities and planning her future. She dreamed of being a professional photographer and living in London. Until just before her diagnosis Liz had been the epitome of health, not missing a day of school since she’d had chicken pox aged seven.
Liz’s symptoms were subtle; a sore left shoulder, periods that had become gradually more painful and in the three weeks prior to her diagnosis feeling bloated. It was Christmas when Liz first mentioned not feeling quite right, she booked an appointment to see a physio in half term and put the bloating down to richer foods. Three weeks later, the bloating had become visible and the pain from her period didn’t end after the bleeding did. An emergency out of hours GP suggested an ovarian cyst was the likely cause, but when pain increased the day after she returned to A & E was given an MRI. The MRI found masses in her ovaries, omentum and liver. A diagnosis of cancer was given the following day and of DSRCT within four days. A week later she started VCD/IE chemotherapy at Leeds General Infirmary. She was their first case of Desmoplastic Small Round Cell Tumour.
In Liz’s case the NHS could not have moved more quickly. She had two cycles of VCD, but unfortunately due to the amount of ascites she had the IE component couldn’t be given. In round three her protocol was switched to VCDE. Round three also brought with it Para influenza, leaving Liz hospitalised and on oxygen support whilst she recovered. Post round three scans showed that her tumour mass had stayed broadly stable and her consultant asked her if she wanted to continue with chemotherapy given the limited effectiveness. Liz was firm that she did! Though terrified of chemotherapy to start with, by then she had lost her hair and had her port fitted. She was determined to continue.
Liz’s first consultant was medically sound, but Liz struggled to relate to her. After that appointment she changed consultants to Professor Bob Phillips; Liz loved him like family and always said speaking to him made her feel better. Bob never shied away from the truth – he gave Liz the facts she needed to live the life she had to the full and supported her in doing so. A virtual consult with Dr Emily Slotkin at Memorial Sloan in the US (who treats lots of DSRCT patients) confirmed that Liz’s condition was terminal but it also gave her the opportunity to ask questions about radiotherapy and surgery that were hard to get from the research.
Post round six scans showed a reduction in all of Liz’s tumours, her pain was vastly decreased and she was only taking 5mg of Oxycodone twice a day. For the first time her family allowed themselves to hope. Unfortunately however because of the impact on the heart, Liz had maxed out on Doxirubicin and despite the success of her current protocol a new one was needed. She had two cycles of VIA followed by two of CE. Neither were successful and her tumours continued to grow. Liz died on the 27/11/24 just ten months after her diagnosis aged only 17.
Ten months was too short but throughout that time Liz truly lived. She went on three separate Disney Cruises and a trip to Disneyland Paris where despite having only 12 weeks left to live she rode every ride across both parks. She took photographs at an investiture at Windsor Castle, on West End stages at both The Royal Ballet and Six The Musical, met Rob Brydon and photographed Michael Macintyre. She spent a day photographing Drag Race UK Drag Queens, and another at Charles Jeffrey’s London Fashion Week Catwalk Show. She photographed with Richard Heathcoate, Gareth Cattermole, David Suh and Rankin. Liz spent the last months of her life living not dying, on the last 10 weeks having stopped chemotherapy she was rarely at home. Instead she spent her time taking photographs and making memories with her family – especially her eight year old brother Mateo.
Liz’s memory lives on through her friends and family, but also through the incredible photographs she took and the lives she touched in doing so. But it also lives on in the research she contributed to in her lifetime, specifically around photobiomodulation treatment for mucusitis; as well as the research she made her family promise to fundraise for after her death.
Hunter was 21 and was working as full time dispatcher, deputy coroner and firefighter when he was diagnosed with Desmoplastic Small Round Cell Tumour. Prior to diagnosis, he spent the little free time he had, hanging out with friends and family, going out on adventures or throwing a get together. He lived an active and proactive lifestyle, with no concept of what would soon come his way.
His first symptoms were abdominal pain and vomiting. It was so severe that he asked his mom to take him to the Emergency Room; something he had previously tried to avoid. At the hospital they carried out imaging scans to determine what was going on. The scans came back showing a large mass taking up space in his abdomen and pelvis. It was a time of huge uncertainty and he and his family were unsure where to go. They couldn’t remove it at the local hospital, who recommended they sought treatment at a more specialized facility. They had received the most frightening of news but were left to figure out the next steps alone.
Hunter and his family had to travel over 2 hours from home to attend doctors’ appointments and seek tests to secure a diagnosis. It wasn’t until after his port was fitted that they found out what they were dealing with. Treatment started two weeks after the mass was first found, but because DSRCT is so rare it took longer for the biopsy to come back. Doctors had tested for multiple conditions before finding the right result.
Once Hunter had the diagnosis he realised quickly that the cancer needed to be hit hard and fast. It was at this point that Hunter’s medical team advised him to go to New York City and be treated by MSKCC. Instead of travelling two hours from home he was travelling half way across the country. The rarity of DSRCT meant he was determined to seek advice from experts in it, a choice he’s glad he made. Whilst challenging to be so far from home, stays at the Ronald McDonald house made it easier.
He started cycles of treatment with the VDC/IE protocol and stayed with that course of treatment until his first surgery. Scans around that time showed that chemo hadn’t achieved a favourable response on the tumours, therefore as the cancer was still operable Hunter’s medical team decided to operate. Once healed he switched between a few other treatment plans, ultimately ending up on irinotecan and temodar as his final treatment and maintenance protocol. Hunter was also part of a trial at MSK which included one last surgery, a radioimmunotherapy flush of his abdomen and 30 days of traditional WART (whole abdominal radiation therapy).
Life during treatment was hard, there were a lot of bad days. Hunter would frequently tell his parents that he was sick and tired of being sick and tired. It takes a strong type of person to go through what patients go through, and the hardships that come with it, but Hunter learned to make the most out of the little things he had control over at the time, like time with friends and family. It was not only the fog of “chemo brain” that made life so challenging but dealing with the complex emotions that raced through his head 24/7. Whilst it wasn’t always easy to do so, these were thoughts Hunter tried to block from his brain, instead focusing on planning the life he wanted to live if he managed to beat the disease.
Now 23 Hunter is in remission and explained that the biggest change he has made is that he now spends more time with family and friends, cherishing every moment he has with them. Back living almost the same life he was before diagnosis, it still doesn’t feel real. A weight has lifted off his shoulders and he is grateful for the steps he took emotionally during treatment to help prepare him mentally for what the future brings.