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In November 2023, my husband Josh Lombardi was diagnosed with a rare and aggressive sarcoma, DSRCT.

Since then, he has endured countless rounds of chemo, trips to Cleveland and Seattle for consults and infusions of novel drugs, a gruesome 13-hour surgery, several hospital admissions, whole abdominal radiation in Toronto for more than a month, targeted SBRT at BC Cancer, overall a profound change to the trajectory of his life, and yet he remains the love of my life and the wonderful guy I married.

There is no cure for DSRCT and outcomes remain poor. We need to do better for the young people like Josh and children who are diagnosed with this devastating disease.

As we commit to this 31-day challenge (while some of us will be pursuing even more ambitious fitness goals as we plan to do a very lengthy bike ride later in the summer), we'll be raising funds to support the Desmoplastic Small Round Cell Tumor (DSRCT) Research Fund at BC Cancer: https://bccancerfoundation.com/why-give/dsrct-research-fund/

Did you already donate to our cause last year or in 2024?

Check out this update from the Nielsen lab:

JANUARY 2026

Due to DSRCT's rarity, it has historically been challenging to build the infrastructure needed to create meaningful research momentum. Thanks to the generous support of donors, this is beginning to change. Progress includes:

1. A more comprehensive DSCRT database accelerating drug screening.

As a result of collaboration with Memorial Sloan Kettering Cancer Centre, Dr. Nielsen's team secured three additional DSRCT research models. These models, called cell lines, are created from DSRCT tumors and grown in a lab, creating a highly comprehensive platform for new research.

The team also ordered a variety of antibodies that identify the presence of certain proteins in DSRCT. These proteins can then be targeted by drugs that are already approved or drugs currently in clinical trials, which can be immediate alternatives either as stand-alone treatments or in combination with standard chemotherapy. The team developed new tissue microarrays, which combine 13 DSRCT samples from nine patients onto one slide. This is allowing researchers to screen drug targets on multiple samples at once, which is more efficient and gives more consistent results than analyzing individual samples. As a result of this work, two targets (PTK7 and B7H3) with available drugs in clinical trials have been identified to be relevant in DSRCT. Further functional studies will be underway to explore these targets in more depth.

2. Applying a cutting-edge lab tool to DSRCT.

Spatial transcriptomics is an advanced lab technique that generates a vast amount of data about gene expression in tumours, mapping interactions between the sarcoma cells and the other cell types that are present in and around the tumour. This approach can reveal new insights about the microenvironment that drive cancer growth or treatment resistance. By leveraging biocomputational tools, the team can then perform complex analyses to unlock the full potential of this new form of data about cancer. Dr. Nielsen's team has recently applied this technique to the new DSRCT microarray. Spatial transcriptomics holds potential to uncover new aspects of cancer biology and help researchers understand two of the biggest challenges in DSRCT treatment: how cancer cells avoid being attacked by the bodys immune system, and drug resistance.

This technique has also helped the research team to identify more than ten novel DSRCT- specific proteins that can potentially be targeted by different therapeutic strategies. These targets are currently being validated in the lab by immunohistochemistry, and top candidates will be studied with further functional studies.

3. Developing the first-ever genetically engineered mouse model for DSRCT, which would be a transformative step for preclinical testing of new treatments and understanding disease initiation and progression.

This innovative work is being done in collaboration with colleagues at UBC and in Europe. Dr. Nielsen says, If successful, this groundbreaking tool will be the closest pre-clinical system to the human disease, opening the door to in-depth biological studies and rigorous drug development.

Thank you!