Personalizing Sarcoma Treatment

How Genetic Mapping and Mouse Avatars Are Revolutionizing Cancer Care

Whole Exome Sequencing Patient-Derived Xenografts Personalized Medicine

Introduction: The Sarcoma Challenge

Imagine facing a cancer so rare and diverse that standard chemotherapy offers little more than a coin flip's chance of response. For patients with sarcoma—a complex group of bone and soft tissue cancers—this scenario is all too common.

140+ Subtypes

Sarcoma represents a diverse group of cancers with over 140 different histological subtypes, making standardized treatment challenging.

Mouse Avatars

Patient-derived xenografts create "avatars" that carry the patient's exact cancer, allowing for personalized treatment testing.

With over 140 different histological subtypes, sarcomas represent the ultimate challenge in cancer treatment: how to find the right therapy for each unique tumor.

The Science Behind the Approach

Whole Exome Sequencing

Your exome represents less than 2% of your total genetic material, yet contains approximately 85% of all disease-causing mutations. Whole exome sequencing (WES) provides a cost-effective method to read this critical portion of DNA, identifying the unique genetic mutations driving an individual's cancer 7 .

Key Benefits:
  • Identifies targetable mutations
  • Cost-effective compared to whole genome sequencing
  • Reveals genetic variance between sarcoma subtypes 1 7

Patient-Derived Xenografts

Patient-derived xenografts (PDXs) take personalized cancer modeling a step further. In this process, fresh tumor tissue collected during a patient's biopsy is implanted into immunodeficient mice, creating "avatars" that carry the patient's exact cancer 1 7 .

Key Benefits:
  • Preserves original tumor heterogeneity 8
  • Maintains complex tumor microenvironment 2
  • More accurate than traditional cell lines

The Power of Combination

By combining WES and PDX technologies, researchers can both identify the genetic drivers of an individual's sarcoma and test potential treatments on an accurate biological model before administering them to the patient.

85%

Disease-causing mutations in exome

< 2%

Of genome represented by exome

58%

PDX success rate in sarcoma study 1 7

A Closer Look: The Pioneering Experiment

Methodology: From Patient to PDX

A landmark 2018 study published in Clinical Sarcoma Research provides a compelling blueprint for how these technologies combine to advance sarcoma treatment 1 7 .

Sample Collection

Tumor samples were obtained during surgical biopsies from patients with various sarcoma subtypes, including osteosarcoma, Ewing's sarcoma, and leiomyosarcoma.

Parallel Processing

Each tumor sample was divided between two arms: one portion underwent whole exome sequencing and the remainder was implanted into immunodeficient NSG mice to establish PDX models.

Bioinformatics Analysis

Sequencing data was processed through a specialized pipeline called IMPACT (Integrating Molecular Profiles with Actionable Therapeutics) to identify mutations that might confer sensitivity to existing chemotherapy drugs or targeted agents 7 .

Validation

Successfully established PDX models underwent their own exome sequencing and were used for direct chemosensitivity testing by exposing them to various therapeutic agents.

Patient Demographics and PDX Success Rates

Sarcoma Type Patients PDX Success Success Rate
Osteosarcoma 3 2 67%
Ewing's Sarcoma 2 1 50%
Leiomyosarcoma 2 1 50%
Other Types 5 3 60%
Total 12 7 58%

Sequencing Metrics

Sequencing Parameter Tumor Samples PDX Samples
Median Depth of Coverage 142x 142x
Median Number of Reads 122,945,876 122,945,876
Median Somatic Mutations 475 34,442
Key Finding

The bioinformatics analysis identified potential actionable therapeutics in all twelve patients based on their mutational profiles 7 .

When researchers compared the genetic profiles of original tumors to their corresponding PDX models, they found significant variations in predicted therapeutic responses in three of the seven matched pairs 1 . This crucial finding highlights both a limitation of the PDX approach and an important consideration for future research.

The Scientist's Toolkit: Essential Research Tools

The integration of WES and PDX models relies on a sophisticated array of laboratory tools and technologies.

NSG Mice

Provide immunodeficient environment for human tumor growth without rejection 1 7 .

Extracellular Matrix

Mimics human tissue environment for implanted tumors, supporting sarcoma growth 7 .

Agilent SureSelect

Isolates exome regions from total DNA, focusing sequencing on medically relevant genomic areas 7 .

Illumina HiSeq

Performs high-throughput DNA sequencing, generating comprehensive exome data 7 .

IMPACT Pipeline

Analyzes sequencing data for actionable mutations, identifying therapeutic targets 7 .

ATP-Based Assay

Measures cell viability after drug exposure, testing chemosensitivity of sarcoma cells 9 .

Beyond the Basics: Emerging Innovations

The field of personalized sarcoma treatment continues to evolve beyond the WES-PDX approach.

Patient-Derived Organoid Xenografts

Some research teams are now using patient-derived cancer organoids (PDCOs)—three-dimensional miniature tumors grown from patient samples—which are then implanted into mice as organoid-derived xenografts (ODX) 5 .

These models may better preserve the complex tissue architecture and stromal components of original tumors, potentially offering more accurate drug response prediction.

Functional Precision Oncology

Newer approaches like the quadratic phenotypic optimization platform (QPOP) go beyond genetic analysis to directly test drug combinations on patient tumor samples 4 .

This functional approach has identified promising novel drug combinations for sarcoma patients, including the pairing of AZD5153 with pazopanib, which showed superior efficacy compared to standard regimens.

AI in Drug Response Prediction

Advanced computational methods are now being applied to predict drug responses based on genomic features.

Deep learning models like DrugS analyze gene expression data alongside drug characteristics to forecast how individual tumors might respond to specific therapeutic agents . These approaches could eventually complement or reduce the reliance on resource-intensive PDX models.

The Future of Sarcoma Treatment

As technologies improve and computational methods become more sophisticated, we move closer to a future where every sarcoma patient receives treatment designed specifically for their cancer's unique genetic blueprint.

Conclusion: The Future of Personalized Sarcoma Treatment

The combination of whole exome sequencing and patient-derived xenografts represents a paradigm shift in how we approach sarcoma treatment.

By moving from population-based chemotherapy regimens to strategies tailored to an individual's specific tumor biology, this approach offers hope for patients who have exhausted standard options.

Current Challenges:
  • Time and resources required to establish PDX models
  • Genetic drift that can occur in PDX systems
  • Limited success rates for some sarcoma subtypes
Future Directions:
  • Improved computational prediction models
  • Integration of multi-omics data
  • Development of faster, more accurate testing methods

The journey from one-size-fits-all chemotherapy to truly personalized sarcoma treatment is well underway, powered by our growing ability to listen to what each individual cancer is telling us—and to test our responses before ever administering them to patients.

References