A father’s perspective on the challenges of treating metastatic chondrosarcoma

At a glance 

• One family’s experience highlights the challenges of diagnosing and treating metastatic chondrosarcoma, a rare cancer with limited treatment options. 
• The author advocates for greater research investment, improved clinical guidance, and more streamlined pathways to investigative therapies. 

Estimated read time: 6 minutes 

Editor’s note: Chondrosarcoma is a rare and deadly cancer that responds poorly to traditional chemotherapy and radiotherapy. Due to the lack of effective treatment, the clinical management of metastatic chondrosarcoma is exceptionally challenging. This article reflects the perspective of a chondrosarcoma patient’s father, who accompanied his daughter throughout her treatment journey and reviewed the available literature in an effort to advocate for her care. It is published in support of July being Sarcoma Awareness Month, as well as the AAOS Musculoskeletal Tumor Registry. 

My daughter Shayna Elise Kramer was a rare diamond, a shining star. She never wanted the spotlight — she was quiet, reserved, and put other people’s needs and feelings before herself.  

Her passion was working with children with special needs. In the summer of 2016, Shayna went to Israel and volunteered at Save a Child’s Heart, working with children from underprivileged countries recovering from surgery due to heart defects. She brought joy to these children, unaware that she was dealing with her own health crisis. 

When Shayna came home, she was diagnosed with chondrosarcoma in her spine and endured two major surgeries. She told us that if the children could travel to a strange country alone and have major heart surgery, then she could deal with her surgery. Not once did Shayna ever complain about being sick or dwell on her suffering.  

Despite having cancer, Shayna worked tirelessly at Chapel Forge Early Childhood Center and went to school in pursuit of a master’s degree in special education. She would have been a wonderful special education teacher. She would have been a wonderful wife and mother. The world indeed lost an angel. 

She was my beloved daughter, and I am in awe of what she endured. I miss her greatly, as do her family and countless friends.  

Shayna’s battle began when she started to present with lower back pain with onset in November 2015. An initial orthopaedic examination and evaluation, including an MRI, diagnosed her with scoliosis at 25 years old. 

Increasing back pain and new neurologic defects were noted as interfering with her gait, prompting a CT scan revealing a spinal mass. After hospital admission in August 2016 came a diagnosis with high-grade (III) conventional chondrosarcoma involving the lumbar spine (L2/L3). The tumor was too large for resection en bloc, thus requiring two major surgeries. The first surgery inserted rods to replace the L2/L3 spinal columns; the second surgery removed the rest of the tumor from the spine and built a cage. Post-surgery, Shayna received 30 treatments of proton radiotherapy. During her recovery from proton radiation and after six months of rehabilitation services, Shayna was able to drive her car and work as a teacher aide with special education preschoolers. 

Per regional spinal MRIs obtained every three months, she remained in remission for the first 18 months postoperatively. Shayna also had an annual CT abdomen to screen for growth of a benign mass on her pancreas. The annual check on her pancreas incidentally suggested tumors on the lower visualized lung fields, later confirmed as 15 bilateral metastatic tumors (average 2 cm in diameter.). In addition, a metastatic tumor was discovered on her left great toe, which several doctors and oncology staff initially treated as an infection. Subsequent biopsies of her lungs and great toe confirmed metastatic chondrosarcoma.  

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After the conclusion of radiation therapy, Shayna developed a high fever requiring hospitalization in October 2019. A positron emission tomography (PET) scan demonstrated diffuse progression and new lung/soft tissue and liver metastases, prompting plans to alter the regimen. In late October 2019, visual field deficits and gastrointestinal pain developed. Seven brain masses were identified, for which whole brain radiation was initiated along with corticosteroid therapy. 

Rapidly following initiation, severe abdominal pain developed, later diagnosed as colonic perforation requiring emergent open laparotomy, where diffuse intraperitoneal metastatic disease and diffuse fecal contamination were identified. A hemicolectomy was performed and was treated with broad-spectrum antibiotics. Surgeons and internal and external oncologists reached consensus to withhold treatment other than whole brain radiotherapy until her abdominal wound healed. 

Shayna’s disease ultimately spread to the lungs and several other sites, illustrating both the aggressive nature of her cancer and the challenges associated with treating metastatic chondrosarcoma.  

Unfortunately, Shayna passed away two days later at home with family and friends. 

At the time of her treatment, no standardized treatment protocol existed, and therapeutic options were limited. Her experience raised important questions about diagnosis, surveillance, treatment selection, and access to clinical trials. 

Challenges encountered during diagnosis and treatment
When it comes to chondrosarcoma, Shayna’s disease progression defied many expert opinions, much clinical knowledge, and widespread expectations. 

Shayna initially received a diagnosis of scoliosis before the underlying tumor was identified approximately nine months later.  

Shayna’s recurrence also demonstrated the difficulty of predicting how a rare cancer will behave. Although local recurrence was expected, she instead developed metastases in the lungs and left great toe. The toe mass initially was thought to represent infection, reflecting the rarity of distal appendicular metastases. She later developed headaches and visual symptoms, and brain metastases were identified in October 2019. 

After brain metastases were identified, abdominal symptoms were initially attributed to neurologic disease progression. Subsequent evaluation revealed a colonic perforation requiring emergency surgery. This experience highlighted the diagnostic complexity of patients with advanced metastatic disease and multiple concurrent symptoms. Subsequent major abdominal surgeries (hemicolectomy and ileostomy) and bed confinement resulted. 

Accessing clinical trials proved challenging because of eligibility requirements, biomarker testing needs, travel demands, and the rapid progression of Shayna’s disease. Determining eligibility often required specialized testing that was not readily available through standard clinical workflows.  

Looking to the future
In response to Shayna’s experience, I established Chondrosarcoma Awareness Day on Feb. 6, Shayna’s birthday, and helped launch the Chondrosarcoma Foundation. The foundation supports patient education, advocacy, research collaboration, and development of resources for patients and families. Early efforts have included organizing researchers and clinicians to develop guidance and improve collaboration in the study and treatment of chondrosarcoma. 

Shayna fought hard to beat chondrosarcoma and remained determined to help others facing the disease. Her experience illustrated the difficulty of treating a rare cancer with limited treatment options and little consensus guidance. Her story — along with the Chondrosarcoma Foundation — will contribute to ongoing efforts to improve research, surveillance, biomarker testing, and access to clinical trials for future patients. 

Jeffrey Kramer founded the Chondrosarcoma Foundation following the death of his daughter, Shayna, from metastatic chondrosarcoma. The foundation promotes patient advocacy, education, research collaboration, and efforts to improve care for patients with the disease. 

References 

1. Nakamura T, Matsumine A, Yamada S, et al. Oncological outcome after lung metastasis in patients presenting with localized chondrosarcoma at extremities: Tokai Musculoskeletal Oncology Consortium study. Onco Targets Ther. 2016;9:4747-4751. doi:10.2147/OTT.S107638. 
2. Que Y, Xiao W, Xu BS, et al. The changing landscape of phase II/III metastatic sarcoma clinical trials—analysis of ClinicalTrials.gov. BMC Cancer. 2018;18:1251. doi:10.1186/s12885-018-5163-2. 
3. Tsavaris O, Economopoulou P, Kotsantis I, et al. Clinical benefit of pazopanib in a patient with metastatic chondrosarcoma: a case report and review of the literature. Front Oncol. 2018;8:45. doi:10.3389/fonc.2018.00045. 
4. Bernstein-Molho R, Kollender Y, Issakov J, et al. Clinical activity of mTOR inhibition in combination with cyclophosphamide in the treatment of recurrent unresectable chondrosarcomas. Cancer Chemother Pharmacol. 2012;70(6):855-860. doi:10.1007/s00280-012-1968-x. 

Advancing knowledge in sarcoma care with the AAOS Musculoskeletal Tumor Registry  

The AAOS Musculoskeletal Tumor Registry (MsTR) captures data on procedures treating bone and soft tissue sarcomas across the body, advancing understanding of this rare and complex disease. Each July, MsTR amplifies patient and surgeon voices to raise awareness of sarcoma. 

View videos from sarcoma patients and MsTR surgeon leaders.  

Download the MsTR’s inaugural Annual Report for insights into patient characteristics, tumor pathology, and treatment patterns.