Sarcoma Awareness Month: Shedding Light on a Rare Cancer
Sarcoma Awareness Month is held annually in July, a time when the global sarcoma community comes together with a unified mission: to raise awareness about this rare and often overlooked group of cancers and to highlight the unique challenges faced by patients and their families.
This month is not just a period of recognition but a call to action for everyone.
Participation can take many forms. From sharing information on social media, and participating in fundraising activities to engaging with policymakers to advocate for better healthcare policies and funding for sarcoma research. Every action, no matter how small, contributes to a larger movement that strives to bring hope and change to those affected by sarcoma.
Sarcoma Awareness Month is a powerful reminder of the strength and resilience of the sarcoma community. It is a time to stand in solidarity with patients and survivors, to honor those who have lost their lives to the disease, and to reinforce our collective commitment to fighting for a future where sarcoma can be detected early, treated effectively, and, ultimately, cured.
Working Together, Making a Difference.
Understanding Sarcoma
Sarcomas are a diverse group of cancers that originate in the bones and soft tissues, such as muscles, fat, blood vessels, nerves, and fibrous tissues. They are relatively rare, accounting for about 1% of all adult cancers but comprise approx. 15% of pediatric cancers. There are two main types: bone sarcomas and soft tissue sarcomas, with further subtypes under each category, totaling approx. 100 distinct forms.
Due to their rarity and the variety of subtypes, diagnosing and treating sarcomas can be particularly challenging. Symptoms are often unclear, leading to delays in diagnosis. However, early detection is crucial, as the prognosis can be improved with prompt and appropriate treatment.
What we know about sarcoma diagnosis: Data & publications📝
Delays in the Management of Retroperitoneal Sarcomas
Retroperitoneal sarcomas, while rare, benefit from centralized treatment. Despite 90% of patients being referred before surgery, delays persist. Analysis of 33 patients showed a median patient delay of 23 days and a median healthcare delay of 94 days, with specific delays of 15 days at the general practitioner, 36 days at local hospitals, and 55 days at the sarcoma center. The study concludes that centralization alone is insufficient for optimal management and suggests that direct referral from primary care to sarcoma centers and coordinated diagnostic packages could reduce these delays.
Seinen J et al.
Patient and diagnostic intervals of survivors of sarcoma: Results from the SURVSARC study
The study focused on sarcoma patients’ delays in diagnosis, separating into patient interval (symptom onset to consultation) and diagnostic interval (consultation to diagnosis). Of 1099 participants, 60% had patient intervals ≥1 month; 36% ≥3 months, with longer intervals associated with skin or pelvic sarcoma, liposarcoma, or rhabdomyosarcoma. Stage III disease correlated with shorter patient intervals. Diagnostic intervals were ≥1 month in 55%, ≥3 months in 28%, longer intervals linked to female sex, age <70 years, synovial sarcoma, or chordoma. Both intervals were prolonged in many survivors, urging better patient and physician awareness to shorten diagnostic delays.
Soomers VLMN, Husson O, Desar IME et al.
Patient and diagnostic intervals of survivors of sarcoma: Results from the SURVSARC study.
Cancer. 2020: 126: 5283-5292. https://doi.org/10.1002/cncr.33181
Delay in referral to a specialist soft-tissue sarcoma unit
Soft-tissue sarcoma often presents with vague symptoms that mimic more common conditions, leading to delayed diagnosis and referral to specialized care. This study found that one-fifth of patients faced significant delays in accessing specialist treatment, potentially affecting their treatment options and survival outcomes.
Clark M.A., Thomas J.M.
Delay in referral to a specialist soft-tissue sarcoma unit
European Journal of Surgical Oncology (EJSO), Volume 31, Issue 4, May 2005, Pages 443-448; https://doi.org/10.1016/j.ejso.2004.11.016
The Chondrosarcoma Foundation Registry:42,3% misdiagnosis in chondrosarcomas
The results from the registry of the Chondrosarcoma Foundation in the US revealed a rate of misdiagnosis in chondrosarcomas of 42.3%. The most frequent symptoms occurred in chondrosarcoma patients were pain (64.5%), lump/swelling (29.6%), and restricted mobility (23.1%).
The prediagnostic general practitioner care of sarcoma patients: A real-world data study
Understanding these patterns can help GPs identify sarcoma earlier, potentially reducing delays in diagnosis and improving patient outcomes.
Holthuis EI, van der Graaf WTA, Drabbe C, et al.
The Complexity of Diagnosis of Sarcoma in a Timely Manner: Perspectives of Health Professionals, Patients, and Carers in Australia
Weaver, R. et al.
Sarcoma: concordance between initial diagnosis and centralized expert review in a population-based study within three European regions
Precise identification of histological diagnosis and prognostic factors is crucial for establishing effective treatment plans. Second opinions (SO) have been proposed to enhance diagnostic accuracy in cases of sarcoma. This study looked at histological data of patients diagnosed with sarcoma in France and Italy and found that more than 40% of first histological diagnoses were modified at second reading, possibly resulting in different treatment decisions.
Ray-Coquard I, et al.
Sarcoma: concordance between initial diagnosis and centralized expert review in a population-based study within three European regions.
Ann Oncol. 2012 Sep;23(9):2442-2449. doi:10.1093/annonc/mdr610.
Italy: Whitebook for Sarcomas reaveals 49,9% of sarcoma misdiagnosis
In Italy, the data collected through the national survey “Living with sarcoma” launched by Fondazione Paola Gonzato in 2023, highlighted that 49,4% of patients received incorrect/misleading diagnoses. With regard to bone sarcomas, this percentage rises up to 56%.
UK: Sarcoma UK's 2020 survey shows that 1 in 3 patients wait for over 6 months to be diagnosed
Data from Sarcoma UK’s National Sarcoma Survey 2020 finds that there is a significant delay in reaching a sarcoma diagnosis after a patient presents to a healthcare professional with symptoms, with nearly 1 in 3 sarcoma patients waiting over 6 months to be diagnosed. This length of time decreases the chances of treating sarcoma at an early stage.
UK: 26% of the Bone Sarcoma Patients in the UK wait longer than 7 months for their diagnosis, reveals the BCRT Patient Survey 2020
This study explores the early medical journey of sarcoma patients during the year before a sarcoma diagnosis. The average number of monthly consultations peaked in the month before diagnosis for both bone and soft-tissue sarcomas. For bone sarcomas, most early diagnoses matched the clinical presentation of the disease. However, for soft-tissue sarcomas, there was a wider range of early diagnoses, reflecting the various subtypes that can appear in different parts of the body.
Understanding these patterns can help GPs identify sarcoma earlier, potentially reducing delays in diagnosis and improving patient outcomes.
Holthuis EI, van der Graaf WTA, Drabbe C, et al.
Improving Diagnosis and Care for Patients With Sarcoma: Do Real-World General Practitioners Data and Prospective Data Collections Have a Place Next to Clinical Trials?
Real-world data (RWD) is essential for tackling complex research questions in rare cancers like sarcomas, where conducting large randomized trials is impractical due to limited patient numbers and logistical challenges. RWD provides insights across the patient journey, from early symptoms in primary care to long-term treatment outcomes. Integrating RWD with observational trials helps evaluate treatment effectiveness, particularly for ultrarare sarcomas. Despite challenges, RWD holds promise in improving sarcoma care by bridging gaps between research and practice, offering insights traditional trials may miss.
Emily I. Holthuis et al.,
Improving Diagnosis and Care for Patients With Sarcoma: Do Real-World General Practitioners Data and Prospective Data Collections Have a Place Next to Clinical Trials?
JCO Clinical Cancer Informatics 2024:8, DOI:10.1200/CCI.24.00054