Rare Disease Day: Spot on Sarcomas (Guest blog)

What are sarcomas? A sarcoma is a cancer which starts in soft tissues such as muscles, joints, fat, nerves or blood vessels (among others) or in the bones. Sarcomas are very rare: They account for only approx. 1% of all cancer diagnoses in adults. What makes them so special is the fact, that sarcomas can arise basically anywhere in the body and occur at any age. This also means that there approx. up to 100 different subtypes with a completely different course of disease, prognosis and treatment.

Challenges for sarcoma patients
Sarcoma patients face a number of challenges typical for patients with rare or ultra-rare diseases:
  • a long and bumpy way to a correct diagnosis
  • wrong or incomplete diagnosis
  • inappropriate treatment choices including adhoc biopsies, (sometimes quite mutilating) surgeries and ineffective medications
  • released from hospital as “cured” with no follow-up
Sarcomas need expert care
With sarcomas being so rare and difficult to diagnose and treat, it is essential that they are managed in expert centers. But what is the advantage of a specialized sarcoma center?
  • First of all: Multidisciplinary care. Experienced experts from different disciplines (oncology, surgery, pathology, radiology and more if necessary) work, discuss and decide together what treatments in what order are best for the individual patient.
  • Second is knowledge: Knowledge about available treatment options including special therapies such as Isolated Limb Perfusion or local ablative therapies. Knowledge about clinical trials and appropriate choices for the patients.
  • Third: Experience. Diagnosing, operating and treating a sarcoma needs – apart from expertise – a lot of experience. Pathologists for example face the challenge that in sarcomas, there are not always clearly defined diagnostic criteria or definitions and morphology is partly overlapping. Surgeons need to be aware of specifics in sarcoma tumours such as the fact that a sarcoma can consist of well-differentiated and undifferentiated parts. Well-differentiated parts might easily be overlooked by non-specialists, leading to incomplete surgeries and poorer outcomes.
Studies show: Patients treated by experts have better outcomes
To date, there is no comprehensive, international study to show differences in outcomes of patients treated in expert centers vs. other institutions. However, research in France, Spain and the USA proof that patients diagnosed and treated in specialized centers have a lower risk of mortality and better outcomes after surgery (e.g. lower risk of local recurrences).

Sarcoma Patients EuroNet (SPAEN) as the international network of sarcoma, GIST and desmoid patient groups therefore calls for all patients with sarcoma or even the suspicion of a sarcoma to be referred to a specialized center or a sarcoma expert as early as possible – in order to foster early diagnosis and improve outcomes and prognosis, possibly for many sarcoma patients.

Author: Kathrin Schuster, SPAEN Communications