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Rare Tumors: The Current Reality and the Challenges of the Future

Rare tumors are those that occur with a frequency of less than 6 cases per 100,000 population per year. Although they seem to affect a very small part of the population, they constitute 24% of all cancers that occur. In fact, approximately 1 in 4 patients with cancer suffers from a rare type, while in the European Union more than 650,000 new cases of rare cancers are recorded every year. It is noteworthy that patients with rare tumors are usually younger.

There are 12 categories of rare tumors, but each category includes many subcategories, with the total number of identified rare tumors reaching 198, according to data from the Rare Cancer Europe initiative.

Rare tumors include, among others:

  • Digestive system tumors (tumors of the rectum, small intestine, gallbladder, peritoneum and retroperitoneum),
  • Neuroendocrine tumors (stomach, small and large intestine, pancreas, lung)
  • Tumors (sarcomas) of soft tissues and bones
  • Tumors of the genitourinary system (tumors of the vagina, vulva, penis, testicle, ureter)
  • Thoracic and chest wall tumors (pleural mesothelioma)
  • Skin tumors (dermatofibrosarcoma proliferative-DFSP, Merkel cell carcinoma, extramammary Paget’s disease)

Rare tumors have traditionally been a difficult subject to study and treat due to their diversity and complexity.

The fact that these tumors are rare limited the amount of available data, resulting in a poor understanding of their etiology and the difficulty of creating clinical studies. The lack of experience and expertise inevitably affected diagnosis and treatment, leading to delayed or even incorrect diagnosis as well as non-holistic treatment.

All of the above have made it imperative to improve diagnostic accuracy, both at the pathological and imaging levels, as well as to optimize the therapeutic treatment of patients with rare tumors.

In recent years, technological developments with new imaging techniques as well as the ability to control the molecular profile of these tumors, through the next generation sequencing (NGS) method, allow for more accurate diagnosis, detecting specific genetic changes, and lead to fully personalized treatment of the disease and improved therapeutic options. At the same time, the evolution of science and technological progress provide the experienced Surgeon-Oncologist with the ability to perform innovative surgical procedures safely, achieving an excellent oncological outcome and ensuring the patient’s quality of life.

The Clinic of Surgical Oncology and the Center for Sarcoma, Melanoma and Rare Tumors of the Athens Medical Group, through a specialized, reputable and experienced interdisciplinary team of different specialties (surgical oncologist, medical oncologist, radiation therapist, pathologist, radiologist), offers holistic, personalized and innovative care and treatment of patients with rare tumors, and, with the use of state-of-the-art structures, advanced technology and the highly trained staff of the Athens Medical Group, aims to achieve the optimal result.

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