by Vera Martinella
There are a hundred different subtypes of sarcomas, rare cancers that affect children, adolescents and adults. You need a precise diagnosis and a center with experience to obtain the most suitable therapies
There are about 3,500 Italians who every year have to deal with a sarcoma, a rare tumor that causes many difficulties for patients. Starting from the diagnosis that often occurs late and is complicated also because there are about 100 different histological types (sarcomas affecting the soft tissues are the most frequent, while those of the bone and gastrointestinal stromal ones are much rarer), up to the therapies , for which it is essential to rely on a specialized center. In July, the month dedicated to these neoplasms at an international level, the awareness and information campaign «Patients, experts and institutions together in the challenge to sarcomas» was organized to help patients and their families, promoted and organized by Paola Gonzato Foundation-ETS Sarcoma Network, which aims to improve the path of patients and to provide useful tools to orient themselves in the course of the disease, without forgetting the psychological aspect and the quality of life of those directly involved.
Sarcomas, what you need to know
What makes these neoplasms particularly complex is not only their rarity (they affect less than 5 people out of 100,000 a year, some ultra-rare less than one person out of a million), but also the fact that they can affect all age groups ( children, adolescents, adults), from pediatric to late adult, and which can affect any area of the body. «When we talk about sarcomas we are talking about different pathologies – explains Franca Fagioli, director of the Pathology and Child Care Department at the Regina Margherita Children’s Hospital in Turin -. They are connective tissue tumors that are found everywhere in the body because they are the body’s “support” tissues: muscles, bone, nerves, cartilage, tendons, deep layers of the skin, blood vessels, adipose tissue. The various neoplasms are then divided into numerous subgroups also on the basis of biomolecular characteristics. Depending on the histotype and extent of the disease, the prognosis can change and having an accurate, precise diagnosis is an indispensable step in choosing the best possible therapy”. Further difficulties derive from the fact that sarcomas do not present specific symptoms in the initial phase, so they are often diagnosed late, not recognized, confused with traumatic pathologies or much more frequent benign tumors.
Diagnosis
Before starting any therapeutic treatment, a diagnosis is required which must always be a pathological diagnosis, performed through a biopsy. «The histopathological diagnosis of rare tumors is very problematic and, as for treatments, experience is decisive – continues Paolo Casali -. The problem is that by definition there are few reference centers for rare tumors, so patients have to manage a further logistical problem: the distance to thespecialized hospital, not always so easy to locate. The networks should be the solution, but the National Rare Cancer Network, the result of a 2017 State-Regions agreement, has not yet been established, but at least we have the list of centers that should participate and this is a very important step”. Five years after diagnosis, on average, 55% of patients are alive. “The odds improve significantly if patients are treated appropriately from the outset in specialist centers within dedicated networks – underlines Ornella Gonzato, founder and president of the Paola Gonzato Network Sarcoma ETS Foundation -. Available data show that more than 30% of sarcoma diagnoses are inaccurate: due to the biological rarity and complexity of sarcomas, as well as the complexity of the technologies and specialized skills required, the diagnosis must be made by pathologists experienced in sarcomas. It is vitally important that patients and their families are aware of it”.
Therapy
The therapy must, in fact, be prescribed on the basis of the specific histotype. The evaluation of each patient by a multidisciplinary team of specialists is essential for the treatment decision: «It is mostly about multidisciplinary treatments (chemotherapy, surgery with or without radiotherapy or immunotherapy) – adds Fagioli -. There are not many differences between children and adults, but it is important to take into account many variables related to the type of disease and be followed by specialist centers experienced in the treatment of sarcomas». «Patients with sarcoma suffer not only from the disease but also from a still sub-optimal management of care, which has a direct impact on their lives, both in terms of survival probability and quality, as well as affecting health care costs and social – concludes Gonzato -. And yet, the model of the National Rare Tumor Network, a “hinge” between the European Reference Networks (ERN) and the Regional Oncological Networks (ROR), as a virtual network of teleconsultations, capable of moving specialist knowledge and skills rather than patients, is not still fully operational. In this context, patient organizations play a crucial role because they constitute a “bridge” between the needs of patients and clinicians, researchers and institutions, fundamental interlocutors in the process of continuous, but tiring, evolution for the improvement of assistance and research. Patient organizations can not only bridge but accelerate the process, trying to stimulate and co-design, together with experts and institutions, a better system».
July 28, 2023 (change July 28, 2023 | 11:07 am)
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