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15/04/2019

Vall d'Hebron promotes the first complete record of patients with sarcoma in the State to improve their diagnosis and treatment

sarcomes_884

15/04/2019

The objective is to improve the anatomopathological diagnosis of a rare cancer (1% of the total) that includes up to 70 subtypes, which makes detection difficult.

Thehttps://www.vallhebron.com/en/specialities/pathological-anatomy Pathological Anatomy Service of the Hospital Universitari Vall d'Hebron is promoting the first complete registry of patients with sarcoma in Spain from the Spanish Group of Research in Sarcomes (GEIS) and the work group of tumors of soft parts of the Spanish Society of Pathological Anatomy (SEAP), with the support of several patient associations. It is the first phase of a project that aims to improve the anatomo-pathological diagnosis of sarcomas, an unusual cancer (represents 1% of the total) that affects soft tissue, visceral and bones and has more than 70 different subtypes. Detection is hampered by its low incidence and diversity: affects 1 in 20,000 people, with 376 cases in Catalonia and about 2,000 in Spain as calculated by GEIS. "In order to combat discord in the diagnosis of sarcomas, experienced pathologists are required and have the appropriate technical infrastructure, state-of-the-art equipment that allows us to study multiple molecular disorders and genetic profiles," explains Dr. Santiago Ramon y Cajal, Head of the Pathological Anatomy Service of Vall d'Hebron and head of the http://en.vhir.org/web_vhir/portal1/grup-equip.asp?s=recerca&contentid=186726&t=Patologia%20Molecular%20Translacional Translational Molecular Pathology group of the Vall d'Hebron Research Institute (VHIR)."We propose the creation of a network of expert pathologists at the Spanish level and that all the sarcomas of the State pass through it," summarizes Dr. Cleofé Romagosa, pathologist specializing in sarcomas of Vall d'Hebron and principal investigator of the research group on Translational Molecular Pathology of the Vall d'Hebron Research Institute (VHIR). The intention is to start the project first in Catalonia and to implement it gradually in the rest of the autonomous communities. As explained by Dr. Cleofé Romagosa, the first year will be dedicated to registering all cases of sarcomas diagnosed in the State, beginning with those of soft tissue and visceral sarcomas. The early diagnosis of these tumors is difficult, especially because they appear in a similar way to benign tumors, which are much more frequent. "These are very rare diseases, the head doctor who is confronted in the first place does not think about them, it is also ubiquitous tumors that can appear anywhere in the body although the most common is in arms and legs, "adds Dr. Claudia Valverde, Vall d'Hebron medical oncologist specialist in sarcomes and researcher at the Vall d'Hebron Institute of Oncology (VHIO). Among the signs of alarm to detect a sarcoma, protrusions of more than three centimeters, deep and growing, enumerates.The project will select hospitals throughout Spain that have a group of experienced pathologists and diagnostic tools that will become the reviewers and coordinators of the new pathologist network. Currently, there are seven Centers, Services and Reference Units (CSUR) for the multidisciplinary treatment of sarcomes in the State, three of them in Catalonia, including Vall d'Hebron. The intention is to ensure that all sarcoma patients have access to be diagnosed by pathologists with experience and technical resources similar to those of the CSUR in order to ensure a proper diagnosis. "This project does not consist of centralizing the study of pathology in a few hospitals, but in creating a network of synergies and collaboration between various hospitals and medical professionals: we offer ourselves as an impelling node," says Dr. Ramon and Cajal. The CSUR number is being pursued with an initiative whose main objective is to share knowledge and foster cooperation."There is a great deal of discord in the diagnosis of soft-tissue sarcomas, especially when comparing diagnostics made by specialized pathologists, experienced and with access to appropriate molecular and immunohistochemical techniques and less experienced pathologists who do not have these techniques." exposes Cleofé Romagosa. The largest study in this regard, conducted in France in a group of 814 patients, showed that only 56% of cases were in total agreement when reviewing the first diagnoses, although only 8% were major discords that involved benign diagnosis changes to malignant and vice versa. In this country, a centralized network already works: 90% of sarcoma receive a second opinion in a health center of reference.During the second year of the project, in addition to the registry of all cases of sarcoma, the aim is to establish a well-coordinated network between the review centers and coordinators, responsible for reviewing all cases diagnosed since its creation. The digital pathology will be applied to the diagnosis of sarcomas so that these centers can share complex cases, and a continuous training plan based on the discrepancies of the observed diagnostic will be designed. The three-year vision of the project is to demonstrate the effectiveness of centralizing the anatomo-pathological diagnosis of sarcoma in the whole State, both in terms of patient's quality of life and cost.Vall d'Hebron, reference center in sarcomaVall Hebron is a CSUR in the treatment of sarcomas: only in 2018 attended 182 patients over 14 years old with this pathology. It has the genitourinary tumor program group of the CNS and sarcoma, which works to provide patients with the newest and best treatments for their respective diseases, which include immunotherapeutic agents, targeted treatments and new antineoplastic medications.Vall d'Hebron is also a reference center in the treatment of pediatric patients with sarcoma and, in 2016, the Comik program (Medicina òmica en Nens) was launched, with personalized medicine in children's solid tumors. Patients who have benefited from Comik include children and adolescents with bone sarcoma (osteoarthritis, Ewing sarcoma and chondrosarcoma), rhabdomyosarcoma and sarcomas of non-rhabdomyosarcoma soft limbs (including patients with malignant rabdomic tumors, desmoplastic small cell tumors, alveolar sarcoma of soft parts and undifferentiated sarcoma), among other types of cancer.Our hospital has cutting-edge technology in massive DNA sequencing, such as the Next-Next Generation Sequency (NNGS), of Roche Diagnostics, which is very useful for detecting molecular disorders that support the diagnosis of a pathologist expert in sarcomas and that it also allows to detect new therapeutic targets customized to optimize the treatments

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