EJP RD ERN Workshop: Desmoid tumors (DTs) in patients with Familial Adenomatous Polyposis (FAP): an interdisciplinary approach

  • 22 May 2023 - 10:30
  • Milan, Italy
ERN Workshops

Concept and aim
DT is defined as a rare benign monoclonal fibroblastic proliferation that arises in soft tissue. They sporadically occur, but 5–20 per cent arise in association with FAP. The incidence of DT is 800-fold to1000-fold higher in patients with FAP than in the general population. Thus, 10–25 per cent of patients with FAP will develop at least one Desmoid Tumour within their lifetime. Desmoid when occur in abdominal cavity is the primary cause of death after colectomy by infiltrating and obstructing nearby structures.

Contrary to sporadic desmoid tumors whose management has better been defined over the last 10 years the complexity of  DTs treatment in FAP patients still requires to be optimized.

Several open question about different topics in FAP-related DTs remain to be clarified:

  • Risk prediction and prevention (genotype-phenotype correlation, open vs. laparoscopic preventive surgery)
  • Diagnosis (radiological vs. histological)
  • Treatment strategy (watchful waiting strategy vs. active treatment)
  • Quality of Life

This workshop aims to promote further studies and researches on this topic.

To date, medical treatment (e.g. chemotherapy or immunological therapy) is preferred to surgery, because the surgical trauma is a well-known risk factor of developing further DTs. The medical treatment is based on DTs’ histology; however, a percutaneous biopsy of intra-abdominal DT is not always feasible and a surgical procedure is necessary.

The creation of a software combining radiological imaging and genetic data, which predicts the nature of intra-abdominal masses in FAP patients would be pivotal in their management and extremely useful for ERN physicians.

The Workshop aims to promote networking in the context of two ERNs, EURACAN and GENTURIS, thanks to their mutual focus on DT in FAP, a solid tumor which is both rare and associated to a genetic condition.

This workshop is addressed to physicians, researchers from ERN Full Members or Affiliated Partners from the junior to senior level. Particularly, this workshop is directed to participants who want to ameliorate their knowledge in FAP patient management and DTs treatment. This networking activity will foster the exchange of experiences and allow participants to appreciate the effectiveness of different approaches, as a basis for future collaborations and joint research projects involving ERN full and affiliated members.

The training methodology will be based on lectures, seminars, and practical sessions, aimed at providing concrete research skills. Specifically, real-life cases will be discussed in interactive seminars, which will introduce specific concepts to deal in the practical sessions, where attendees will have the opportunity to verify and apply their knowledge.
The research questions aforementioned will be shared and discussed among all the participants.
There will be interactive plenary presentations and question & answer sessions between speakers and participants. 

To register please fill out the application form here until 30 April 2023. 

The course and registration are free of charge according to ERN recommendation.
A maximum of 25 attendees will be admitted in order to ensure active participation.
Travel and accommodation expenses will be reimbursed for a maximum of 20 participants according to the ERN funding model – the travel and accommodation costs of the remaining 5 additional participants will be covered by the workshop’s host institution.

Affiliated to an ERN-Full Member or Affiliated Partner institution.

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At the end of the training workshop a Certificate of Attendance will be sent to the participants who attended the entire workshop.
No credits of Continuing Education in Medicine will be issued. 

Workshops will run in English.

Fondazione IRCCS Istituto Nazionale dei Tumori di Milano. Via Venezian 1, Milan, Italy.

For specific questions related to the workshop please contact: Marco Vitellaro, MD| email: marco.vitellaro@istitutotumori.mi.it

For questions related to travelling, accommodation, dinner please contact: desmoidworkshop@inventawide.com

For general inquiries about the workshops in the ERN RD Support and Training Program please write to:
Sanja.hermanns@ejprd-project.eu
RareEndoERN@lumc.nl

EVENT INFO :

  • Start Date:22 May 2023
  • Start Time:10:30
  • End Date:23 May 2023
  • End Time:17:00
  • Location:Milan, Italy