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CME: Challenges and updates in the management of von Hippel-Lindau disease and related tumors

ACCREDITATION EXPIRED: September 08, 2024

Activity Description / Statement of Need:

In this online, self-learning activity:

Von Hippel-Lindau (VHL) disease is a rare genetic condition caused by an autosomal dominant mutation of the VHL tumor suppressor gene. The mutation gives rise to an abnormal VHL protein that cannot bind effectively to protein HIF-1α, leading to the transcription of multiple genes and upregulation of growth factors. The condition is thought to affect between 1 in 39,000 and 1 in 91,000, with a birth incidence of between 1 in 36,000 and 1 in 45,500. Diagnosis of VHL disease is established when the patient undergoes genetic testing and a pathogenic mutation of the VHL gene is found. Genetic testing is typically conducted if the patient has a family history of VHL or they are showing signs of VHL-related symptoms. VHL disease is characterized by tumors and cysts growing in various parts of the body, including the brain, spine, eyes, inner ears, pancreas, kidneys, adrenal glands, and reproductive tract. The clinical presentation of the disease is different in every patient and is impossible to predict, so close monitoring is required. Complications of the disease are on a case-by-case basis, but patients with VHL are at an increased risk of developing some cancers, particularly clear cell renal cell carcinoma (RCC) and pancreatic cancer.

Target Audience:

HCPs including: Medical and genitourinary oncologists, urologists, and nephrologists; physician assistants, nurse practitioners, and pharmacists specializing in the aforementioned areas of specialty; and any other HCPs involved or interested in treatment of VHL disease. 


Commercial Support Disclosure: This program is supported by an educational grant from Merck.

This activity is free of charge.


Release Date: September 08, 2023 -- Expiration Date: September 08, 2024

Faculty: Eric Jonasch, MD

Agenda

Faculty introduction and disclosures 

Introductory content and review of VHL disease and related tumors 

  • Epidemiology 
  • Age of onset 
  • Pathophysiology, VHL types 
  • Health burden 

Management of VHL disease and related tumors 

  • Clinical features and medical management
  • RCC 
  • pNETs 
  • Hemangioblastomas 
  • Pheochromocytomas 
  • Indications for surgical intervention, transplant, and radiotherapy, where applicable 
  • Surveillance protocols by age 
  • Patient case(s)

Summary, conclusions, and best practice recap 

Learning Objectives

By the end of the session the participant will be able to:

  • Summarize challenges to the healthcare burden, surveillance, and management of VHL and related tumors.
  • Identify the currently available and emerging pharmacotherapeutic treatment options for the management of VHL-associated tumors and complications.
  • Formulate a treatment plan for a patient with VHL disease-related tumors.

Accreditation

ACCME Activity #202548110

ACCREDITATION FOR THIS COURSE HAS EXPIRED. YOU MAY VIEW THE PROGRAM, BUT CME / CE IS NO LONGER AVAILABLE AND NO CERTIFICATE WILL BE ISSUED.

Faculty Disclosure and Resolution of COI

As a provider of continuing medical education, it is the policy of ScientiaCME to ensure balance, independence, objectivity, and scientific rigor in all of its educational activities. In accordance with this policy, faculty and educational planners must disclose any significant relationships with commercial interests whose products or devices may be mentioned in faculty presentations, and any relationships with the commercial supporter of the activity. The intent of this disclosure is to provide the intended audience with information on which they can make their own judgments. Additionally, in the event a conflict of interest (COI) does exist, it is the policy of ScientiaCME to ensure that the COI is resolved in order to ensure the integrity of the CME activity. For this CME activity, any COI has been resolved thru content review by ScientiaCME.

Disclosures of Faculty: Eric Jonasch, MD, Professor, Department of Genitourinary Medical Oncology, University of Texas MD Anderson Cancer Center, has received financial compensation from Aveo, Arrowhead, Corvus, DAVA, Eisai, Exelixis, GSK, Ipsen, Merck, NiKang, Novartis, Takeda and Telix for research and/or consulting work. 

Disclosures of Educational Planners: Charles Turck, PharmD, BCPS, BCCCP, President of ScientiaCME, has no relevant financial disclosures.

Faculty WILL discuss off-label uses of a commercial product.

All relevant financial relationships have been mitigated.

ScientiaCME adheres to the ACCME’s Standards for Integrity and Independence in Accredited Continuing Education. Any individuals in a position to control the content of a CE activity, including faculty, planners, reviewers or others are required to disclose all relevant financial relationships with ineligible entities (commercial interests). All relevant conflicts of interest have been mitigated prior to the commencement of the activity.

Commercial Support Disclosure: This program is supported by an educational grant from Merck.

Instructions

  • Read the learning objectives above
  • Take the Pre-Test (optional). Completion of the pre-test will help us evaluate the knowledge gained by participating in this CME activity.
  • View the online activity. You may view this is in more than one session, and may pause or repeat any portion of the presentation if you need to.
  • Minimum participation threshold: Take the post-test. A score of 70% or higher is required to pass and proceed to the activity evaluation.
  • Complete the activity evaluation and CME registration. A CE certificate will be emailed to you immediately.

Cultural/Linguistic Competence & Health Disparities

System Requirements

PC
Windows 7 or above
Internet Explorer 8
*Adobe Acrobat Reader
MAC
Mac OS 10.2.8
Safari or Chrome or Firefox
*Adobe Acrobat Reader
Internet Explorer is not supported on the Macintosh

*Required to view Printable PDF Version


Perform Pre-Test (optional)

Please take a few minutes to participate in the optional pre-test. It will help us measure the knowledge gained by participating in this activity.


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