Advancing the multidisciplinary management of rare and unusual NETs: Integrating new approaches to treatment and care

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touchMDT Advancing the multidisciplinary management of rare and unusual NETs: Integrating new approaches to treatment and care

Watch leading specialists involved in the multidisciplinary care of patients with rare and unusual neuroendocrine tumours discuss new and emerging approaches for diagnosis and treatment.

 Overview & Learning Objectives

Patient with neuroendocrine tumour
Medical Oncologist, Nuclear Medicine Physician and Endocrinologist
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Achieving a timely and accurate diagnosis: How can multidisciplinary input address current challenges? Dr Diane Reidy-Lagunes, Dr Thomas Hope, Prof. Dr med. Marianne Pavel

Watch leading specialists from the MDT discuss the diagnostic workup and challenges associated with diagnosing rare and unusual NETs.

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Medical Oncologist, Nuclear Medicine Physician and Endocrinologist
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Mapping an individualized treatment plan: What role does the MDT play? Dr Diane Reidy-Lagunes, Dr Thomas Hope, Prof. Dr med. Marianne Pavel

Watch leading specialists from the MDT discuss the importance of a coordinated approach to clinical decision making in a patient with a lung NET.

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Medical Oncologist, Nuclear Medicine Physician, Nurse Specialist
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Supporting treatment adherence: What strategies are needed for safety management? Dr Diane Reidy-Lagunes, Dr Thomas Hope, Ms Catherine Bouvier Ellis

Watch leading specialists from the MDT discuss the side effects associated with treatment in a patient with liver-dominant disease and the importance of addressing patient needs in ensuring adherence to treatment.

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Medical Oncologist, Nuclear Medicine Physician, Endocrinologist, Nurse Specialist
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Managing disease progression: Considerations for treatment selection and sequencing Dr Diane Reidy-Lagunes, Dr Thomas Hope, Prof. Dr med. Marianne Pavel, Ms Catherine Bouvier Ellis

Watch leading specialists from the MDT discuss the role of monitoring in identifying disease progression, and review the current and emerging treatment options for a patient with a pancreatic NET.

Watch Video
Medical Oncologist, Nuclear Medicine Physician and Endocrinologist

Watch leading specialists from the MDT discuss the diagnostic workup and challenges associated with diagnosing rare and unusual NETs.
Expert Spotlight
Dr Diane Reidy-Lagunes
MSK Cancer Center, New York, NY, USA
Dr Thomas Hope
UCSF Health, San Francisco, CA, USA
Prof. Dr med. Marianne Pavel
Friedrich–Alexander University, Erlangen, Germany

Dr Diane Reidy-Lagunes, Dr Thomas Hope and Prof. Dr med. Marianne Pavel discuss the challenges in diagnosing patients with rare and unusual NETs, and the importance of referral to a specialist unit to ensure patients receive the appropriate diagnostic workup for a timely and accurate diagnosis.

Listen on the Go

 Learn more Back to MDT Hub Time: 12:35
 
Medical Oncologist, Nuclear Medicine Physician and Endocrinologist

Watch leading specialists from the MDT discuss the importance of a coordinated approach to clinical decision making in a patient with a lung NET.
Expert Spotlight
Dr Diane Reidy-Lagunes
MSK Cancer Center, New York, NY, USA
Dr Thomas Hope
UCSF Health, San Francisco, CA, USA
Prof. Dr med. Marianne Pavel
Friedrich–Alexander University, Erlangen, Germany

Dr Diane Reidy-Lagunes, Dr Thomas Hope and Prof. Dr med. Marianne Pavel discuss the case of a female patient who presents with a lung NET and the factors guiding treatment selection, together with emerging treatment options for patients with lung NETs.

Listen on the Go

 Learn more Back to MDT Hub Time: 10:32
 
Medical Oncologist, Nuclear Medicine Physician, Nurse Specialist

Watch leading specialists from the MDT discuss the side effects associated with treatment in a patient with liver-dominant disease and the importance of addressing patient needs in ensuring adherence to treatment.

Expert Spotlight
Dr Diane Reidy-Lagunes
MSK Cancer Center, New York, NY, USA
Dr Thomas Hope
UCSF Health, San Francisco, CA, USA
Ms Catherine Bouvier Ellis
Neuroendocrine Cancer UK, Leamington Spa, UK

Dr Diane Reidy-Lagunes, Dr Thomas Hope and Ms Catherine Bouvier Ellis discuss the case of a patient with a liver-dominant NET of unknown primary origin, and the importance of managing patient expectations regarding long-term treatment and adverse event management.

Listen on the Go

 Learn more Back to MDT Hub Time: 10:07
 
Medical Oncologist, Nuclear Medicine Physician, Endocrinologist, Nurse Specialist

Watch leading specialists from the MDT discuss the role of monitoring in identifying disease progression, and review the current and emerging treatment options for a patient with a pancreatic NET.
Expert Spotlight
Dr Diane Reidy-Lagunes
MSK Cancer Center, New York, NY, USA
Dr Thomas Hope
UCSF Health, San Francisco, CA, USA
Prof. Dr med. Marianne Pavel
Friedrich–Alexander University, Erlangen, Germany
Ms Catherine Bouvier Ellis
Neuroendocrine Cancer UK, Leamington Spa, UK

Dr Diane Reidy-Lagunes, Dr Thomas Hope, Prof. Dr med. Marianne Pavel and Ms Catherine Bouvier Ellis, discuss the current and emerging treatment options for a patient with a well-differentiated pancreatic NET who now presents with progressive disease.

Listen on the Go

 Learn more Back to MDT Hub Time: 11:23
 
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Overview & Learning Objectives
Overview

Watch leading specialists discuss new and emerging approaches to diagnosis and treatment of rare and unusual neuroendocrine tumours (NETs), including metastatic pheochromocytomas and paragangliomas (mPPGLs), NETs arising in the lung or pancreas, or with liver-dominant disease, and the impact of effective multidisciplinary teamwork and coordination on patient care.

This activity is jointly provided by USF Health and touchIME. read more

Target Audience

This activity has been designed to meet the educational needs of specialists involved in the multidisciplinary management of neuroendocrine tumours, including oncology specialists (gastro-oncologists and lung oncologists), endocrinologists, gastroenterologists, radiologists and nuclear medicine specialists, and pulmonologists.

Disclosures

USF Health adheres to the Standards for Integrity and Independence in Accredited Continuing Education. All individuals in a position to influence content have disclosed to USF Health any financial relationship with an ineligible organization. USF Health has reviewed and mitigated all relevant financial relationships related to the content of the activity. The relevant relationships are listed below. All individuals not listed have no relevant financial relationships.

Faculty

Dr Diane Reidy-Lagunes discloses Advisory board/panel fees from Advanced Accelerator Applications, Chiasma Inc., Ipsen and Novartis. Grants/research support fees from Ipsen, Merck and Novartis.

Dr Thomas Hope discloses Advisory board or panel fees from BlueEarth Diagnostics; Ipsen (relationship terminated). Consultancy fees from Bayer, Curium, ITM and RayzeBio. Grant/research support from Advanced Accelerator Applications, Bayer, Clovis Oncology and GE Healthcare; Advanced Accelerators Applications and Philips (relationships terminated). Stock/shareholder (self-managed) from RayzeBio.

Prof. Marianne Pavel discloses Advisory board or panel fees from Advanced Accelerators Applications, Esteve Pharmaceuticals (Riemser Pharma), Ipsen, Lilly, MSD, Novartis. Consultancy fees from Advanced Accelerators Applications, Boehringer Ingelheim, HUTCHMED, Ipsen.

Ms Catherine Bouvier Ellis discloses Grant/research support from Advanced Accelerator Applications and Ipsen.

Content reviewer

Angela Massey Hill, Pharm.D., CPh, RPh. has no financial interests/relationships or affiliations in relation to this activity.

Touch Medical Director

Kathy Day has no financial interests/relationships or affiliations in relation to this activity.

USF Health Office of Continuing Professional Development and touchIME staff have no financial interests/relationships or affiliations in relation to this activity.

Requirements for Successful Completion

In order to receive credit for this activity, participants must review the content and complete the post-test and evaluation form. Statements of credit are awarded upon successful completion of the post-test and evaluation form.

If you have questions regarding credit please contact cpdsupport@usf.edu.

Accreditations

Physicians

This activity has been planned and implemented in accordance with the accreditation requirements and policies of the Accreditation Council for Continuing Medical Education (ACCME) through a joint providership of USF Health and touchIME. USF Health is accredited by the ACCME to provide continuing medical education for physicians.

USF Health designates this enduring material for a maximum of 1.0 AMA PRA Category 1 CreditTM. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

The European Union of Medical Specialists (UEMS) – European Accreditation Council for Continuing Medical Education (EACCME) has an agreement of mutual recognition of continuing medical education (CME) credit with the American Medical Association (AMA). European physicians interested in converting AMA PRA Category 1 CreditTM into European CME credit (ECMEC) should contact the UEMS (www.uems.eu).

Advanced Practice Providers

Physician Assistants may claim a maximum of 1.0 Category 1 credits for completing this activity. NCCPA accepts AMA PRA Category 1 CreditTM from organizations accredited by ACCME or a recognized state medical society.

The AANPCP accepts certificates of participation for educational activities approved for AMA PRA Category 1 CreditTM by ACCME-accredited providers. APRNs who participate will receive a certificate of completion commensurate with the extent of their participation.

Date of original release: 27 September 2022. Date credits expire: 27 September 2023.

If you have any questions regarding credit please contact cpdsupport@usf.edu

Learning Objectives

After watching this activity, participants should be better able to:

  • Recognize the role of the MDT in addressing diagnostic and treatment challenges associated with rare and unusual neuroendocrine tumours (NETs)
  • Evaluate the factors guiding treatment selection and clinical management decisions in rare and unusual NETs
  • Appraise the efficacy and safety of new and emerging treatment options for rare and unusual NETs
Faculty & Disclosures
Dr Diane Reidy-Lagunes

Memorial Sloan Kettering Cancer Center, New York, NY, USA

Diane Reidy-Lagunes, MD, is the vice chair of the Regional Care Network at the Memorial Sloan Kettering (MSK) Cancer Center and past president of MSK’s medical staff. During the COVID crisis, she served as chief of clinical care in the COVID setting as part of the Hospital Incident Command System (HICS). read more

As a researcher, Dr Reidy-Lagunes focuses on developing methods to integrate molecular-based therapies into the treatment of neuroendocrine and endocrine tumours. She is a member of the National Cancer Institute Taskforce, and a member of the National Comprehensive Cancer Network Guidelines. 

Dr Reidy-Lagunes is the recipient of several awards and honours, including the Paul Sherlock Resident Housestaff Teaching Award, Castle Connolly Top Doctors, and the American Cancer Society Mother of the Year Award. She is the creator and host of the podcast ‘Cancer Straight Talk from MSK’. In addition, she maintains a high-volume gastrointestinal oncology clinic.

Dr Diane Reidy-Lagunes discloses: Advisory board/panel fees from Advanced Accelerator Applications, Chiasma Inc., Ipsen and Novartis. Grants/research support fees from Ipsen, Merck and Novartis.
Dr Thomas Hope

UCSF Health, San Francisco, CA, USA

Thomas Hope, MD, is the vice chair of clinical operations and strategy in the Department of Radiology, director of molecular therapy, and chief of nuclear medicine at the San Francisco VA Medical Center. He is also chair of the Cancer Center’s Molecular Imaging & Radionuclide Therapy Site Committee. read more

In 2007, he received his medical degree from Stanford University School of Medicine and he completed a 1-year internship at Kaiser Permanente, San Francisco. From 2008 to 2012, Dr Hope completed a residency in diagnostic radiology at the University of California, San Francisco, followed by a clinical fellowship in body magnetic resonance imaging (MRI) and nuclear medicine from Stanford Medical Center in 2013. Dr Hope’s main research focus is on novel imaging agents and therapies, particularly in prostate cancer and neuroendocrine tumours. He led the development of Ga-68 PSMA-11, which subsequently led to the approval of this drug by the FDA. He has combined his interest in MRI with PET in the simultaneous modality PET/MRI, helping lead the development of the clinical PET/MRI programme. Additionally, he leads the PRRT (peptide receptor radionuclide therapy) programme for neuroendocrine tumours and PSMA Radioligand Therapy at UCSF.

Dr Thomas Hope discloses Advisory board or panel fees from BlueEarth Diagnostics; Ipsen (relationship terminated). Consultancy fees from Bayer, Curium, ITM and RayzeBio. Grant/research support from Advanced Accelerator Applications, Bayer, Clovis Oncology and GE Healthcare; Advanced Accelerators Applications and Philips (relationships terminated). Stock/shareholder (self-managed) from RayzeBio.
Prof. Dr med. Marianne Pavel

Friedrich–Alexander University, Erlangen, Germany

Marianne Pavel, MD, is chair of the Division of Endocrinology at the Department of Medicine at Friedrich–Alexander University of Erlangen–Nürnberg, Erlangen, Germany, where she recently established another European Neuroendocrine Tumor Society (ENETS) Centre of Excellence. read more

Prof. Pavel received her medical degree in 1992 from the Georg–August University in Göttingen, Germany. In 1994, she completed her residency at the Friedrich–Alexander University of Erlangen–Nürnberg, Germany. Prof. Pavel became board certified as a specialist in internal medicine in 2000, and in endocrinology and diabetes in 2001. She was appointed as vice head of the Department of Endocrinology and Metabolism at the Friedrich–Alexander University in 2001. 

From May 2007 to April 2017, Prof. Pavel was assistant director at the Charité University Hospital and the lead of the Neuroendocrine Tumour Section at the Department of Hepatology and Gastroenterology, Charité University Hospital, Campus Virchow–Klinikum in Berlin, Germany. She established a Clinical Trial Centre for NETs at the Charité University and conducted numerous clinical trials in NETs. In 2009, under her guidance, the NET Centre at the Charité University was certified as one of the first ENETS Centres of Excellence. Prof. Pavel was conferred a professorship for NET disease at the Charité University in January 2010. Since 2005, she has participated in ENETS activities and has been a member of the Executive Committee of ENETS since April 2014. Prof. Pavel was president of ENETS from April 2020 to April 2022.

Prof. Marianne Pavel discloses Advisory board or panel fees from Advanced Accelerators Applications, Esteve Pharmaceuticals (Riemser Pharma), Ipsen, Lilly, MSD, Novartis. Consultancy fees from Advanced Accelerators Applications, Boehringer Ingelheim, HUTCHMED, Ipsen.
Ms Catherine Bouvier Ellis

Neuroendocrine Cancer UK, Leamington Spa, UK

Catherine Bouvier Ellis has dedicated the past 20 years of her career to improving the care and outcomes for patients with neuroendocrine cancer. As the first dedicated neuroendocrine cancer clinical nurse specialist (CNS) in the UK, and having set up the first UK-wide charity, she has experienced extensive challenges alongside a significant sense of achievement. read more

Her passion is to see the following eight strategic priorities be achieved in the field of neuroendocrine cancer: 

  • Protecting the UK’s world-class neuroendocrine cancer services 
  • Improving the impact and outcomes for patients who follow a less common disease pathway 
  • Establishing patient experience as being on a par with clinical effectiveness and safety 
  • Promoting the necessary investment and supportive infrastructure to deliver a modern and high-quality service for patients, both in the acute and community setting 
  • Providing standardized, useful and accurate patient and public information 
  • Significantly increasing research, education and awareness in neuroendocrine cancers 
  • Ensuring commissioning processes are fit for purpose 
  • Promoting equity – the right of all diagnosed with cancer

Ms Catherine Bouvier Ellis discloses Grant/research support from Advanced Accelerator Applications and Ipsen.
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This Activity Is Funded By:

An independent medical education grant from Advanced Accelerator Applications, and is provided by USF Health and touchIME.

The information provided by this CME activity is for continuing education purposes only and is not meant to substitute for the independent medical/clinical judgment of a healthcare provider relative to diagnostic and treatment options of a specific patient’s medical condition.

USF is an Equal Opportunity / Affirmative Action / Equal Access Institution.

USF Health is accredited by the Accreditation Council for Continuing Medical Education, the American Nurses Credentialing Center and the Accreditation Council for Pharmacy Education to provide continuing education to healthcare professionals. As an accredited provider, USF Health is required to disclose personal information to relevant accredited bodies that certify CME to process credits/contact hours, comply with reporting requirements, and for internal recordkeeping and regulatory purposes. USF Health does not share or sell any individual’s contact information or unique identifiers to any commercial supporter, advertiser, or third party without the specific permission of the individual.

Unapproved products or unapproved uses of approved products may be discussed by the faculty; these situations may reflect the approval status in one or more jurisdictions. The presenting faculty have been advised by touchIME and USF Health to ensure that they disclose any such references made to unlabelled or unapproved use. No endorsement by touchIME or USF Health of any unapproved products or unapproved uses is either made or implied by mention of these products or uses in touchIME or USF Health activities. touchIME and USF Health accept no responsibility for errors or omissions.

This content is intended for healthcare professionals.

Date of original release: 27 September 2022. Date credits expire: 27 September 2023.

This content is intended for healthcare professionals only. Please confirm that you are a healthcare professional.

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Question 1/5
Based on available real-world data, which of the following best describes the current diagnostic landscape for NETs?

HCP, healthcare professional; NET, neuroendocrine tumour.
 Correct

Real-world data show that many patients with NETs are frequently misdiagnosed initially. Data from the global Survey of Challenges in Access to Diagnostics and Treatment for Neuroendocrine Tumour Patients (SCAN Survey) revealed that 44% of patients (n/N=1,043/2,359) were initially misdiagnosed with other conditions at least once.1

SCAN Survey data also revealed that the majority of patients in the EU (55%) and USA (61%) had stage IV disease at NET diagnosis (compared with 46% of patients globally).2 The first global NET survey reported that over one-half of patients (58%) had metastases at the time of diagnosis.3

Of patients who were misdiagnosed, 81% were not accurately diagnosed within 1 year and the mean time to accurate diagnosis was 5 years.1 Most HCPs (86%) responding to a global survey reported limited access to specialist diagnostic tools (including 68Ga-PET/CT scans) as an ongoing challenge.4

Abbreviations

68Ga-PET/CT, 68Gallium-positron emission tomography/computed tomography; HCP, healthcare professional; NET, neuroendocrine tumour. 

References

  1. Bouvier C, Jervis N. Curr Opin Endocrine Metabol Res. 2021;18:254–7.
  2. Kolaraova T, et al. Ann Oncol. 2021;32(Suppl. 5):S917.
  3. Singh S, et al. J Glob Oncol. 2017;3:43–53.
  4. Leyden S, et al. Int J Cancer. 2020;146:1316–23.
Question 2/5
Your 36-year-old female patient presents with recurrent hypertension, headache, palpitations, diaphoresis and pallor. You suspect PPGL. Which hormone tests would you recommend to confirm a diagnosis of PPGL versus a NET?

ACTH, adrenocorticotropic hormone; MIBG, meta-iodobenzylguanidine; NET, neuroendocrine tumour; PPGL, pheochromocytoma/paraganglioma; SPECT, single photon emission computed tomography.
 Correct

Ideally, diagnosis should be performed at a specialist centre with an MDT, as this approach is more likely to catch rare and difficult NETs than local investigations.1 To diagnose PPGL, metanephrine levels are required.2–4 Dexamethasone suppression tests and plasma ACTH are relevant for adrenal, pituitary and ectopic NETs.2,4

Abbreviations

ACTH, adrenocorticotropic hormone; MDT, multidisciplinary team; NET, neuroendocrine tumour;            PPGL, pheochromocytoma/paraganglioma.

References

  1. Morin C, et al. Endocrine Connections. 2022;11:e220097. 
  2. National Comprehensive Cancer Network. NCCN Guidelines: Neuroendocrine and adrenal tumors (version 1.2022; May 23, 2022). Available at: www.nccn.org/guidelines/ (accessed 19 August 2022). 
  3. Fishbein L, et al. Pancreas. 2021;50:469–93.
  4. Fassnacht M, et al. Ann Oncol. 2020;31:1476–90.
Question 3/5
Your patient is a 41-year-old female with a bronchopulmonary NET atypical carcinoid tumour, with Ki67 of 30% and p53 wildtype. You have ordered a DOTATATE PET/CT scan; how should you interpret the avidity of your scan results with regard to choice of PRRT versus chemotherapy for this patient?

NET, neuroendocrine tumour; PET/CT, positron electron tomography/computed tomography; PRRT, peptide receptor radionuclide therapy; SUV, standard uptake value.
 Correct

SUV on DOTATATE PET/CT has been correlated with treatment response to PRRT in patients with NETs, with a higher SUV (defined as greater uptake than the liver) associated with improved response to therapy.1 For patients unlikely to respond to PRRT, chemotherapy represents the alternative treatment option.2

Abbreviations

NET, neuroendocrine tumour; PET/CT, positron electron tomography/computed tomography;                      PRRT, peptide receptor radionuclide therapy; SUV, standard uptake value.

Reference

  1. Puliani G, et al. Front Endocrinol (Lausanne). 2022;13:861434.
  2. Fassnacht M, et al. Ann Oncol. 2020;31:1476–90.
Question 4/5
Your patient underwent resection and embolization, and is receiving SSA treatment for a liver-dominant NET of unknown primary origin. During their latest follow-up appointment, they admit to feeling sad and tired all the time. They report worrying about the impact of symptoms on home and work life. How would you approach the next steps with your patient?

NET, neuroendocrine tumour; SSA, somatostatin analogue.
 Correct

Global survey data show that general fatigue (including muscle fatigue and weakness) and diarrhoea are often reported by patients with NETs (56% and 48%, respectively).1 According to real-world data, most patients (71%) with NETs report a moderate to substantial burden of disease on their daily life, including negative impacts on energy levels and role function.1 Patients also report increased satisfaction with treatment when they are visiting a specialist centre,2 indicating the importance of a capable multidisciplinary team to address their individual patient needs, including psychological support.3

Abbreviation

NET, neuroendocrine tumour.

References

  1. Singh S, et al. J Glob Oncol. 2017;3:43–53.
  2. Wolin EM, et al. Pancreas. 2017;46:639–47. 
  3. Bouvier C, et al. Curr Opin Endocrine Metabol Res. 2021;18:254–7.
Question 5/5
Which concept is currently being studied in the Alliance A021901 trial?:

NET, neuroendocrine tumour; PRRT, peptide receptor radionuclide therapy.
 Correct

The Alliance A021901 study is investigating PRRT versus everolimus in patients with somatostatin receptor-positive bronchial NETs.1 Cabozantinib versus placebo is being studied in patients with advanced NETs, including bronchial NETs, in the CABINET trial,2 capecitabine plus temozolomide in metastatic lung neuroendocrine neoplasms is under investigation in the CAPTEM (capecitabine to temozolomide) trial,3 and everolimus plus best supportive care is the subject of the RADIANT4 study.4

Abbreviations

NET, neuroendocrine tumour; PRRT, peptide receptor radionuclide therapy.

References

  1. NCT04665739. Available at: https://clinicaltrials.gov/ (accessed 26 August 2022).
  2. NCT03375320. Available at: https://clinicaltrials.gov/ (accessed 26 August 2022).
  3. Al-Toubah T, et al. Oncologist. 2020;25:e48–52.
  4. Fazio N, et al. Cancer Sci. 2018;109:174–81.
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