68-Gallium DOTATATE Scan for Von Hippel-Lindau Syndrome

Overseen By Naris Nilubol, M.D.

Age: Any Age

Sex: Any

Trial Phase: Phase 2

Sponsor: National Cancer Institute (NCI)

Disqualifiers: Inability to undergo imaging

No Placebo Group All trial participants will receive the active study treatment (no placebo)

Prior Safety Data This treatment has passed at least one previous human trial

Approved in 2 Jurisdictions This treatment is already approved in other countries

Compensation: Varies

Number of Visits: 3

740 Participants Needed

What You Need to Know Before You Apply

What is the purpose of this trial?

The trial aims to understand how von Hippel-Lindau (VHL) disease affects the pancreas by studying pancreatic tumors. Researchers seek to determine if a 68-Gallium DOTATATE PET/CT scan can more effectively detect these tumors. Success in this area could lead to improved treatments and care for individuals with VHL. The trial seeks participants aged 12 and older who have VHL and experience tumors or cysts in their pancreas. Participants will undergo various scans and tests to monitor their condition over time. As a Phase 2 trial, this research measures the treatment's effectiveness in an initial, smaller group, allowing participants to contribute to advancements in VHL care.

Will I have to stop taking my current medications?

The trial information does not specify whether you need to stop taking your current medications.

What prior data suggests that the 68-Gallium DOTATATE scan is safe for detecting pancreatic tumors in VHL patients?

Research has shown that 68-Gallium DOTATATE has been safely used to help doctors visualize neuroendocrine tumors. This imaging agent highlights certain tumors, making them easier to detect.

Reports of serious side effects with 68-Gallium DOTATATE have not emerged. However, as this trial is in an early stage, researchers are still carefully studying the treatment's safety. Participants should ask questions and discuss any concerns with the trial team, who can provide detailed information about what to expect. ^{1 2 3 4 5}

Why are researchers excited about this trial?

Researchers are excited about the use of 68-Gallium DOTATATE scans for Von Hippel-Lindau (VHL) syndrome because this imaging agent offers a new way to monitor pancreatic neuroendocrine tumors. Unlike traditional imaging methods, 68-Gallium DOTATATE binds specifically to somatostatin receptors, which are often overexpressed in these tumors, allowing for more precise detection and monitoring. This could potentially lead to earlier interventions and better management of tumor progression in VHL patients, setting it apart from the standard imaging techniques typically used.

What evidence suggests that the 68-Gallium DOTATATE scan is effective for detecting pancreatic tumors in VHL patients?

Research has shown that 68-Gallium DOTATATE PET/CT scans effectively detect tumors in individuals with von Hippel-Lindau (VHL) disease. In a study involving 36 VHL patients, this scan identified more tumors than traditional CT and MRI scans, revealing additional tumors that other methods missed. The trial will compare two approaches: one group will undergo yearly 68-Gallium DOTATATE PET/CT research scans, while the other group will be observed without these scans. This suggests that the 68-Gallium DOTATATE scan might be superior for early tumor detection, which is crucial for managing VHL. By identifying more tumors, this scan could enhance care and treatment planning. ^{1 2 3 6 7}

Who Is on the Research Team?

Naris Nilubol, M.D.

Principal Investigator

National Cancer Institute (NCI)

Are You a Good Fit for This Trial?

This trial is for people aged 12 or older with von Hippel-Lindau (VHL) disease, which causes tumors and cysts in the pancreas. Participants must have a confirmed VHL diagnosis through genetic testing or clinical criteria and be able to give informed consent.

Inclusion Criteria

I have pancreatic issues related to VHL, confirmed by imaging.

I have been diagnosed with VHL based on genetic testing or clinical criteria.

I am 12 years old or older.

Exclusion Criteria

I cannot have repeated imaging tests.

Timeline for a Trial Participant

Screening

Participants are screened for eligibility to participate in the trial

2-4 weeks

1 visit (in-person)

Initial Evaluation

Participants undergo a comprehensive evaluation including physical examination, blood and urine tests, and imaging studies such as CT, PET/CT, MRI, or ultrasound.

1-2 weeks

1 visit (in-person)

Longitudinal Monitoring

Participants are monitored with annual or biennial visits for imaging studies, blood and urine sample collection, and potential surgical recommendations based on tumor growth or metastasis risk.

Ongoing (annually or biennially)

1 visit per year or every 2 years (in-person)

Follow-up

Participants are monitored for safety and effectiveness after any surgical intervention or significant treatment changes.

4 weeks

What Are the Treatments Tested in This Trial?

Interventions

• 68-Gallium DOTATATE

Trial Overview The study aims to understand how VHL affects the pancreas and assess if a new type of scan called 68-Gallium DOTATATE PET/CT can better detect pancreatic tumors in these patients.

How Is the Trial Designed?

Group I: 1/ Arm 1 Experimental Treatment 1 Intervention

Study natural history of VHL pancreatic neuroendocrine tumors with yearly 68-Gallium DOTATATE PET/CT research scans.

Group II: 2/ Arm 2 Active Control 1 Intervention

Study natural history of VHL pancreatic neuroendocrine tumors without research scans.

Who Is Running the Clinical Trial?

National Cancer Institute (NCI)

Lead Sponsor

Trials

14,080

Recruited

41,180,000+

Published Research Related to This Trial

Ga-68 somatostatin receptor PET/CT imaging is effective for diagnosing and managing various malignancies associated with Von Hippel-Lindau (VHL) disease, which includes a range of tumors such as hemangioblastomas and pheochromocytomas.

This imaging technique serves as a comprehensive tool for evaluating and monitoring the multiple tumor types that can arise in patients with VHL disease, highlighting its potential as a 'one-stop-shop' for clinical management.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Ga-68 Somatostatin Receptor PET/CT in von Hippel-Lindau Disease. Oh, JR., Kulkarni, H., Carreras, C., et al.[2021]

68Ga-DOTANOC PET/CT imaging can reveal somatostatin receptor expression in various conditions, including in cases of Von Hippel-Lindau syndrome, which is linked to multiple tumor types.

In a specific case, the imaging technique showed tracer uptake in swollen eyelids of a patient suspected of having Von Hippel-Lindau syndrome, but clinical examination later confirmed the condition as viral conjunctivitis, highlighting the importance of accurate diagnosis.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Incidental Detection of Viral Conjunctivitis on 68Ga-DOTANOC PET/CT in a Patient Suspected With Von Hippel-Lindau Syndrome. Mathur, Y., Krishnaraju, VS., Singh, H., et al.[2023]

In a study of 14 patients with Von Hippel-Lindau (VHL) disease, the 68Ga-DOTANOC PET/CT scan successfully detected a total of 67 lesions across various sites, demonstrating its efficacy in identifying both benign and malignant tumors associated with this hereditary condition.

The 68Ga-DOTANOC PET/CT scan may serve as a convenient one-stop imaging solution for VHL patients, potentially replacing multiple separate imaging tests, thus saving time and reducing costs for patients undergoing screening and follow-up evaluations.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

68Ga-DOTANOC PET/CT for Screening and Surveillance of Von Hippel-Lindau (VHL) disease. Shamim, SA., Arora, G., Kumar, N., et al.[2023]

In a study involving 36 patients with von Hippel-Lindau (VHL) disease, 68Ga-DOTATATE PET/CT demonstrated a higher detection rate of lesions compared to traditional imaging methods like CT and MRI, making it a promising tool for monitoring tumors associated with VHL.

68Ga-DOTATATE PET/CT not only showed superior detection capabilities but also offered lower radiation exposure, suggesting it could be a safer and more effective option for the surveillance of VHL-related tumors.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

The utility of 68Gallium-DOTATATE PET/CT in the detection of von Hippel-Lindau disease associated tumors. Shell, J., Tirosh, A., Millo, C., et al.[2021]

68 Ga-DOTATATE PET/CT imaging proved effective in identifying previously unknown primary tumors and metastatic disease in sinonasal neuroendocrine tumors, highlighting its potential for better diagnosis compared to conventional imaging methods.

In two cases of esthesioneuroblastoma, 68 Ga-DOTATATE detected abnormal uptake that was not visible on 18F-FDG PET/CT, suggesting it may be crucial for treatment planning, including identifying candidates for targeted therapies like 177 Lu-DOTATATE.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

The value of 68 Gallium-DOTATATE PET/CT in sinonasal neuroendocrine tumor management: A case series. Liu, KY., Goldrich, DY., Ninan, SJ., et al.[2021]

A rare case of metastatic renal cell carcinoma was identified in a 66-year-old man with von Hippel-Lindau syndrome, showing 68Ga-DOTATATE uptake in multiple locations, including the pancreas and spine.

The presence of 68Ga-DOTATATE avidity in VHL-associated cancers can influence clinical decision-making, highlighting the importance of this imaging technique in detecting and managing such rare metastatic presentations.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

68Ga-DOTATATE Avid Metastatic Vertebral Renal Cell Carcinoma in the Setting of von Hippel-Lindau Syndrome. Patel, M., Jha, A., Antic, T., et al.[2023]

In a study of 81 patients with neuroendocrine tumors (NETs), Ga-68 DOTATATE PET/CT was more effective than F-18 FDG PET/CT in detecting positive scans in patients with low and intermediate-grade tumors, with 82.1% of DOTATATE scans positive compared to 54.5% for FDG (P = 0.04).

For high-grade, poorly-differentiated NETs, both DOTATATE and FDG PET/CT scans were equally effective, with all patients showing positive results, indicating that while DOTATATE is superior for lower-grade tumors, it does not provide additional benefits for high-grade tumors compared to FDG.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Ga-68 DOTATATE PET/CT and F-18 FDG PET/CT in the evaluation of low and intermediate versus high-grade neuroendocrine tumors. You, H., Kandathil, A., Beg, M., et al.[2021]

Gallium-68 DOTATATE imaging effectively identified the primary tumor site in 45% of patients with neuroendocrine tumors (NETs) of unknown primary origin, highlighting its utility in disease localization.

The study found that systemic exposure to long-acting somatostatin analogs did not significantly affect the mean Standardized Uptake Value (SUV) in gallium-68 DOTATATE scans, suggesting that these treatments do not compromise imaging quality.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Post FDA approval analysis of 200 gallium-68 DOTATATE imaging: A retrospective analysis in neuroendocrine tumor patients. Chauhan, A., El-Khouli, R., Waits, T., et al.[2023]

In a study of 20 patients with von Hippel-Lindau disease, (68)Ga-DOTATOC PET/CT detected pancreatic neuroendocrine tumors (pNETs) in 55% of patients, outperforming traditional imaging methods like CT and MRI, which identified only 45%.

The study suggests that (68)Ga-DOTATOC PET/CT is a more effective screening tool for pNETs in vHLD patients compared to the previously recommended (111)In-octreoscan, as it identified a higher proportion of tumors without any patients showing progressive disease during follow-up.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Potential role of (68)Ga-DOTATOC PET/CT in screening for pancreatic neuroendocrine tumour in patients with von Hippel-Lindau disease. Prasad, V., Tiling, N., Denecke, T., et al.[2019]

Citations

1. pmc.ncbi.nlm.nih.gov pmc.ncbi.nlm.nih.gov/articles/PMC8290514/

The utility of 68Gallium-DOTATATE PET/CT in the detection of ... Ga-DOTATATE PET/CT has a higher detection rate for VHL-associated lesions when compared to CT and MRI. There was no significant difference in the number of ...

2. pmc.ncbi.nlm.nih.gov pmc.ncbi.nlm.nih.gov/articles/PMC10504222/

68Ga-DOTANOC PET/CT for Screening and Surveillance of ... Ga-DOTANOC scan was able to detect additional 43 lesions (64%) in 14 patients. In 8 of 14 patients, 68 Ga-DOTANOC scan helped in establishing/ ...

3. clinicaltrials.gov clinicaltrials.gov/study/NCT04074135

Natural History and Management of Von Hippel-Lindau ... Study natural history of VHL pancreatic neuroendocrine tumors with yearly 68-Gallium DOTATATE PET/CT research scans. Intervention/Treatment, Drug : 68-Gallium ...

4. withpower.com withpower.com/trial/phase-3-neuroendocrine-tumors-5-2020-c36f1?condition=Pancreatic+Neuroendocrine+Tumors&overallStatus=Recruiting&hasNoPlacebo=true

68-Gallium DOTATATE Scan for Von Hippel-Lindau ... In a study involving 36 patients with von Hippel-Lindau (VHL) disease, 68Ga-DOTATATE PET/CT demonstrated a higher detection rate of lesions compared to ...

5. jnm.snmjournals.org jnm.snmjournals.org/content/61/supplement_1/127

Role of 68Ga-DOTANOC PET/CT scan in von Hippel-Lindau ... Conclusions: Our results show that 68Ga-DOTANOC PET/CT may be a useful modality for screening and follow up of associated tumours in ...

6. journals.lww.com journals.lww.com/nuclearmed/fulltext/2017/03000/18f_fdg_and_68ga_dotatate_pet_ct_in_von.6.aspx

18F-FDG and 68Ga-DOTATATE PET/CT in von Hippel- ... We report a case of a VHL patient with retinal hemangioblastoma who underwent PET/CT scans using 18F-FDG and 68Ga-DOTATATE. The tumor showed low-level 18F-FDG ...

7. mayoclinic.org mayoclinic.org/drugs-supplements/gallium-ga-68-dotatate-intravenous-route/description/drg-20312288

Gallium ga 68 dotatate (intravenous route) - Side effects & ... Safety and efficacy have not been established. Geriatric. Appropriate studies performed to date have not demonstrated geriatric-specific ...