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PheochromocytomaPhase 2NCT07714551

Zanzalintinib in Unresectable and Progressive MPGGs

A Phase 2 study testing Zanzalintinib in people with Pheochromocytoma and Paraganglioma, run by Dana-Farber Cancer Institute at 2 sites in United States.

Not yet recruiting

Opening soon. The study is approved but enrolment has not started yet. Sites may already take enquiries.

Sites and contacts
Official record
Search all trials

Trial summary

This study is to examine the effects of oral daily zanzalintinib in participants with unresectable, progressive metastatic pheochromocytoma or paraganglioma. Read more about this study

Who is it for?
Ages 18 years and older
Conditions the study is for
Pheochromocytoma, Paraganglioma
What is being tested?
Zanzalintinib
Type of treatment
Pill or oral medicine
Could I receive a placebo?
No, everyone receives an active treatment
How many people
14 participants
Where
2 sitesUnited States
Study dates
9 Jan 2027to 1 Jan 2032
Phase
Phase 2
Are there results?
Not yet: the study is under way

Key requirements

Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.

  • Ages 18 years and older
  • All sexes
  • No healthy volunteers
  • Adult, Older adult

Who may be able to join

  • Age ≥ 18 years. As no dosing or adverse event data are currently available in participants < 18 years of age, children and adolescents are excluded from this study.
  • Documentation of Disease
  • Histologic Documentation: Histologically-proven advanced (metastatic or unresectable primary) pheochromocytoma or paraganglioma.
  • Stage: Advanced (metastatic or unresectable primary) disease
  • Tumor Site: Histologically-proven pheochromocytoma or paraganglioma
  • Radiographic Evaluation: Radiographic evidence of disease progression by RECIST v1.1 criteria in the 12 months prior to registration.
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  • Measurable disease
  • Lesions must be accurately measured in at least one dimension (longest diameter to be recorded) as ≥ 1 cm with CT or MRI (or ≥ 1.5 cm for lymph nodes). Non-measurable disease includes disease smaller than these dimensions or lesions considered truly non-measurable including: leptomeningeal disease, ascites, pleural or pericardial effusion, lymphangitic involvement of skin or lung.
  • Prior Treatment
  • Prior treatment with other somatostatin analog, chemotherapy, radiotherapy (including peptide radionuclide receptor therapy [PRRT]), or surgery is permitted.
  • Eastern Cooperative Oncology Group (ECOG) performance status of ≤ 2.
  • Organ and marrow function and laboratory values as follows within 4 days prior to the first dose of zanzalintinib:
  • Absolute neutrophil count (ANC) ≥ 1500/mm3without colony stimulating factor support
  • Platelets ≥ 100,000/mm3
  • Hemoglobin ≥ 9 g/dL
  • Bilirubin ≤ 1.5 the upper limit of normal (ULN). For subjects with known Gilbert's disease, bilirubin ≤ 3.0 mg/dL
  • Serum albumin ≥ 2.8 g/dl
  • Serum creatinine ≤ 1.5 ULN or creatinine clearance (CrCl) ≥ 50 mL/min. For creatinine clearance estimation, the Cockcroft and Gault equation should be used:
  • Male: CrCl (mL/min) = (140 - age) × wt (kg) / (serum creatinine × 72) Female: Multiply above result by 0.85
  • Alanine aminotransferase (ALT) and aspartate aminotransferase (AST) ≤ 3.0 ULN
  • Lipase < 2.0 x the upper limit of normal and no radiologic or clinical evidence of pancreatitis
  • Urine protein/creatinine ratio (UPCR) ≤ 1
  • Serum phosphorus, calcium, potassium ≥ LLN and magnesium ≥ 1.2 mg/dL
  • Capable of understanding and complying with the protocol requirements and has signed the informed consent document.
  • Sexually active patients (men and women) must agree to use medically accepted barrier methods of contraception (eg, male or female condom) during the course of the study and for 4 months after the last dose of study drug(s), even if oral contraceptives are also used. All subjects of reproductive potential must agree to use both a barrier method and a second method of birth control during the course of the study and for 4 months after the last dose of study drug(s).
  • Women of childbearing potential must have a negative pregnancy test at screening. Women of childbearing potential include women who have experienced menarche and who have not undergone successful surgical sterilization (hysterectomy, bilateral tubal ligation, or bilateral oophorectomy) or are not postmenopausal. Post-menopause is defined as amenorrhea ≥ 12 consecutive months. Note: women who have been amenorrheic for 12 or more months are still considered to be of childbearing potential if the amenorrhea is possibly due to prior chemotherapy, antiestrogens, ovarian suppression or any other reversible reason.
  • Both men and women of all races and ethnic groups are eligible for this trial.
  • NIH policy requires that women and members of minority groups and their subpopulations be included in all NIH-supported biomedical and behavioral research projects involving NIH-defined clinical research unless a clear and compelling rationale and justification establishes to the satisfaction of the funding Institute \& Center (IC) Director that inclusion is inappropriate with respect to the health of the subjects or the purpose of the research. Exclusion under other circumstances designated by the Director, NIH, upon the recommendation of an IC Director based on a compelling rationale and justification. Cost is not an acceptable reason for exclusion except when the study would duplicate data from other sources. Women of childbearing potential should not be routinely excluded from participation in clinical research. Please see http://grants.nih.gov/grants/funding/phs398/phs398.pdf.

Who cannot join

  • Received cytotoxic chemotherapy (including investigational cytotoxic chemotherapy) or biologic agents (eg, cytokines or antibodies) within 3 weeks, or nitrosoureas/ mitomycin C within 6 weeks before the first dose of study treatment.
  • Prior treatment with zanzalintinib
  • Radiation therapy for bone metastasis within 2 weeks, any other external radiation therapy within 4 weeks before the first dose of study treatment. Subjects with clinically relevant ongoing complications from prior radiation therapy are not eligible.
  • Received radionuclide treatment (i.e. I 131 meta-iodo- benzyl guanidine) within 6 months of the first dose of study treatment
  • Receipt of any type of small molecule kinase inhibitor (including investigational kinase inhibitor) within 14 days before the first dose of study treatment.
  • Receipt of any other type of investigational agent within 28 days before the first dose of study treatment.
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  • The subject has not recovered to baseline or CTCAE ≤ Grade 1 from toxicity due to all prior therapies except alopecia, fatigue, and other non-clinically significant AEs.
  • Prothrombin time (PT)/ International Normalized Ratio (INR) or partial thromboplastin time (PTT) test ≥ 1.3 the laboratory ULN within 7 days before the first dose of study treatment.
  • The subject requires concomitant treatment, in therapeutic doses, with anticoagulants such as warfarin or warfarin-related agents, heparin, thrombin, or antiplatelet agents (eg, clopidogrel). Low dose aspirin (≤ 81 mg/day), , prophylactic low molecular weight heparin (LMWH), and or specified direct Fxa inhibitors rivaroxaban, edoxaban, or apixabanare permitted in subjects without known brain metastases.
  • Subjects must have discontinued oral anticoagulants within 3 days or 5 half-lives prior to first dose of study treatment, whichever is longer.
  • The subject requires chronic concomitant treatment of strong CYP3A4 inducers (eg, dexamethasone, phenytoin, carbamazepine, rifampin, rifabutin, rifapentin, phenobarbital, and St. John's Wort).
  • Any complementary medications (eg, herbal supplements or traditional Chinese medicines) to treat the disease under study within 2 weeks before first dose of study treatment.
  • Known brain metastases or cranial epidural disease unless adequately treated with radiotherapy and/or surgery (including radiosurgery) and stable for at least 4 weeks before first dose of study treatment. Note: Eligible subjects must be neurologically asymptomatic and without corticosteroid treatment at the time of enrollment. Note: Base of skull lesions without definitive evidence of dural or brain parenchymal involvement are allowed.
  • The subject has experienced any of the following: clinically significant gastrointestinal bleeding within 6 months before the first dose of study treatment hemoptysis of ≥ 0.5 teaspoon (2.5ml) of red blood within 3 months before the first dose of study treatment
  • ·any other signs indicative of pulmonary hemorrhage within 3 months before the first dose of study treatment
  • Radiographic evidence of cavitating pulmonary lesion(s)
  • Tumor invading or encasing > 180 degrees any major blood vessels
  • Evidence of tumor invading the GI tract (esophagus, stomach, small or large bowel, rectum or anus), or any evidence of endotracheal or endobronchial tumor within 28 days before the first dose of zanzalintinib.
  • ·Uncontrolled, significant intercurrent or recent illness including, but not limited to, the following conditions:
  • Cardiovascular disorders including
  • Congestive heart failure (CHF): New York Heart Association (NYHA) Class III (moderate) or Class IV (severe) at the time of screening
  • Concurrent uncontrolled hypertension defined as sustained BP > 150 mm Hg systolic, or > 90 mm Hg diastolic despite optimal antihypertensive treatment within 7 days of the first dose of study treatment
  • Any history of congenital long QT syndrome
  • Any of the following within 6 months before the first dose of study treatment:
  • unstable angina pectoris
  • clinically-significant cardiac arrhythmias
  • stroke (including TIA, or other ischemic event)
  • myocardial infarction
  • thromboembolic event requiring therapeutic anticoagulation (Note: subjects with a venous filter (e.g. vena cava filter) are not eligible for this study)
  • Gastrointestinal disorders particularly those associated with a high risk of perforation or fistula formation including:
  • Any of the following within 28 days before the first dose of study treatment
  • intra-abdominal tumor/metastases invading GI mucosa
  • known gastric or esophageal varices, ascites, pleural effusion, or pericardial fluid requiring drainage in last 4 weeks
  • active peptic ulcer disease; patients must be completely recovered
  • acute flare of inflammatory bowel disease (including ulcerative colitis and Crohn's disease), diverticulitis, cholecystitis, symptomatic cholangitis or appendicitis; patients must be completely recovered from these conditions
  • malabsorption syndrome
  • Any of the following within 6 months before the first dose of study treatment:
  • abdominal fistula
  • gastrointestinal perforation
  • bowel obstruction or gastric outlet obstruction
  • intra-abdominal abscess. Note: Complete resolution of an intra-abdominal abscess must be confirmed prior to initiating treatment with zanzalintinib even if the abscess occurred more than 6 months before the first dose of study treatment.
  • Other disorders associated with a high risk of fistula formation including PEG tube placement within 3 months before the first dose of study therapy
  • -Other clinically significant disorders such as:
  • Active infection requiring systemic treatment within 28 days before the first dose of study treatment.
  • Known infection with acute or chronic hepatitis B or C, known human immunodeficiency virus (HIV) or acquired immunodeficiency syndrome (AIDS)-related illness except for subjects meeting all of the following criteria: (1) on stable anti-retroviral therapy; (2) CD4+ T cell count ≥ 200/µL; and (3) an undetectable viral load. Note: HIV testing will be performed at screening if and as required by local regulation. Note: To be eligible, participants taking CYP inhibitors (eg, zidovudine, ritonavir, cobicistat, didanosine) or CYP3 inducers (efavirenz) must change to a different regimen not including these drugs 7 days prior to initiation of study treatment. Anti-retroviral therapies (ART) must have been received for at least 4 weeks prior to the first dose. Note: CD4+ T cell counts, and viral load are monitored per standard of care by the local health care provider. Serious non-healing wound/ulcer/bone fracture within 28 days before the first dose of study treatment
  • History of organ transplant
  • Concurrent uncompensated hypothyroidism or thyroid dysfunction within 7 days before the first dose of study treatment
  • Major surgery within 12 weeks before the first dose of study treatment. Complete wound healing from major surgery must have occurred 1 month before the first dose of study treatment. Minor surgery within 28 days before the first dose of study treatment with complete wound healing at least 10 days before the first dose of study treatment. Subjects with clinically relevant ongoing complications from prior surgery are not eligible.
  • Unable to swallow tablets
  • A corrected QT interval calculated by the Fridericia formula (QTcF) >500 ms within 28 days before first dose of study treatment. Three ECGs must be performed. If the average of these three consecutive results for QTcF is ≤ 500 msec, the subject meets eligibility in this regard.
  • Pregnant or breastfeeding.
  • A previously identified allergy or hypersensitivity to components of the study treatment formulation.
  • Unable or unwilling to abide by the study protocol or cooperate fully with the investigator or designee.
  • Evidence within 2 years of the start of study treatment of another malignancy which required systemic treatment except for cured nonmelanoma skin cancer or cured in situ cervical carcinoma
  • Any other severe acute or chronic medical or psychiatric condition or laboratory abnormality which, in the judgment of the investigator, would have made the patient inappropriate for entry into this study.
  • Moderate to severe hepatic impairment (Child-Pugh B or C).
  • Requirement for hemodialysis or peritoneal dialysis.

Trial locations and contacts

2 sites in 1 country. Contact the study team or a site near you to ask about taking part.

United States2 sites
  • Brigham and Women's Hospital (BWH)

    Boston, Massachusetts, 02215

    Kimberly Perez, MD, 6176326491, Kimberly_Perez@DFCI.HARVARD.EDU

    Kimberly Perez, MD

  • Dana-Farber Cancer Institute

    Boston, Massachusetts, 02215

    Kimberly Perez, MD, 6176326491, Kimberly_Perez@DFCI.HARVARD.EDU

Other trials in United States

About this study

Official title: A Phase 2 Clinical Trial of Zanzalintinib in Patients With Unresectable and Progressive Metastatic Pheochromocytoma or Paraganglioma (MPPGs)

This study is to examine the effects of oral daily zanzalintinib in participants with unresectable, progressive metastatic pheochromocytoma or paraganglioma.

Read the detailed description

This is a Phase II, single-arm investigational study evaluating zanzalintinib in patients with unresectable and progressive metastatic pheochromocytoma or paraganglioma (MPPGs). Participants will receive zanzalintinib 60 mg orally once daily. Zanzalintinib is investigational and has not been approved by the U.S. Food and Drug Administration as a treatment for any disease.

The research study procedures include: screening for eligibility; medical history; physical exams; vital signs; performance status assessments; tumor imaging with CT, MRI, or PET; blood tests; urine tests; pregnancy testing for women of childbearing potential; electrocardiograms (ECGs); use of previously collected archival tissue; patient drug diary completion; health-related quality-of-life questionnaires; study treatment visits; an end-of-treatment visit; and follow-up by telephone or medical record review.

It is expected that about 14 people will take part in this research study. Participation in this research study will continue for as long as the participant does not have serious side effects and the disease does not get worse. After treatment ends, participants will have an end-of-treatment visit within 30 days of the last dose, followed by long-term follow-up every 3 months for 1 year.

Being tested
Zanzalintinib
Keywords
Pheochromocytoma, Paraganglioma, Metastatic pheochromocytoma or paraganglioma, Unresectable, progressive advanced pheochromocytoma/paraganglioma
Medical subject headings
Pheochromocytoma, Paraganglioma

What participants receive

What participants receive. In a randomised study each person is assigned to one group by chance.

  • ZanzalintinibDrug

    Also called XL092

    Route: Oral Schedule: Daily

Study groups in NCT07714551
Study groupRoleWhat they receive
ZanzalintinibExperimentalParticipants receive zanzalintinib orally once daily.
Receives: Drug: Zanzalintinib

What is being measured

The primary outcome is the main question the study is designed to answer.

  1. Objective Response Rate (ORR)

    ORR was defined as the percentage of participants achieving complete response (CR) or partial response (PR) on treatment based on RECIST 1.1 criteria. Per RECIST 1.1 for target lesions: CR is complete disappearance of all target lesions and PR is at least a 30% decrease in the sum of longest diameter (LD) of target lesions, taking as reference baseline sum LD. PR or better overall response assumes at a minimum incomplete response/stable disease (SD) for the evaluation of non-target lesions and absence of new lesions.

    Measured: Assessed every 8 weeks during treatment. The estimated treatment duration is up to 16 months.

Secondary outcomes (7)
  1. Median Progression-Free Survival (PFS)

    PFS based on Kaplan-Meier method is defined as the time from registration to the earlier of progression or death due to any cause. Participants alive without disease progression are censored at date of last disease evaluation. Per RECIST 1.1 for target lesions: PD is at least a 20% increase in sum LD, taking as reference the smallest sum on study with at least 5 mm absolute increase. For non-target lesions, progression-free means no new lesions or unequivocal progression on existing non-target lesions or not evaluated.

    Measured: Assessed every 8 weeks during treatment. The treatment duration is up to 16 months. If treatment discontinued prior to progression, participants will be followed every 3 months for up to 52 weeks or until death.

  2. Median Overall Survival (OS)

    OS based on Kaplan-Meier method is defined as the time from registration to death due to any cause, or censored at date last known alive.

    Measured: Treatment duration is up to 16 months. Following treatment discontinuation, participants will be followed every 3 months for up to 52 weeks or until death.

  3. Global Adverse Event (AE) Rate

    Global AE rate is defined as the proportion of participants who experience at least one adverse event during the treatment period. Aes are summerized and graded based on Common Terminology Criteria for Adverse Events (CTCAE) version 5.0.

    Measured: AE will be assessed every 2 weeks through Week 8 and every 4 weeks thereafter until the end of treatment (up to 16 months).

  4. Blood Pressure Control Rate

    The blood pressure control rate is defined as the proportion of participants who require the addition or discontinuation of antihypertensive medication during the treatment period.

    Measured: Assessed every 8 weeks on treatment. Treatment duration is up to 16 months.

  5. Biochemical Response Rate

    The biochemical response rate is defined as the proportion of participants who demonstrate improvement in at least one biochemical marker (serum catecholamines, serum metanephrines, methoxytyramine [MTX], urine catecholamines, or urine metanephrines) during the treatment period.

    Measured: Treatment duration is up to 16 months.

  6. Change of Functional Assessment of Cancer Therapy - General (FACT-G) Score from Baseline

    The FACT-G total score is calculated as the sum of the Physical Well-Being (PWB), Social/Family Well-Being (SWB), Emotional Well-Being (EWB), and Functional Well-Being (FWB) subscale scores. Items are scored from 0 to 4, with selected negatively worded items reverse scored according to the FACT-G scoring guidelines. Subscale scores are prorated when items are missing. The total score ranges from 0 to 108, with higher scores indicating better health-related quality of life.

    Measured: Assessed at baseline and then every 8 weeks on treatment. Treatment duration is up to 16 months.

  7. Change of European Organisation for Research and Treatment of Cancer Quality of Life Questionnaire-Core 30 (EORTC QLQ-C30) Score from Baseline

    The EORTC QLQ-C30 is a 30-item questionnaire assessing quality of life and symptoms in patients with cancer. The first 28 items are scored on a 4-point scale ranging from 1 to 4, with higher scores indicating greater symptom burden or impairment. The final 2 items assessing overall health and quality of life are scored on a 7-point scale ranging from 1 to 7, with higher scores indicating better overall quality of life.

    Measured: Assessed at baseline and then every 8 weeks on treatment. Treatment duration is up to 16 months.

Timeline

From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.

  1. First posted20 Jul 2026
  2. Study start9 Jan 2027 (estimated)
  3. Primary completion1 Jan 2031 (estimated)
  4. Study completion1 Jan 2032 (estimated)

Resources

More details

For doctors and researchers: how the study is designed, who runs it, and its registry IDs.

Study design
Study type
Interventional
Phase
Phase 2
Primary purpose
Treatment
Allocation
Not applicable
Intervention model
Single group
Masking
None (open label)
Enrolment
14 participants (estimated)
IDs, dates and publications
ClinicalTrials.gov ID
NCT07714551
Sponsor's protocol ID
26-340
Status last verified
Jul 2026
First submitted
15 Jul 2026
Last updated
20 Jul 2026
Results posted
No
Data monitoring committee
Yes
FDA-regulated drug
Yes
FDA-regulated device
No
Individual data sharing
Yes: The Harvard Cancer Consortium encourages and supports the responsible and ethical sharing of data from clinical trials. De-identified participant data from the final research dataset used in the published manuscript may only be shared under the terms of a Data Use Agreement. Requests may be directed to: \[contact information for Sponsor Investigator or designee\]. The protocol and statistical analysis plan will be made available on Clinicaltrials.gov only as required by federal regulation or as a condition of awards and agreements supporting the research

Questions about this trial

What is this clinical trial studying?

A Phase 2 study testing Zanzalintinib in people with Pheochromocytoma and Paraganglioma, run by Dana-Farber Cancer Institute at 2 sites in United States.

The full registered description is in the About this study section and the official record.

Who may be eligible to take part?

The sponsor lists 28 inclusion and 57 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.

Is this clinical trial currently recruiting?

The record says: opening soon, as of the sponsor's last update on 20 Jul 2026. Status can change between updates, so confirm with the study contact before making plans.

Where is the study taking place?

The record lists 2 sites in United States. Whether a site is taking part right now should be confirmed with that site. See every site.

What are the possible benefits and risks?

They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.

What does this phase mean?

Phase 2 studies give the treatment to a larger group to see whether it has the intended effect and to keep checking safety.

Not every study follows the same path, and some studies (observational studies, device studies) have no phase.

Are results available for this study?

This study is still under way. Results are not available in the source record yet.

How can a doctor refer a patient or contact the research team?

Contact Kimberly Perez, MD (6176326491, Kimberly_Perez@DFCI.HARVARD.EDU), or a site near the patient. AzTrials does not enrol participants or take referrals.

What does the primary outcome measure?

Objective Response Rate (ORR) (time frame: Assessed every 8 weeks during treatment. The estimated treatment duration is up to 16 months.). It is what the study was designed to measure, not a finding.

Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?

No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.

How often is this information updated?

AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 20 Jul 2026.

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