<clinical_study>
  <!-- This xml conforms to an XML Schema at:
    https://clinicaltrials.gov/ct2/html/images/info/public.xsd -->
  <required_header>
    <download_date>ClinicalTrials.gov processed this data on September 20, 2023</download_date>
    <link_text>Link to the current ClinicalTrials.gov record.</link_text>
    <url>https://clinicaltrials.gov/ct2/show/NCT04447014</url>
  </required_header>
  <id_info>
    <org_study_id>200110</org_study_id>
    <secondary_id>20-C-0110</secondary_id>
    <nct_id>NCT04447014</nct_id>
  </id_info>
  <brief_title>Natural History Study of Children and Adults With Adrenocortical Cancer (ACC)</brief_title>
  <official_title>Natural History Study of Children and Adults With Adrenocortical Cancer</official_title>
  <sponsors>
    <lead_sponsor>
      <agency>National Cancer Institute (NCI)</agency>
      <agency_class>NIH</agency_class>
    </lead_sponsor>
  </sponsors>
  <source>National Institutes of Health Clinical Center (CC)</source>
  <brief_summary>
    <textblock>
      Background:&#xD;
&#xD;
      Adrenocortical cancer (ACC) is a rare tumor. The prognosis is very poor for people with&#xD;
      advanced stages of ACC. Some people may live with ACC for years; others live for just months.&#xD;
      Treatment options for ACC often do not work well. Researchers want to study the clinical&#xD;
      course of the disease. They want to understand how adrenocortical cancer appear on imaging&#xD;
      scans, how they respond to therapies, and the best treatment for them.&#xD;
&#xD;
      Objective:&#xD;
&#xD;
      To gain a better understanding of adrenal cancer.&#xD;
&#xD;
      Eligibility:&#xD;
&#xD;
      People ages 2 and older with ACC who are enrolled in NCI protocol 19-C-0016&#xD;
&#xD;
      Design:&#xD;
&#xD;
      Participants will be screened with a review of their medical records, tumor scans, and cancer&#xD;
      test results and reports.&#xD;
&#xD;
      Participants may have CT and other scans. For the scans, they will lie in a machine that&#xD;
      takes pictures of the body. They may have blood tests. They may have a 24-hour urine&#xD;
      collection. They may be asked to sign a new consent form for some of these tests.&#xD;
&#xD;
      Participants will complete paper or electronic surveys. The surveys will ask about the&#xD;
      effects of cancer on their emotional, physical, and behavioral well-being.&#xD;
&#xD;
      Participants will receive recommendations about how to manage their issues and potential&#xD;
      treatment options for their cancer.&#xD;
&#xD;
      Participants home physician will be contacted every 6 to 12 months to collect medical&#xD;
      information such as test results and scans.&#xD;
&#xD;
      Participants may be asked to return to the NIH every 6 to 12 months for follow-up tests.&#xD;
&#xD;
      Participants will contact study staff if there are any changes in their tumor.&#xD;
&#xD;
      Participants will be followed on this study for life.&#xD;
    </textblock>
  </brief_summary>
  <detailed_description>
    <textblock>
      Background:&#xD;
&#xD;
        -  Adrenocortical carcinoma (ACC) is a rare tumor with an incidence of 1.5 to 2 per million&#xD;
           people per year. It has a very poor prognosis with an overall 5-year mortality rate of&#xD;
           75-90% and an average survival from the time of diagnosis of 14.5 months. Approximately&#xD;
           10% of ACC cases are associated with a hereditary cancer predisposition syndrome.&#xD;
&#xD;
        -  The treatment of choice for a localized primary or recurrent tumor is surgical&#xD;
           resection. Patients with recurrent or metastatic disease are infrequently curable by&#xD;
           surgery alone.&#xD;
&#xD;
        -  As with most solid tumors, chemotherapy options have limited benefit, although&#xD;
           platinumbased therapies have response rates of 25 to 30%. To date no targeted therapy&#xD;
           has been shown to be of any value in this disease. The role of neoadjuvant and adjuvant&#xD;
           therapies, including systemic chemotherapy and radiotherapy, remains poorly defined and&#xD;
           has been reported to have only a modest or no therapeutic effect.&#xD;
&#xD;
        -  The natural history of ACC can vary greatly with some patients surviving only months&#xD;
           while others can live with disease for years. The basis for these differing clinical&#xD;
           presentations is not known. While one cannot exclude an immune or other host component&#xD;
           as responsible for the diverse clinical courses, it is also possible that there may be a&#xD;
           genetic basis for this phenomenon.&#xD;
&#xD;
        -  Patients with rare tumors seek expert advice in the management of their care. A natural&#xD;
           history study would establish a more formal mechanism for such referrals, while allowing&#xD;
           the systematic collection of epidemiologic, genomic, molecular data.&#xD;
&#xD;
      Objective:&#xD;
&#xD;
      -Characterize the natural history of adrenocortical cancer (ACC). Data will include patient&#xD;
      demographics, clinical characteristics, patterns of disease progression, response or lack of&#xD;
      response to therapeutic interventions, disease recurrence and overall survival.&#xD;
&#xD;
      Eligibility:&#xD;
&#xD;
        -  Subjects with documented ACC&#xD;
&#xD;
        -  Age greater than or equal to 2 years old.&#xD;
&#xD;
      Design:&#xD;
&#xD;
        -  This protocol is a subprotocol to protocol 19C0016 Natural History and Biospecimen&#xD;
           Accrual Study for Children and Adults with Rare Solid Tumors . After enrollment on the&#xD;
           master protocol and undergoing evaluations detailed in the master protocol, patients&#xD;
           will be enrolled on this subprotocol specific for ACC.&#xD;
&#xD;
        -  Medical history will be collected from medical records and patients followed throughout&#xD;
           the course of their illnesses, with particular attention to patterns of disease&#xD;
           recurrence and progression, response to therapies, duration of responses and hormone&#xD;
           production in patients with hormone production as a manifestation of their disease, and&#xD;
           patient reported outcomes. Tumor growth rates will also be calculated throughout the&#xD;
           course of the disease.&#xD;
    </textblock>
  </detailed_description>
  <overall_status>Terminated</overall_status>
  <why_stopped>
    To avoid the duplication of resources and effort associated with the PI switching branches.&#xD;
  </why_stopped>
  <start_date type="Actual">June 25, 2020</start_date>
  <completion_date type="Actual">January 12, 2023</completion_date>
  <primary_completion_date type="Actual">June 27, 2022</primary_completion_date>
  <study_type>Observational</study_type>
  <has_expanded_access>No</has_expanded_access>
  <study_design_info>
    <observational_model>Cohort</observational_model>
    <time_perspective>Prospective</time_perspective>
  </study_design_info>
  <primary_outcome>
    <measure>Characterize the natural history of adrenocortical cancer (ACC). Data will include clinical presentation, patterns of disease progression, response or lack of response to therapeutic interventions, disease recurrence and overall survival</measure>
    <time_frame>10 years</time_frame>
    <description>Characterize the natural history of adrenocortical cancer (ACC). Data will include clinical presentation, patterns of disease progression, response or lack of response to therapeutic interventions, disease recurrence and overall survival</description>
  </primary_outcome>
  <number_of_groups>1</number_of_groups>
  <enrollment type="Actual">15</enrollment>
  <condition>Adrenocortical Carcinoma</condition>
  <condition>Adrenocortical Cancer</condition>
  <arm_group>
    <arm_group_label>Cohort 1</arm_group_label>
    <description>Subjects with confirmed adrenocortical cancer (ACC)</description>
  </arm_group>
  <eligibility>
    <study_pop>
      <textblock>
        primary clinical&#xD;
      </textblock>
    </study_pop>
    <sampling_method>Probability Sample</sampling_method>
    <criteria>
      <textblock>
        -  INCLUSION CRITIERIA:&#xD;
&#xD;
          -  Subjects enrolled into NCI protocol 19C0016 Natural History and Biospecimen&#xD;
             Acquisition Study for Children and Adults with Rare Solid Tumors&#xD;
&#xD;
          -  Subjects with histologically or cytologically documented ACC.&#xD;
&#xD;
          -  Age greater than or equal to 2 years old&#xD;
&#xD;
          -  Ability of subject or parent/guardian to understand and the willingness to sign a&#xD;
             written consent document.&#xD;
&#xD;
        EXCLUSION CRITIERIA:&#xD;
&#xD;
        None&#xD;
      </textblock>
    </criteria>
    <gender>All</gender>
    <minimum_age>2 Years</minimum_age>
    <maximum_age>N/A</maximum_age>
    <healthy_volunteers>No</healthy_volunteers>
  </eligibility>
  <overall_official>
    <last_name>Jaydira Del Rivero, M.D.</last_name>
    <role>Principal Investigator</role>
    <affiliation>National Cancer Institute (NCI)</affiliation>
  </overall_official>
  <location>
    <facility>
      <name>National Institutes of Health Clinical Center</name>
      <address>
        <city>Bethesda</city>
        <state>Maryland</state>
        <zip>20892</zip>
        <country>United States</country>
      </address>
    </facility>
  </location>
  <location_countries>
    <country>United States</country>
  </location_countries>
  <link>
    <url>https://clinicalstudies.info.nih.gov/cgi/detail.cgi?A_2020-C-0110.html</url>
    <description>NIH Clinical Center Detailed Web Page</description>
  </link>
  <verification_date>January 2023</verification_date>
  <study_first_submitted>June 24, 2020</study_first_submitted>
  <study_first_submitted_qc>June 24, 2020</study_first_submitted_qc>
  <study_first_posted type="Actual">June 25, 2020</study_first_posted>
  <last_update_submitted>January 13, 2023</last_update_submitted>
  <last_update_submitted_qc>January 13, 2023</last_update_submitted_qc>
  <last_update_posted type="Actual">January 17, 2023</last_update_posted>
  <responsible_party>
    <responsible_party_type>Sponsor</responsible_party_type>
  </responsible_party>
  <keyword>Clinical Outcome</keyword>
  <keyword>Advice in the Management of Cancer</keyword>
  <keyword>Patterns of Disease Progression</keyword>
  <keyword>Response or Lack of Response to Therapeutic Interventions</keyword>
  <keyword>Natural History</keyword>
  <condition_browse>
    <!-- CAUTION:  The following MeSH terms are assigned with an imperfect algorithm            -->
    <mesh_term>Adrenocortical Carcinoma</mesh_term>
    <mesh_term>Adrenal Cortex Neoplasms</mesh_term>
  </condition_browse>
  <!-- Results have not yet been posted for this study                                          -->
</clinical_study>


download date: ClinicalTrials.gov processed this data on September 20, 2023
link text: Link to the current ClinicalTrials.gov record.
url: https://clinicaltrials.gov/ct2/show/NCT04447014
org study id: 200110
secondary id: 20-C-0110
nct id: NCT04447014
lead sponsor: 
Background:

      Adrenocortical cancer (ACC) is a rare tumor. The prognosis is very poor for people with
      advanced stages of ACC. Some people may live with ACC for years; others live for just months.
      Treatment options for ACC often do not work well. Researchers want to study the clinical
      course of the disease. They want to understand how adrenocortical cancer appear on imaging
      scans, how they respond to therapies, and the best treatment for them.

      Objective:

      To gain a better understanding of adrenal cancer.

      Eligibility:

      People ages 2 and older with ACC who are enrolled in NCI protocol 19-C-0016

      Design:

      Participants will be screened with a review of their medical records, tumor scans, and cancer
      test results and reports.

      Participants may have CT and other scans. For the scans, they will lie in a machine that
      takes pictures of the body. They may have blood tests. They may have a 24-hour urine
      collection. They may be asked to sign a new consent form for some of these tests.

      Participants will complete paper or electronic surveys. The surveys will ask about the
      effects of cancer on their emotional, physical, and behavioral well-being.

      Participants will receive recommendations about how to manage their issues and potential
      treatment options for their cancer.

      Participants home physician will be contacted every 6 to 12 months to collect medical
      information such as test results and scans.

      Participants may be asked to return to the NIH every 6 to 12 months for follow-up tests.

      Participants will contact study staff if there are any changes in their tumor.

      Participants will be followed on this study for life.
Background:

        -  Adrenocortical carcinoma (ACC) is a rare tumor with an incidence of 1.5 to 2 per million
           people per year. It has a very poor prognosis with an overall 5-year mortality rate of
           75-90% and an average survival from the time of diagnosis of 14.5 months. Approximately
           10% of ACC cases are associated with a hereditary cancer predisposition syndrome.

        -  The treatment of choice for a localized primary or recurrent tumor is surgical
           resection. Patients with recurrent or metastatic disease are infrequently curable by
           surgery alone.

        -  As with most solid tumors, chemotherapy options have limited benefit, although
           platinumbased therapies have response rates of 25 to 30%. To date no targeted therapy
           has been shown to be of any value in this disease. The role of neoadjuvant and adjuvant
           therapies, including systemic chemotherapy and radiotherapy, remains poorly defined and
           has been reported to have only a modest or no therapeutic effect.

        -  The natural history of ACC can vary greatly with some patients surviving only months
           while others can live with disease for years. The basis for these differing clinical
           presentations is not known. While one cannot exclude an immune or other host component
           as responsible for the diverse clinical courses, it is also possible that there may be a
           genetic basis for this phenomenon.

        -  Patients with rare tumors seek expert advice in the management of their care. A natural
           history study would establish a more formal mechanism for such referrals, while allowing
           the systematic collection of epidemiologic, genomic, molecular data.

      Objective:

      -Characterize the natural history of adrenocortical cancer (ACC). Data will include patient
      demographics, clinical characteristics, patterns of disease progression, response or lack of
      response to therapeutic interventions, disease recurrence and overall survival.

      Eligibility:

        -  Subjects with documented ACC

        -  Age greater than or equal to 2 years old.

      Design:

        -  This protocol is a subprotocol to protocol 19C0016 Natural History and Biospecimen
           Accrual Study for Children and Adults with Rare Solid Tumors . After enrollment on the
           master protocol and undergoing evaluations detailed in the master protocol, patients
           will be enrolled on this subprotocol specific for ACC.

        -  Medical history will be collected from medical records and patients followed throughout
           the course of their illnesses, with particular attention to patterns of disease
           recurrence and progression, response to therapies, duration of responses and hormone
           production in patients with hormone production as a manifestation of their disease, and
           patient reported outcomes. Tumor growth rates will also be calculated throughout the
           course of the disease.
observational model: Cohort
time perspective: Prospective
measure: Characterize the natural history of adrenocortical cancer (ACC). Data will include clinical presentation, patterns of disease progression, response or lack of response to therapeutic interventions, disease recurrence and overall survival
time frame: 10 years
description: Characterize the natural history of adrenocortical cancer (ACC). Data will include clinical presentation, patterns of disease progression, response or lack of response to therapeutic interventions, disease recurrence and overall survival
arm group label: Cohort 1
description: Subjects with confirmed adrenocortical cancer (ACC)
study pop: 
sampling method: Probability Sample
criteria: 
gender: All
minimum age: 2 Years
maximum age: N/A
healthy volunteers: No
last name: Jaydira Del Rivero, M.D.
role: Principal Investigator
affiliation: National Cancer Institute (NCI)
facility: 
country: United States
url: https://clinicalstudies.info.nih.gov/cgi/detail.cgi?A_2020-C-0110.html
description: NIH Clinical Center Detailed Web Page
responsible party type: Sponsor
mesh term: Adrenocortical Carcinoma
mesh term: Adrenal Cortex Neoplasms