Clinical Trials Logo

Clinical Trial Details — Status: Completed

Administrative data

NCT number NCT02015026
Other study ID # 140029
Secondary ID 14-C-0029
Status Completed
Phase
First received
Last updated
Start date December 13, 2013
Est. completion date March 2, 2018

Study information

Verified date March 2, 2018
Source National Institutes of Health Clinical Center (CC)
Contact n/a
Is FDA regulated No
Health authority
Study type Observational

Clinical Trial Summary

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.

- 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 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. A bio-specimen repository will be a major step towards more comprehensive studies of this very rare and unusual tumor, and allow us to begin to characterize subgroups within the disease.

- Patients with rare tumors seek expert advice in the management of their care. Dr. Fojo has such expertise and is frequently asked to consult in the care of ACC patients throughout the world. A natural history study would establish a more formal mechanism for such referrals, while allowing the systematic collection of epidemiologic data as well as much needed tumor samples.

Objective:

-To characterize the natural history of adrenocortical cancer, and in the process, collect blood, and tissue samples to study genetic/biochemical pathways involved in the development and progression of adrenocortical cancer (ACC).

Eligibility:

- Patients greater than or equal to 12 years of age with biopsy-proven ACC

- Patients greater than or equal to 12 years of age suspected of having ACC

Design/Schema:

- Patients will be offered clinical consultation with treatment recommendations, including standard of care and clinical trial options. Computed tomography scans of the thorax, abdomen and pelvis will be performed for staging purposes as indicated; occasionally, magnetic resonance imaging will be performed for the visualization of lesions in the liver, spine, or other anatomic sites.

- Medical histories will be documented 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. Tumor growth rates will also be calculated throughout the course of the disease.

- Blood and tumor samples will be obtained at baseline and at follow-up intervals when surgery is indicated. Tumor samples may include samples harvested at other facilities during or prior to enrollment on this trial.

- Genetic and epigenetic analysis of tumors and in selected cases expression array analysis will be performed.


Description:

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.

- 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 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. A bio-specimen repository will be a major step towards more comprehensive studies of this very rare and unusual tumor, and allow us to begin to characterize subgroups within the disease.

- 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 data as well as much needed tumor samples.

Objective:

-To characterize the natural history of adrenocortical cancer, and in the process, collect blood, and tissue samples to study genetic/biochemical pathways involved in the development and progression of adrenocortical cancer (ACC).

Eligibility:

- Patients greater than or equal to 12 years of age with biopsy-proven ACC

- Patients greater than or equal to 12 years of age suspected of having ACC

Design/Schema:

- Patients will be offered clinical consultation with treatment recommendations, including standard of care and clinical trial options. Computed tomography scans of the thorax, abdomen and pelvis will be performed for staging purposes as indicated; occasionally, magnetic resonance imaging will be performed for the visualization of lesions in the liver, spine, or other anatomic sites.

- Medical histories will be documented 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. Tumor growth rates will also be calculated throughout the course of the disease.

- Blood and tumor samples will be obtained at baseline and at follow-up intervals when surgery is indicated. Tumor samples may include samples harvested at other facilities during or prior to enrollment on this trial.

- Genetic and epigenetic analysis of tumors and in selected cases expression array analysis will be performed.


Recruitment information / eligibility

Status Completed
Enrollment 67
Est. completion date March 2, 2018
Est. primary completion date October 16, 2015
Accepts healthy volunteers Accepts Healthy Volunteers
Gender All
Age group 12 Years to 100 Years
Eligibility - INCLUSION CRITERIA:

- Patients greater than or equal to 12 years of age with biopsy-proven ACC that has been confirmed by the Laboratory of Pathology, NCI.

- Patients greater than or equal to 12 years of age suspected of having ACC. Patients with suspected ACC will undergo baseline workup at the clinical center and be referred for definitive surgical diagnosis and treatment.

- Ability of subject or Legally Authorized Representative to understand and the willingness to sign a written informed consent document.

Study Design


Related Conditions & MeSH terms


Locations

Country Name City State
United States National Institutes of Health Clinical Center, 9000 Rockville Pike Bethesda Maryland

Sponsors (1)

Lead Sponsor Collaborator
National Cancer Institute (NCI)

Country where clinical trial is conducted

United States, 

References & Publications (3)

Fassnacht M, Terzolo M, Allolio B, Baudin E, Haak H, Berruti A, Welin S, Schade-Brittinger C, Lacroix A, Jarzab B, Sorbye H, Torpy DJ, Stepan V, Schteingart DE, Arlt W, Kroiss M, Leboulleux S, Sperone P, Sundin A, Hermsen I, Hahner S, Willenberg HS, Tabarin A, Quinkler M, de la Fouchardière C, Schlumberger M, Mantero F, Weismann D, Beuschlein F, Gelderblom H, Wilmink H, Sender M, Edgerly M, Kenn W, Fojo T, Müller HH, Skogseid B; FIRM-ACT Study Group. Combination chemotherapy in advanced adrenocortical carcinoma. N Engl J Med. 2012 Jun 7;366(23):2189-97. doi: 10.1056/NEJMoa1200966. Epub 2012 May 2. — View Citation

Stein WD, Yang J, Bates SE, Fojo T. Bevacizumab reduces the growth rate constants of renal carcinomas: a novel algorithm suggests early discontinuation of bevacizumab resulted in a lack of survival advantage. Oncologist. 2008 Oct;13(10):1055-62. doi: 10.1634/theoncologist.2008-0016. Epub 2008 Sep 30. — View Citation

Terzolo M, Berruti A. Adjunctive treatment of adrenocortical carcinoma. Curr Opin Endocrinol Diabetes Obes. 2008 Jun;15(3):221-6. doi: 10.1097/MED.0b013e3282fdf4c0. Review. — View Citation

Outcome

Type Measure Description Time frame Safety issue
Primary 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. 10 years
Secondary Establish a bio-specimen repository for genetic and epigenetic analysis of tumors and in selected cases for expression array analysis by collecting blood, and tissue samples to study the biology of ACC development and progression. 10 years
Secondary Estimate the growth and regression rate constants of tumor treated with standard of care or experimental therapies and correlate with genetic, epigenetic and gene expression profiles 10 years
Secondary Correlate clinical evolution with genetic alterations, DNA methylation patterns and gene expression profiles identified within tumors. 10 years
Secondary Correlate patterns of disease recurrence and impact on progression-free survival and overall survival following a laparoscopic resection or open laparotomy for ACC 10 years
See also
  Status Clinical Trial Phase
Completed NCT04373265 - Study of Relacorilant in Combination With Pembrolizumab for Patients With Adrenocortical Carcinoma Which Produces Too Much Stress Hormone (Cortisol) Phase 1
Recruiting NCT06050057 - Surgical Treatment of Adrenal Diseases- Laparoscopic vs. Robotic-assisted Adrenalectomy
Recruiting NCT06066333 - Study of Radiotherapy and Pembrolizumab in People With Adrenocortical Carcinoma Phase 2
Recruiting NCT03127774 - Surgery and Heated Intraperitoneal Chemotherapy for Adrenocortical Carcinoma Phase 2
Completed NCT01048892 - Seneca Valley Virus-001 and Cyclophosphamide in Treating Young Patients With Relapsed or Refractory Neuroblastoma, Rhabdomyosarcoma, or Rare Tumors With Neuroendocrine Features Phase 1
Recruiting NCT00457587 - Preclinical Study Towards an Immunotherapy in Adrenocortical Carcinoma
Recruiting NCT00669266 - Adrenal Tumors - Pathogenesis and Therapy
Recruiting NCT05999292 - Phase 1 Study of 68Ga-R8760 Phase 1
Completed NCT05361083 - First-in-human Evaluation of [18F]CETO Early Phase 1
Recruiting NCT05660889 - Adrenal Vein Sampling as a Tool to Identify Biomarkers That Aid the Diagnosis of Adrenocortical Carcinoma
Completed NCT00003038 - Combination Chemotherapy With Suramin Plus Doxorubicin in Treating Patients With Advanced Solid Tumors Phase 1
Enrolling by invitation NCT03474237 - A Prospective Cohort Study for Patients With Adrenal Diseases
Recruiting NCT04119024 - Gene Modified Immune Cells (IL13Ralpha2 CAR T Cells) After Conditioning Regimen for the Treatment of Stage IIIC or IV Melanoma or Metastatic Solid Tumors Phase 1
Enrolling by invitation NCT05036434 - Phase II Trial of Pembrolizumab Plus Lenvatinib in Advanced Adrenal Cortical Carcinoma Phase 2
Terminated NCT05012397 - Milademetan in Advanced/Metastatic Solid Tumors Phase 2
Not yet recruiting NCT06333314 - Dostarlimab for Locally Advanced or Metastatic Cancer (Non-colorectal/Non-endometrial) With Tumor dMMR/MSI Phase 2
Withdrawn NCT00469469 - Treatment Study Using Bevacizumab for Patients With Adrenocortical Carcinoma Phase 2
Completed NCT00002608 - Combination Chemotherapy and Tamoxifen in Treating Patients With Solid Tumors Phase 2
Not yet recruiting NCT05839886 - The Adverse Event of Mitotane Therapy in Patients With Adrenocortical Carcinoma
Recruiting NCT05634577 - A Phase II Study to Evaluate the Efficacy and Safety of Pembrolizumab in Combination With Mitotane in Patients With Advanced Adrenocortical Carcinoma Phase 2