ClinicalTrials.gg
RecruitingNCT00001595Updated Oct 19, 2026

An Investigation of Pituitary Tumors and Related Hypothalmic Disorders

An observational study in Panhypopituitarism, Gigantism/Acromegaly and Prolactinoma, sponsored by Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD). Recruiting at 1 site in United States. Open to participants aged 2 Years to 70 Years, including healthy volunteers. Per ClinicalTrials.gov, last updated 2026-10-19.

Sponsored by Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD) · Observational

From the registry’s dates

  • Started Apr 1997; still recruiting 29 years 6 months later.
Study type
Observational
Model
Cohort
Time perspective
Prospective
Enrollment
2,000
Ages
2 Years to 70 Years
Sex
All
01

Study summary

There is a variety of tumors affecting the pituitary gland in childhood; some of these tumors (eg craniopharyngioma) are included among the most common central nervous system tumors in childhood. The gene(s) involved in the pathogenesis of these tumors are largely not known; their possible association with other developmental defects or inheritance pattern(s) has not been investigated. The present study serves as a (i) screening/training, and, (ii) a research protocol.

As a screening and training study, this protocol allows our Institute to admit children with tumors of the hypothalamic-pituitary unit to the pediatric endocrine clinics and wards of the NIH Clinical Center for the purposes of

(i) training our fellows and students in the identification of genetic defects associated with pituitary tumor formation, and

(ii) teaching our fellows and students the recognition, management and complications of pituitary tumors

As a research study, this protocol aims at

(i) developing new clinical studies for the recognition and therapy of pituitary tumors; as an example, two new studies have emerged within the context of this protocol: (a) investigation of a new research magnetic resonance imaging (MRI) tool and its usefulness in the identification of pituitary tumors, and (b) investigation of the psychological effects of cortisol secretion in pediatric patients with Cushing disease. Continuation of this protocol will eventually lead to new, separate protocols that will address all aspects of diagnosis of pituitary tumors and their therapy in childhood.

(ii) Identifying the genetic components of pituitary oncogenesis; those will be investigated by (a) studying the inheritance pattern of pituitary tumors in childhood and their possible association with other conditions in the families of the patients, and (ii) collecting tumor tissues and examining their molecular genetics. As with the clinical studies, the present protocol may help generate ideas for future studies on the treatment and clinical follow up of pediatric patients with tumors of the pituitary gland and, thus, lead to the development of better therapeutic regimens for these neoplasms.

Read the detailed description

Study Description:\<TAB>

This protocol aims to evaluate subjects with tumors of the hypothalamic pituitary unit to: identify genetic components of pituitary and hypothalamic oncogenesis, to develop new clinical studies for the recognition and therapy of pituitary tumors, and to investigate the psychological effects of cortisol secretion.

Objectives:\<TAB>

Primary Objective:

To collect peripheral blood samples and tumor tissues and examine the molecular genetics of the specimens, in an effort to elucidate developmental pathways leading to pituitary gland oncogenesis and/or other defects.

Secondary Objectives:

To serve as a screening protocol for future studies on the treatment and clinical follow up of our patients with pituitary tumors; it is our hope that the protocol will continue to be a vehicle for the development of more related clinical studies.

To collect biospecimens.

To evaluate the cognitive, psychological, and patient-reported health status of mental and social well-being and symptoms of hypercortisolemia and adrenal insufficiency (AI) associated with recovery from Cushing syndrome (CS) in children with this disease.

Endpoints:

\<TAB>

Primary Endpoint:

Molecular genetic testing

Secondary Endpoints:

Pre- and/or post- treatment assessment of research variables related to pituitary/hypothalamic tumors and comorbidities.

Cognitive, behavioral, psychological, and patient-reported outcomes

02

Conditions studied

  • Panhypopituitarism
  • Gigantism/Acromegaly
  • Prolactinoma
  • Cushing Disease

Keywords

  • Developmental Defect
  • Oncogenesis
  • Evaluation and Management
  • Psychological
  • Cushing Disease
  • Natural History
03

In context

Acromegaly

202 studies on the registry are indexed under Acromegaly; 19 are open to participants now.

This study's planned enrollment of 2,000 is above the median of 100 across 81 observational studies indexed under Acromegaly.

Browse Acromegaly studies →

Lead sponsor

Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD) is the lead sponsor of 422 studies on the registry; 24 are open to participants now.

Of its 14 completed or terminated interventional studies of FDA-regulated products, 11 (79%) have results posted.

Counted across the registry records on this site, refreshed daily.

04

Who can participate

Ages eligible
2 Years to 70 Years
Sexes eligible
All
Accepts healthy volunteers
Yes
Sampling method
Non-probability sample

Study population

Subjects with known or suspected pituitary or hypothalamic tumors or related disorders. Family members (adult and pediatric; affected and unaffected) may be enrolled in the DNA linkage analysis part of this protocol.

Go through the criteria one by one — yes, no or not sure — and take the list to your doctor or the study team. Kept in this browser only.

Inclusion criteria

In order to be eligible to participate in this study, an individual must meet all of the following criteria:

  1. Stated willingness to comply with all study procedures and availability for the duration of the study.
  2. Male or female with:

    1. Evidence for the existence of a tumor of the hypothalamic-pituitary unit or related disorder, as indicated by previously obtained imaging studies or biochemical investigation of the hypothalamo-hypophyseal function (aged 2 years to 70 years)

      or

    2. Family members (any age) of patients with a family history of tumors of the hypothalamic-pituitary unit or related disorders as part of the linkage part of the study, or
    3. Members (any age) of a kindred suspected of having an inherited form of neoplasia of the hypothalamic-pituitary unit or related disorder, as evidenced by results of a patient enrolled in this protocol, as part of the linkage part of the study
  3. Ability of the subject or LAR to understand and the willingness to sign a written document.

Exclusion criteria

EXCLUSION CRITERIA:

An individual who meets any of the following criteria will be excluded from participation in this study:

  1. Pregnancy: Pregnancy will be evaluated only in participants of reproductive age (from 10 years old until 60 years of age unless menopause has already occurred per clinical report of the participant).

    For participants enrolled as external participants or under the Linkage study (where research activities include no more than blood draws), any female who could possibly become pregnant will be screened using clinical criteria (history, with pregnancy testing only if indicated) for exclusion and this information will be documented in the consent process note in EMR. If a participant has initially been registered as external location and then presents on-site, then pregnancy test will be performed if within the reproductive age group.

  2. Patients with any medical, physical, psychiatric, or social condition, which, in the opinion of the investigators, would make participation in this protocol not in their best interest, will be excluded from the study.
  3. Patients who are critically ill, unstable, or with severe organ failure that may affect/limit the endocrine evaluation and place unsustainable demands on CC or NICHD resources may be excluded.
Terms in these criteria, in plain language(1)

Underlined terms in the criteria open these too. Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.

Informed consent

Before you join, the study team explains what taking part involves — its purpose, what happens, the risks and possible benefits — and answers your questions, so you can decide freely. Consent is usually recorded on a form (the informed consent form, ICF) signed by you or, where the law allows, by someone legally authorised to decide for you.

You can keep asking questions during the study, you are told of new information that matters, and you can choose to leave at any time.

05

Study design

Observational model
Cohort
Time perspective
Prospective
Enrollment
2,000 participants (estimated)

Groups and cohorts

  • Patients with pituitary tumors or hypothalmic defects

    Patients with pituitary tumors or hypothalmic defects

    Procedure: Tissue specimen collection · Procedure: MRI

Interventions

  • ProcedureTissue specimen collection

    Tissue specimen collection

  • ProcedureMRI

    MRI scan

06

What researchers measure

Primary outcomes

  1. Molecular genetic testing, whole exome sequencing

    To collect peripheral blood samples and tumor tissues and examine the molecular genetics of the specimens, in an effort to elucidate developmental pathways leading to pituitary gland oncogenesis and/or other defects.

    Time frame: ongoing

Secondary outcomes

  1. To investigate the usefulness of a new MRI of the pituitary gland

    Comparison of MRI techniques to identify micro adenomas

    Time frame: completed

  2. To identify the clinical and genetic features of pituitary tumors by investigating their inheritance pattern and possible association with other conditions in the families of the patients.

    Genetic studies of pituitary and hypothalamic tumors or related disorders

    Time frame: ongoing

  3. To evaluate the cognitive, psychological, and patient-reported health status of mental and social well-being and symptoms of adrenal insufficiency associated with Cushing syndrome in children with this disease.

    Descriptive study of cognitive, psychological, and patient-reported health status of mental and social well-being associated with Cushing and after treatment

    Time frame: ongoing

  4. To collect peripheral blood samples and tumor tissues and examine the molecular genetics of the specimens, in an effort to elucidate developmental pathways leading to pituitary gland oncogenesis and/or other defects

    Genetic studies of pituitary and hypothalamic tumors or related disorders

    Time frame: ongoing

07

Study locations

1 of 1 sites recruiting
  • National Institutes of Health Clinical Center
    Bethesda, Maryland 20892, United States
    Recruiting
    • For more information at the NIH Clinical Center contact Office of Patient Recruitment (OPR) · Contactccopr@nih.gov · 800-411-1222
08

References and documents

Publications

  • Trivellin G, Daly AF, Faucz FR, Yuan B, Rostomyan L, Larco DO, Schernthaner-Reiter MH, Szarek E, Leal LF, Caberg JH, Castermans E, Villa C, Dimopoulos A, Chittiboina P, Xekouki P, Shah N, Metzger D, Lysy PA, Ferrante E, Strebkova N, Mazerkina N, Zatelli MC, Lodish M, Horvath A, de Alexandre RB, Manning AD, Levy I, Keil MF, Sierra Mde L, Palmeira L, Coppieters W, Georges M, Naves LA, Jamar M, Bours V, Wu TJ, Choong CS, Bertherat J, Chanson P, Kamenicky P, Farrell WE, Barlier A, Quezado M, Bjelobaba I, Stojilkovic SS, Wess J, Costanzi S, Liu P, Lupski JR, Beckers A, Stratakis CA. Gigantism and acromegaly due to Xq26 microduplications and GPR101 mutation. N Engl J Med. 2014 Dec 18;371(25):2363-74. doi: 10.1056/NEJMoa1408028. Epub 2014 Dec 3. PubMed 25470569 ↗
  • Stratakis CA, Carney JA, Lin JP, Papanicolaou DA, Karl M, Kastner DL, Pras E, Chrousos GP. Carney complex, a familial multiple neoplasia and lentiginosis syndrome. Analysis of 11 kindreds and linkage to the short arm of chromosome 2. J Clin Invest. 1996 Feb 1;97(3):699-705. doi: 10.1172/JCI118467. PubMed 8609225 ↗
  • Magiakou MA, Mastorakos G, Oldfield EH, Gomez MT, Doppman JL, Cutler GB Jr, Nieman LK, Chrousos GP. Cushing's syndrome in children and adolescents. Presentation, diagnosis, and therapy. N Engl J Med. 1994 Sep 8;331(10):629-36. doi: 10.1056/NEJM199409083311002. PubMed 8052272 ↗
  • Trivellin G, Sanchez-Gaya V, Grasso A, Pasinska M, Stratakis CA, Milnes D, Kirk EP, Beckers A, Lania AG, Petrossians P, Rada-Iglesias A, Franke M, Daly AF. Distinguishing benign from pathogenic duplications involving GPR101 and VGLL1-adjacent enhancers in the clinical setting with the bioinformatic tool POSTRE. NPJ Genom Med. 2026 Jan 15;11(1):12. doi: 10.1038/s41525-025-00548-7. PubMed 41540017 ↗
  • Omotosho YB, Reynolds JC, Yanovski JA, Tatsi C. Body Composition and Fat Deposition in Children with Cushing Disease and Associations with Cardiometabolic Risk Factors. J Pediatr. 2026 Mar;290:114933. doi: 10.1016/j.jpeds.2025.114933. Epub 2025 Dec 3. PubMed 41349930 ↗
  • Zainab R, Kaur S, Lack J, Similuk M, Tandon M, Ghosh R, Seifert BA, Tokita M, Flippo C, Yan J, Walkiewicz M, Chittiboina P, Tatsi C. Genetic evaluation of pediatric pituitary adenomas and USP8-related genotype-phenotype correlations in Cushing's disease. Pituitary. 2025 Aug 14;28(5):92. doi: 10.1007/s11102-025-01557-6. PubMed 40813536 ↗
  • Hernandez-Ramirez LC, Pankratz N, Lane J, Faucz FR, Chittiboina P, Kay DM, Beethem Z, Mills JL, Stratakis CA. Genetic drivers of Cushing's disease: Frequency and associated phenotypes. Genet Med. 2022 Dec;24(12):2516-2525. doi: 10.1016/j.gim.2022.08.021. Epub 2022 Sep 23. PubMed 36149413 ↗
  • Martinez de LaPiscina I, Hernandez-Ramirez LC, Portillo N, Gomez-Gila AL, Urrutia I, Martinez-Salazar R, Garcia-Castano A, Aguayo A, Rica I, Gaztambide S, Faucz FR, Keil MF, Lodish MB, Quezado M, Pankratz N, Chittiboina P, Lane J, Kay DM, Mills JL, Castano L, Stratakis CA. Rare Germline DICER1 Variants in Pediatric Patients With Cushing's Disease: What Is Their Role? Front Endocrinol (Lausanne). 2020 Jul 3;11:433. doi: 10.3389/fendo.2020.00433. eCollection 2020. PubMed 32714280 ↗
  • Chasseloup F, Pankratz N, Lane J, Faucz FR, Keil MF, Chittiboina P, Kay DM, Hussein Tayeb T, Stratakis CA, Mills JL, Hernandez-Ramirez LC. Germline CDKN1B Loss-of-Function Variants Cause Pediatric Cushing's Disease With or Without an MEN4 Phenotype. J Clin Endocrinol Metab. 2020 Jun 1;105(6):1983-2005. doi: 10.1210/clinem/dgaa160. PubMed 32232325 ↗
  • Hodes A, Meyer J, Lodish MB, Stratakis CA, Zilbermint M. Mini-review of hair cortisol concentration for evaluation of Cushing syndrome. Expert Rev Endocrinol Metab. 2018 Sep;13(5):225-231. doi: 10.1080/17446651.2018.1517043. Epub 2018 Sep 20. PubMed 30234410 ↗
  • Saldarriaga C, Lyssikatos C, Belyavskaya E, Keil M, Chittiboina P, Sinaii N, Stratakis CA, Lodish M. Postoperative Diabetes Insipidus and Hyponatremia in Children after Transsphenoidal Surgery for Adrenocorticotropin Hormone and Growth Hormone Secreting Adenomas. J Pediatr. 2018 Apr;195:169-174.e1. doi: 10.1016/j.jpeds.2017.11.042. Epub 2018 Feb 1. PubMed 29395172 ↗
  • Birdwell L, Lodish M, Tirosh A, Chittiboina P, Keil M, Lyssikatos C, Belyavskaya E, Feelders RA, Stratakis CA. Coagulation Profile Dynamics in Pediatric Patients with Cushing Syndrome: A Prospective, Observational Comparative Study. J Pediatr. 2016 Oct;177:227-231. doi: 10.1016/j.jpeds.2016.06.087. Epub 2016 Aug 2. PubMed 27496264 ↗
  • Gourgari E, Lodish M, Keil M, Wesley R, Hill S, Xekouki P, Lyssikatos C, Belyavskaya E, De La Luz SM, Stratakis CA. Post-operative growth is different in various forms of pediatric Cushing's syndrome. Endocr Relat Cancer. 2014;21(6):L27-31. doi: 10.1530/ERC-14-0405. Epub 2014 Sep 25. No abstract available. PubMed 25258026 ↗
  • Lodish MB, Gourgari E, Sinaii N, Hill S, Libuit L, Mastroyannis S, Keil M, Batista DL, Stratakis CA. Skeletal maturation in children with Cushing syndrome is not consistently delayed: the role of corticotropin, obesity, and steroid hormones, and the effect of surgical cure. J Pediatr. 2014 Apr;164(4):801-6. doi: 10.1016/j.jpeds.2013.11.065. Epub 2014 Jan 10. PubMed 24412141 ↗
  • Keil MF, Graf J, Gokarn N, Stratakis CA. Anthropometric measures and fasting insulin levels in children before and after cure of Cushing syndrome. Clin Nutr. 2012 Jun;31(3):359-63. doi: 10.1016/j.clnu.2011.11.007. Epub 2011 Dec 7. PubMed 22154461 ↗
  • Keil MF, Stratakis CA. Facial metrics in children with corticotrophin-producing pituitary adenomas suggest abnormalities in midface development. J Pediatr Endocrinol Metab. 2009 Jan;22(1):47-53. doi: 10.1515/jpem.2009.22.1.47. PubMed 19344074 ↗
  • Keil MF, Merke DP, Gandhi R, Wiggs EA, Obunse K, Stratakis CA. Quality of life in children and adolescents 1-year after cure of Cushing syndrome: a prospective study. Clin Endocrinol (Oxf). 2009 Sep;71(3):326-33. doi: 10.1111/j.1365-2265.2008.03515.x. Epub 2008 Dec 17. PubMed 19170709 ↗

Individual participant data

Plan to share: Yes — Deidentified Samples/data, including genomic data, will be shared with NIH \& non-NIH collaborators after an MTA is obtained. If coded data shared, the key code will not be shared with collaborators \& stay at NIH. If Data \& samples shared with AIs and IC with an FWA/operating under the DOH and reported at the time of CR. Sharing with AIs without an FWA/operating under the DoH will be submitted for prospective IRB approval. Submissions to NIH-sponsored or supported databases and repositories will be reported at the time of CR. Submission to non-NIH sponsored or supported databases \& repositories will be submitted for prospective IRB approval.@@@@@@Required approvals from the collaborating institution will be obtained and materials will be shipped in accordance with NIH and federal regulations. This study will comply with the NIH GDS Policy, which applies to all NIH-funded research that generates large-scale human or non-human GD, as well as the use of these data for subsequent research.

Supporting information: Study protocol

Time frame: AIs in the NIH intramural program may participate with NIH/ non-NIH collaborators under which de-identified human materials are transferred from the intramural program to another site for correlative studies that are part of the approved protocol. In this case, the protocol clearly documents the tests conducted under the correlative studies\& each institution participating in the clinical study is bound by the terms of this Protocol\& their obligations under the statutes \& regulations. @@@@@@This study will comply with the NIH DSP \&Policy on the Dissemination of NIH-Funded CT Information \& Registration \& Results Information Submission rule. As such, this trial will be registered at CT.gov,\& results information from this trial will be submitted to CT.gov. In addition, every attempt will be made to publish results in peer-reviewed journals. Data from this study may be requested from other researchers 3 years after the completion of the primary endpoint by contacting PI or NICHD-DASH.

Access criteria: This study will comply with the NIH Data Sharing Policy and Policy on the Dissemination of NIH-Funded Clinical Trial Information and the Clinical Trials Registration and Results Information Submission rule. As such, this trial will be registered at ClinicalTrials.gov, and results information from this trial will be submitted to ClinicalTrials.gov. In addition, every attempt will be made to publish results in peer-reviewed journals. Data from this study may be requested from other researchers 3 years after the completion of the primary endpoint by contacting PI or NICHD-DASH.

09

Timeline

1 registry update since Sep 25, 2026

Started Apr 1997 (actual).

How these figures are worked out, and which studies are left out of them.

Registered
Nov 3, 1999
First posted
Nov 4, 1999
StartActual
Apr 21, 1997
Last update
Oct 19, 2026
What these dates mean
Start.
When the first participant enrolled; while estimated, when the study expects to open to recruitment.
Actual and Est.
Actual means the registry says the date has happened. Est. (estimated) is a date the sponsor plans or expects. It can still move, and the sponsor is expected to change it to actual once it has passed.
Minor edits
Nothing that changes what the study is or who can join. Edited: verification date
1 update, last Oct 19, 2026
Show all 1 update
  1. Oct 19, 2026
    Minor edits only
    + 1 other change: verification date

From the registry record's own update history. This site started tracking changes on Sep 25, 2026; for anything earlier, see the record history on ClinicalTrials.gov ↗

10

Registry details

Key details

Study ID
NCT00001595
Responsible party
Sponsor
First posted
Nov 4, 1999
Start date
Apr 21, 1997
Last update
Oct 19, 2026

Study contacts

Ka Wing J Lam
Contact
kawing.lam@nih.gov
(301) 402-8084
Deborah P Merke, M.D.
Contact
dmerke@nih.gov
(301) 496-0718
Deborah P Merke, M.D.
principal investigator · Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD)

Oversight

FDA-regulated drug
No
FDA-regulated device
No
View the source record on ClinicalTrials.gov ↗

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