An Investigation of Pituitary Tumors and Related Hypothalmic Disorders

  • STATUS
    Recruiting
  • participants needed
    2000
  • sponsor
    Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD)
Updated on 19 September 2021
pituitary
craniopharyngioma
linkage analysis
cushing's syndrome
central nervous system tumors

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)<TAB>training our fellows and students in the identification of genetic defects associated with pituitary tumor formation, and

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

As a research study, this protocol aims at

(i)<TAB>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)<TAB>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.

Description

There are a variety of tumors affecting the pituitary gland; 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 patients with tumors of the hypothalamic-pituitary unit to the 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 (only) patients with Cushing disease, and (c) to study (longitudinally) whether CS in pediatric patients is also associated with a hypercoagulable state. 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 patients with tumors of the pituitary gland and, thus, lead to the development of better therapeutic regimens for these neoplasms.

Details
Condition Cushing's disease, Endocrine disorder, Prolactinoma, Cushing's Syndrome, Craniopharyngioma, Pituitary Adenoma, Atresia, Hypopituitarism, CONGENITAL ANOMALY NOS, Growth Hormone Deficiencies/Abnormalities, Female Hormonal Deficiencies/Abnormalities, Male Hormonal Deficiencies/Abnormalities, Gigantism/Acromegaly, Gigantism/Acromegaly, Birth Defects, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, prolactinomas, panhypopituitarism, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly, Gigantism/Acromegaly
Clinical Study IdentifierNCT00001595
SponsorEunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD)
Last Modified on19 September 2021

Eligibility

Yes No Not Sure

Inclusion Criteria

Patients eligible for the study (3- 70 years old)
Evidence for the existence of a tumor of the hypothalamic-pituitary unit, as indicated by previously obtained imaging studies or
biochemical investigation of the hypothalamo-hypophyseal function
Family members (any age) of patients with a family history of tumors and who will agree to participate in the DNA/linkage analysis study
Member of a kindred suspected of having an inherited form of pituitary neoplasia, as evidenced by results of a patient studied under A (above)

Exclusion Criteria

Children younger than 3 years cannot be admitted and treated at the NIH Clinical Center due to lack of specialized infant and small
children units; likewise, older patients may not be admitted due to lack of
appropriate geriatric care
Pregnant women are excluded from enrollment
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