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.

.

Condition	Treatment or Intervention
Abnormalities Craniopharyngioma Cushing's Syndrome Endocrine Disease Pituitary Neoplasm	Procedure: MRI  Procedure: Tissue specimen collection

Further Study Details: 

Expected Total Enrollment:  1200

There is 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 gene 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. 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; and (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 (b) 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 an clinical follow up of patients with tumors of the pituitary gland and, thus, lead to the development of better therapeutic regimens for these neoplasms.

Genders Eligible for Study:  Both

Criteria

INCLUSION CRITERIA:
A. The following criteria must be met for all, who enter the study:
Age 3-70 years
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.
Patients may withdraw from the study at any time.
B. For family members studied for linkage analysis, the following criteria must be met:
Any age
Member of a kindred suspected of having an inherited form of pituitary neoplasia, as evidenced by results of a patient studied under A (above).

Maryland
      National Institute of Child Health and Human Development (NICHD), 9000 Rockville Pike,  Bethesda,  Maryland,  20892,  United States; Recruiting

Study ID Numbers:  970076; 97-CH-0076
Record last reviewed:  February 2, 2005
Last Updated:  February 11, 2005
Record first received:  November 3, 1999
ClinicalTrials.gov Identifier:  NCT00001595
Health Authority: United States: Federal Government
ClinicalTrials.gov processed this record on 2005-04-08