An Investigation of Pituitary Tumors and Related Hypothalmic Disorders



Status:Recruiting
Conditions:Cancer, Brain Cancer, Neurology, Women's Studies, Endocrine
Therapuetic Areas:Endocrinology, Neurology, Oncology, Reproductive
Healthy:No
Age Range:3 - 70
Updated:4/6/2019
Start Date:February 13, 1997
Contact:Margaret F Keil, C.R.N.P.
Email:keilm@mail.nih.gov
Phone:(301) 435-3391

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A Clinical and Genetic Investigation of Pituitary and HYPOTHALAMIC Tumors and Related Disorders

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....

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. 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.

- 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).
We found this trial at
1
site
9000 Rockville Pike
Bethesda, Maryland 20892
Phone: 800-411-1222
?
mi
from
Bethesda, MD
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