# Handbook of Genetic Counseling/Pituitary Tumor

Pituitary Tumor

## Introduction

* Acknowledge prior contact and receipt of family history information
* Assess the most important concerns since there are so many issues at hand
* Discuss family plans (timing, size, etc.) and how those plans may be influenced by what is learned today

## Intake and Family History

* Review family history information and personal health information already obtained
* Use pedigree to make the review easier
* Specifically ask about cancer in the family
* Ask about pituitary tumor
** When was it diagnosed? How? By whom?
** What symptoms was she having?
** Did she receive any treatment beside the drug mentioned?
** Was surgery or radiation therapy ever discussed?
** Has her physician discussed recurrence risks with her?

## What is the Pituitary Gland?

* The pituitary gland is a small, bean-shaped gland located in the center of the brain just above the back of the nose
* The pituitary gland makes hormones that affect the growth and functions of other glands in the body; therefore it is sometimes called the "master gland"
* Some hormones produced by the pituitary include: Thyroid Stimulating Hormone, Growth Hormone, Adrenocorticotropic Hormone, Luteinizing and Follicle Stimulating Hormones, Vasopressin, and others
* When there is an abnormality of the pituitary gland, it often produces either too much or too little of one or a combination of hormones. This can result in a variety of hormonal problems that can affect the whole body.
* Enlargement of the gland can also cause visual disturbances and/or impairment

## Pituitary Tumors

* Pituitary tumors or adenomas are almost always benign which means that they grow slowly and do not spread to other parts of the body
* Pituitary tumors are rare.
* Some may be "silent" lesions that are incidentally found at autopsy
* The manifestations of a pituitary tumor depend on the hormones secreted by the tumor and the pattern of growth
* Some hormonally active tumors are those that produce adrenocorticotropic hormone (ACTH), prolactin, or growth-hormone
* Hormonally-inactive adenomas are often larger and may exhibit invasive properties. Thus, the likelihood for blindness is greater in patients with this tumor type
* Pituitary tumors are seen in one of the multiple endocrine neoplasia (MEN) syndromes. MEN type I is well recognized, dominantly inherited, and comprises tumors of the parathyroid, pancreas and pituitary. Eighty percent of patients have involvement of 2 or more glands, and pituitary tumors occur in 54% to 80% of patients with MEN I.
* MRI most readily determines the presence of a pituitary tumor, although it may also be established by CT (computer tomography) scan.
* Many patients experience visual loss. Headaches occur in about 20%.

## Treatment

* Treatment depends upon the type of tumor and if it extends into the brain around the pituitary.
* Hormone-secreting tumors may be treated with surgery or radiation therapy. Drug therapy with bromocriptine has been used with success in patients with prolactin-secreting tumors.
* Lesions that extend beyond the confines of the pituitary often require radiation therapy and surgery

## Risk for

* I could not find a population rate for pituitary tumors, only that they are "very rare"
* sister was probably treated with bromocriptine which was used in the past for lactation cessation and is currently used to treat prolactin-secreting pituitary tumors
* Her tumor was likely small at the start, and she has responded well to treatment thus far
* Even if sister has a recurrence of the tumor, it will not affect the risk that this couple would have a child with a pituitary tumor

## References

* Buckman, Robert. What you Really Need to Know about Cancer, p61.
* CancerNet (A service of the NCI) online database
* DeVita, Vincent T., Samuel Hellman, and Steven A. Rosenbert. Cancer: Principles & Practice of Oncology. Volume 2: pp1725, 2066-2068
* Genetics in Clinical Oncology, pp108-109.
* Pituitary Network Association (PNA) website

## Notes

The information in this outline was last updated in 2002.