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.