Diagnosis and Management of Pituitary Disorders (Contemporary Endocrinology)

Dispelling the Myths about Rarely Diagnosed Pituitary Disorders
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After completing this educational activity, participants will be able to do the following:. Register Now. Overview Innovations and advances in the field of pituitary disease — including evaluation, diagnosis, treatment, and prevention — continue to occur at a rapid pace, challenging both specialists and generalists to stay abreast of the data and their implications for clinical practice.

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However, age-related changes and associated pathological conditions should be individually evaluated to determine optimal indications and modalities of hormone-replacement therapy. Of particular importance are abnormalities of glucose metabolism, cardiovascular diseases, bone demineralization and the eventuality of endocrine-dependent cancer.

Pituitary Patient Resource Guide Sixth Edition Additional Information and References

Basic medical treatment of hypopituitarism in the elderly relies on thyroid- and adrenal-replacement therapy, which is mandatory in all documented cases. There is no counter-indications to thyroid- or adrenal-replacement therapy in the elderly, provided that overtreatment is avoided because of the potential adverse effects of both hormones on the cardiovascular system and bone metabolism L-Thyroxine is the treatment of choice for central hypothyroidism and should be preceded by adequate adrenal replacement.

Clinical parameters and both plasma FT 3 and FT 4 concentrations should be used for treatment monitoring, since low thyroid-stimulating hormone levels in such patients are not indicative of overtreatment, which may be best recognized by FT 3 measurement in some cases To our knowledge, in the absence of age-related recommendations concerning adrenal replacement in the elderly, it is reasonable to use the lowest daily dose able to correct clinical symptoms such as asthenia and hypotension and maintain serum electrolytes in the normal range in our experience a 25 mg daily dose of cortisone acetate is sufficient in most cases; see Table 2.

Pre-operative treatment of adrenal and thyroid failure are advisable before surgery if indicated. Replacement therapy with sex steroids should be considered with caution in the elderly, because of its potential cardiovascular side effects and increase in steroid-dependent cancer risk. In women, oestrogen-replacement therapy is generally no longer considered in this age group, since most authors agree to recommend it for a limited period after menopause In men, stable levels of plasma testosterone in the low normal range can be best achieved by transdermal preparations, which avoid the supraphysiological peaks following intramuscular injections.

GH-replacement therapy has also been proposed to elderly hypopituitary patients. Recent data from KIMS show beneficial effects almost similar to those seen in younger patients; however, adverse side effects were more frequent than in younger patients 77 and lower doses of GH may be required Thus, indications and dose regimen of GH replacement in this age group still need to be better defined.

In addition, the close relationship between the IGF system, cell proliferation and cancer, and epidemiological evidence that increased circulating IGF-I levels are associated with an increased risk for the development of several common malignant neoplasia 79 , should be kept in mind. Of note, these include breast and prostate cancer, so that combined replacement therapy with sex steroids and GH should be proposed with special caution. Recently, a synergic effect of testosterone and GH-replacement therapy on prostate volume has been documented after only 12 months of treatment in adult hypopituitary patients, including an elderly subgroup, strongly indicating that a longer follow-up is required to look for the possible development of prostate abnormalities Conventional fractionated radiotherapy is frequently administered to patients with residual or recurrent pituitary disease.

An increased risk of cerebrovascular mortality compared with the general population has been reported in patients with PAs treated with surgery and radiotherapy 84 , Possible risk factors include hypopituitarism, radiotherapy and extent of surgery, but none are at present proven causes.

No consensus guidelines are available about the use of radiation therapy in elderly patients with PAs, but limited experience indicates that conventional radiotherapy is feasible in this age group.

Anterior Pituitary - CRASH! Medical Review Series

Though it has been proposed as an alternative to surgery in selected cases 6 , 11 , it is generally viewed as a post-operative treatment, which reduces the recurrence rate in the elderly too Table 3. Considering the low incidence of recurrences following total or subtotal tumour resection and the shorter expectancy of life in such patients, it is reasonable to propose that radiotherapy should not be planned for grossly resected NFPAs unless regrowth can be documented by radiological follow-up, while it may be considered in patients with large post-operative residual tumour abutting the optic chiasm.

Prolactinomas are generally reported to be less sensitive to radiotherapy, but experience is relatively scarce because of its limited indications. Thus, medical therapy is advisable for elderly patients with post-operative remnants of GH-or PRL-secreting tumours to achieve a rapid normalization of hormonal hypersecretion, whereas radiotherapy remains an important option in those unresponsive to medical treatment.

Single-dose stereotactic techniques using either a gamma knife or a modified linear accelerator, also known as radiosurgery, have been developed to achieve high disease control rates while minimizing the risk to the central nervous system. Because of the damaging effects of single high-radiation doses, radiosurgery is only suitable for small lesions at least 5 mm away from the optic chiasm. Although more long follow-up periods are needed, it is likely that radiosurgery can partially overcome the limits due to the slow effects of conventional irradiation.

Another promising approach is stereotactic conformal radiotherapy, which combines the precision of stereotactic techniques with the fractionation of conventional techniques Though longer follow-up periods are also necessary to better assess its efficacy and toxicity 87 , it may be especially attractive for use in the elderly.

An increasing proportion of PAs are being recognized in the elderly, raising the question of their optimal management. On the contrary, medical treatment with DA is the first-line treatment for prolactinomas, regardless of their volume and the presence of neurological symptoms. SMAs are useful in acromegalic patients with large tumours, with a high anaesthesiological risk or with a post-operative active disease.

Background

Although radiotherapy can play an important role in the control of tumour regrowth and hormonal hypersecretion, its indications and modalities in the elderly should be better defined. The clinical importance of hypopituitarism should not be underestimated, and thyroid- and adrenal-replacement therapy are mandatory in the presence of documented hormone deficiency, carefully avoiding overtreatment to limit possible side effects on the cardiovascular system and bone mineralization.

Table 1 Pituitary adenomas in the elderly: current available literature. Clinical presentation The clear predominance of NFPAs largely explains the leading symptoms reported in this age group. Diagnosis The diagnosis of pituitary tumours relies on neuroradiological imaging — essentially magnetic resonance imaging — and endocrine assessment of pituitary function. Peculiarity of bioclinical endocrine changes in the elderly Ageing is associated with a number of endocrine and metabolic changes changes, including significant changes in the hypothalamo-pituitary axis and related peripheral endocrine glands Medical therapy The development of new therapeutic agents has dramatically increased the opportunity for successful medical therapy for secreting PAs over the last two decades.

Treatment of hypopituitarism The impact of hypopituitarism on life quality should be considered in elderly as well as in younger patients. Radiotherapy Conventional fractionated radiotherapy is frequently administered to patients with residual or recurrent pituitary disease. Conclusion An increasing proportion of PAs are being recognized in the elderly, raising the question of their optimal management.

Table 2 Clinical presentation and evolution of nine elderly patients with prolactinomas. Reference No. Headache 3—6 months ; amenorrhea 44 years old ; primary infertility; osteoporosis. Ma- SSE grade A, invasive cavernous sinus , max diam 2. Progressive visual defects 2 years ; headache 5 months ; post- partum amenorrhea 38 years old ; osteoporosis. Ma- SSE grade B, invasive cavernous sinus and sphenoid sinus.

BRC up to 7. CAB 0. Progressive visual defects , bitemporal hemianopsy, amenorrhea 37 years old , g oitre. Headache , amenorrhea 18 years old , o steoporosis. Some pituitary tumours can exist for years without causing symptoms and some will never produce symptoms. Most pituitary tumours occur in people with no family history of pituitary problems and the condition is not usually passed on from generation to generation.

Diagnosis and Management of Pituitary Disorders

Only very occasionally are tumours inherited - for example, in a condition known as multiple endocrine neoplasia MEN1. It can cause headaches and visual problems or it can press on the pituitary gland, causing it to stop producing the required amount of one or more of the pituitary hormones. This effect can also occur following treatment you are given for a tumour, such as surgery or radiotherapy. The more common pituitary conditions include acromegaly, Cushing's, diabetes insipidus, hypogonadism, hypopituitarism and prolactinoma - please see our individual booklets about these conditions for further information.

This type of tumour or cystic mass is quite rare and congenital from birth. It exerts pressure on the hypothalamus which lies just above the pituitary gland and is responsible for releasing hormones that start and stop the release of pituitary hormones. Faster growing craniopharyngiomas affect children whilst slower growing ones affect adults.

This type of tumour can cause headaches and vision problems and can affect hunger, thirst and sleep patterns. Onset of puberty and growth hormone production in children can also be affected, plus growth hormone production may also be affected in adults. In Rathke's Cleft Cyst, the Rathke's pouch eventually forms the pituitary gland and normally closes early in foetal development.

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A remnant may persist as a cleft that lies within the pituitary gland and occasionally this remnant gives rise to a fluid-filled large cyst called Rathke's Cleft Cyst RCC. This condition is more usually seen in adults but can occur at any age. Arachnoid Cysts and Cystic Pituitary Adenomas are other rare cyst-like tumours. There are two types of ESS: primary and secondary.

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Primary ESS happens when a small anatomical defect above the pituitary gland increases pressure in the sella turcica and causes the gland to flatten out. The pituitary gland may be smaller than usual. Primary ESS can be associated with obesity and high blood pressure in women. The function of the pituitary gland is usually normal and may be an incidental finding when a brain MRI scan is done for other reasons.

Secondary ESS is the result of the pituitary gland regressing within the cavity after an injury, surgery or radiation therapy. Individuals with secondary ESS may have symptoms that are due to the loss of pituitary functions, for example loss of periods, infertility, fatigue and intolerance to stress and infection.

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Elster AD. Roelfsema, O. Although gonadotroph adenomas may exceptionally trigger symptoms related to gonadotropin hypersecretion, they account for the vast majority of non-functioning pituitary adenomas and therefore will not be discussed in the present review [ 1 , 2 ]. The newest medication for Cushing syndrome is pasireotide Signifor , and it works by decreasing ACTH production from a pituitary tumor. Smit, and J. Pituitary Tumors Associated with Acromegaly. Feldkamp J, Santen R, Harms E, et al: Incidentally discovered pituitary lesions: high frequency of macroadenomas and hormone-secreting adenomas — results of a prospective study.

In children, ESS can be associated with early onset of puberty, growth hormone deficiency and pituitary tumours. MRI scans are useful in evaluating ESS and differentiating it from other disorders that produce an enlarged sella. The resulting severe blood loss causes tissue death in her pituitary gland and leads to hypopituitarism following the birth. Conditions that increase the risk of an obstetric haemorrhage include multiple pregnancies twins or triplets and abnormalities of the placenta. Blood tests would establish hormone levels and a scan to rule out other abnormalities of the pituitary such as a tumour.

The affected glands may then produce abnormally increased amounts of hormones which in turn cause a variety of different symptoms. Diagnosis is made when a patient has two or more growths common to MEN, or a patient has only one growth, but there is a family history of MEN.