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We have recently reported the presence of aromatase P450 in the rat hypophysis. This enzyme is responsible for the aromatization of testosterone to estradiol. Since the induction of prolactinomas has been demonstrated in the rat following chronic treatment with estradiol, the aim of the present study was to analyze whether a relationship exists between the presence of pituitary aromatase and the appearance...
A 29-year-old man presented with diplopia and decreased strength in the left arm. A magnetic resonance image (MRI) showed an extensive hypothalamic and pituitary gland mass, and hormonal studies showed partial hypopituitarism and mild hyperprolactinemia without diabetes insipidus. Biopsies of the hypothalamic lesion and of a mediastinal lymph node demonstrated noncaseating granulomas, and a Kveim-Siltzbach...
Lymphocytic Hypophysitis is a rare autoimmune disease of the pituitary presenting mainly with features of a mass lesion and loss of pituitary function. Its cousre is quite unpredictable and its treatment is still controversial as experience in the field remains scarce. We describe a 45 year-old woman with a history of recurrent fever and meningeal symptoms who was referred 3 years later to our department...
Chronic effects of excessive GII secretion are not only disabling but also disfiguring. Most acromegalic patients present with 4–10 years history of changes in features, bony overgrowth, soft tissue swelling, skin changes, diabetes mellitus, hypertension and other cardiovascular symptoms. In addition to sign and symptoms of GII excess the patients demonstrate a loss initiative and spontaneity, mood...
Around fifteen percent of the deaths reported in acromegaly are attributable to malignancy (SM Melmed, J Clin Endocrinol Metab 2001;86:2929–2934; A Mestrón, SM Webb, In: Endocrine Society, San Francisco, June 2002 (abstract)), uncontrolled disease is associated with a growth advantage for concurrent neoplasms, which are more likely to be aggressive; however, there is no clear evidence of de novo cancer...
Isolated familial somatotropinomas (IFS) rarely occurs in the absence of multiple endocrine neoplasia type I (MEN1) or the Carney complex. In the present study we report two Italian siblings affected by GH-secreting adenomas. There was no history of parental consanguinity. The sister presented at 18 years of age with secondary amenorrhea and acromegalic features and one of her two brothers presented...
Surgical excision of an ACTH-producing pituitary tumor is the optimal therapy for Cushing's disease. However, medical therapy may have either a primary or adjunctive role if the patient cannot safely undergo surgery, if surgery fails, or if the tumor recurs. When medication is the only therapy, a major disadvantage is the need for lifelong therapy; in general, recurrence follows discontinuation of...
Disorders of body fluids, notably central diabetes insipidus (CDI) and the syndrome of inappropriate antidiuretic hormone secretion (SIADH), are relatively uncommon as a presenting symptom of sellar and suprasellar masses, but quite common following surgical resection of such lesions. It therefore behooves clinicians treating such patients to have a good understanding of the pathophysiology, the differential...
The morphology of the various pituitary cell types is highly dynamic and allows recognition of many cellular functions. Most pituitary cells show morphologic changes that reflect stimulation or inhibition by hormones. Drugs have also been shown to alter the morphology of several pituitary tumor types, allowing a measure of therapeutic efficiency and a careful dissection of the mechanisms of action...
The primary treatment of acromegaly remains transsphenoidal adenomectomy, yet the tissue overgrowth of acromegaly often progresses following surgery, and responds to radiotherapy only after significant delay. Persistently elevated serum growth hormone (GH) and insulin-like growth factor-I (IGF-I) concentrations can be normalized in about half of post-surgery acromegalics using the pharmacologic alternatives...
The development of efficacious surgical and medical therapies for pituitary adenomas as well as the improvement of hormone therapy for ovulation induction has made pregnancy possible for women harboring pituitary tumors. However, gestational risks due to the possibility of tumor growth during pregnancy, mainly in women with macroadenomas, raise a concern. Bromocriptine has a well-established role...
Thyrotropin-secreting pituitary tumors (TSH-omas) are a rare cause of hyperthyroidism and account for less than 1% of all pituitary adenomas. It is however noteworthy that the number of reported cases tripled in the last years as a consequence of the routine use of ultrasensitive immunometric assays for measuring TSH levels. Contrary to previous RIAs, ultrasensitive TSH assays allow a clear distinction...
Hypopituitarism is a disease complex with variable clinical manifestations. Recent studies have improved our understanding of its pathophysiology, particularly in patients with pituitary adenomas. In that setting, hypopituitarism was previously considered a permanent and irreversible process, requiring life-long hormone replacement therapy. While this could be true in some instances, recent data demonstrated...
Clinically nonfunctioning pituitary adenomas are one of the most common types of pituitary tumors. Unless they present with symptoms related to local mass effect, most tumors are detected incidentally when imaging studies are performed for other reasons. Although clinically nonfunctioning, most of these tumors have evidence, in vitro, of gonadotropin hormone or glycoprotein subunit production. The...
Prolactinomas are a common cause of reproductive/sexual dysfunction. Once other causes of hyperprolactinemia have been excluded with a careful history and physical examination, routine chemistries, a pregnancy test and a TSH, imaging with MRI or CT will delineate the size and extent of the tumor. Medical therapy is the initial treatment of choice. When infertility is the primary indication for treatment,...
Stereotactic radiosurgery is being used with increased frequency in the treatment of residual or recurrent pituitary adenomas. The major risk associated with radiosurgical treatment of residual or recurrent pituitary tumor adjacent to normal functional pituitary gland is radiation of the pituitary, which frequently leads to the development of hypopituitarism. The authors describe a technique of pituitary...
Most patients with acromegaly have some degree of insulin resistance. The principal mediator of insulin resistance in acromegaly is hypersecretion of growth hormone. Growth hormone acts at several levels to block insulin actions including inhibiting phosphorylation of the insulin receptor and one of its principal signaling molecules IRS-1 in response to insulin administration. This leads to reduced...
Pituitary tuberculomas are extremely rare lesions, with only few cases described in the literature, usually mistaken as pituitary tumors. Its heterogeneous clinical and imaging profile preclude preoperative diagnosis which ultimately relies on the histopathological examination. We describe a 46 years old woman who presented with an episode of confusion and hypopituitarism with no evidence of systemic...
Combined pituitary hormone deficiency (CPHD) can be caused by mutation of the pituitary transcription factors POU1F1 or PROP1. More recently mutations in the HESX1, the LHX3 and LHX4 transcription factor genes have also been described as a cause in patients with CPHD. In most patients the disorder is characterized by an impaired production of GH, TSH, PRL and gonadotropins. In some cases of CPHD adrenocorticotropin...
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