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Lymphocytic adenohypophysitis: report of two cases.

Two women with lymphocytic adenohypophysitis not related to pregnancy are reported on. In obtaining a differential diagnosis of lymphocytic adenohypophysitis preoperatively, it is useful to note swelling of the anterior hypophyseal tissue and the existence of a posterior pituitary lobe on sagittal sections of magnetic resonance imaging scans. Hypofunction of the anterior hypophyseal gland is more severe in lymphocytic adenohypophysitis than in pituitary adenomas. Marked adhesion of fibrotic pituitary capsule to the dura is a characteristic observation during surgery for lymphocytic adenohypophysitis.

Adenoma↗

Premature mortality due to cardiovascular disease in hypopituitarism.

333 consecutive patients with hypopituitarism diagnosed between 1956 and 1987 were retrospectively examined. The patients had been given routine replacement therapy. The overall mortality was higher than in an age and sex matched population. Deaths from vascular disorders were also significantly increased (60 [40 male, 20 female] versus 30.8 expected [23.5, 7.4 female]). The hazard function for vascular death was independent of age at diagnosis, time after diagnosis, calendar year of diagnosis, gender, degree of pituitary insufficiency, hypertension, and diabetes mellitus. Mortality risk was raised irrespective of whether hypopituitarism was due to pituitary adenoma or secondary to other diseases. 7 patients (3 male, 4 female) died from malignant diseases (expected 10.1 and 4.1, respectively). These observations indicate that life expectancy is shortened in patients with hypopituitarism. Growth-hormone deficiency could be a factor in this increased mortality from cardiovascular disease.

Adenocarcinoma↗

[Hypophyseal adenomas: functional and tumor evaluation and therapeutic approaches].

Patients with pituitary adenomas present with hypersecretion syndrome(s), and/or pituitary failure(s), and/or signs of mass effect, or incidentally. Pituitary function evaluation, visual acuity and field check-up, and MRI or at least CAT are compulsory for diagnosis, and for therapeutic approach; surgery for Cushing's disease, dopamine agonists for prolactinomas, somatostatin analogs or surgery for thyrotroph adenomas, surgery and/or somatostatin analogs and/or radiotherapy in acromegaly, surgery with additional irradiation in most adenomas of other types, or even expectation in some instances.

Adenoma↗

Antagonism of oestrogen-induced prolactin release by medroxyprogesterone acetate.

Previous studies conducted at our clinic suggested that the administration of hormone replacement therapy (HRT) in postmenopausal women could result in the inhibition of oestrogen-induced prolactin (PRL) release. The aim of this study was to determine how the pituitary function is affected by the sequential addition of medroxyprogesterone acetate (MPA) to oestrogen replacement therapy. Twenty-one postmenopausal women receiving no other medication were treated with a standard dose (0.625 mg/day) of conjugated equine oestrogens (CEE) for a period of 24 days, plus 5 mg/day MPA added sequentially during the last 12 days of the oestrogen therapy. Blood samples were collected before treatment, during oestrogen and oestrogen-progestogen administration and after cessation of treatment. Follicle-stimulating hormone (FSH), luteinizing hormone (LH), 17 beta-oestradiol (E2) and PRL levels were studied. During treatment gonadotrophin concentrations decreased significantly, while after cessation of HRT the levels of FSH and LH increased. These gonadotrophin fluctuations indicated a sharp rise in E2 levels during therapy and a significant decrease during the treatment-free period. PRL levels were found to be higher during CEE therapy, but they fell when patients received CEE in combination with MPA. These observations suggest that the role of progestogens in a variety of experimental and clinically relevant situations needs to be investigated not only as regards their direct action but also their modulation of the effect of oestrogen.

Climacteric↗

The long-term effect of recombinant methionyl human leptin therapy on hyperandrogenism and menstrual function in female and pituitary function in male and female hypoleptinemic lipodystrophic patients.

Lipodystrophy patients are hypoleptinemic and insulin resistant. Women have enlarged polycystic ovaries, hyperandrogenism, and amenorrhea. We have determined the role of correction of hypoleptinemia on these metabolic and neuroendocrine parameters. Ten females and 4 males with generalized lipodystrophy were treated with recombinant methionyl human leptin (r-metHuLeptin) in physiologic doses in an open-labeled study for a period of 12 and 8 months, respectively. In the female group, serum free testosterone decreased from 39.6 +/- 11 to 18.9 +/- 4.5 ng/dL (P < 0.01) and serum sex hormone binding globulin increased from 14 +/- 2.5 to 25 +/- 4.8 nmol/L (P < 0.02). Luteinizing hormone (LH) responses to LH releasing hormone were more robust after therapy and significantly changed in the youngest group of 3 female patients (P < 0.01). Ovarian ultrasound showed a polycystic ovarian disease pattern in all patients and did not change after therapy. Eight of the 10 patients had amenorrhea prior to therapy and all 8 developed normal menses after therapy. In the male group, serum testosterone tended to increase from 433 +/- 110 to 725 +/- 184 ng/dL (P = 0.1) and sex hormone binding globulin also increased from 18.25 +/- 2.6 to 27 +/- 1.7 nmol/L (P < 0.04) following r-metHuLeptin therapy. Serum LH response to LH releasing hormone did not show significant changes. Five additional hypoleptinemic male subjects with minimal metabolic abnormalities underwent normal pubertal development without receiving r-metHuLeptin therapy. In both genders, insulin-like growth factor increased significantly and there were no differences in growth hormone, thyroid, or adrenal hormone levels following r-metHuLeptin therapy. Glycemic parameters significantly improved after r-metHuLeptin therapy in both groups. Hypoglycemic medications were discontinued in 7 of 12 patients and dramatically reduced in 5 patients. r-metHuLeptin therapy plays an important role in insulin sensitivity. In females, it plays an additional role in normalizing menstrual function. This is likely to occur both from increasing insulin sensitivity and from restoring LH pulsatility. The persistent hypoleptinemic state in these subjects did not inhibit pubertal development.

Adolescent↗

Bilateral optic disk melanocytoma in a 10-month-old infant.

PURPOSE: To report bilateral optic disk melanocytoma in a healthy infant, METHOD: Case report. RESULTS: A 10-month-old healthy boy presented with intermittent right exotropia. Dilated fundus examination showed a heavily pigmented mass within both optic disks. The optic disks were hypoplastic, and there was diffuse retinal pigment epithelial mottling around the optic disks. Systemic evaluation including thyroid function tests, pituitary gland hormone levels, adrenal gland hormone levels, and magnetic resonance imaging of the brain revealed normal findings. After 1 year, the lesions remained stable, and the patient was healthy with normal growth and development. CONCLUSION: Bilateral optic disk melanocytoma is uncommon. Although this patient had no systemic problem, a literature review revealed an association of bilateral optic disk melanocytoma, optic disk hypoplasia, and central nervous system abnormalities such as meningioma and hypopituitarism. Systemic evaluation for central nervous system abnormalities is warranted in such cases.

Humans↗

Current concepts of prolacting physiology in normal and abnormal conditions.

Currently the physiology and pathophysiology of pituitary prolactin secretion are under intensive investigation. Development of sensitive, specific radioimmunoassays for hPRL and improved roentgenographic techniques have increased the diagnostic acumen for incipient pituitary microadenomas. Several modalities of treatment are available at the present time which can result in improvement in the clinical symptoms of the amenorrheagalactorrhea syndromes. Eponymic classification of amenorrhea-galactorrhea syndromes should be discarded and appropriate diagnostic studies initiated to determine the etiology of the inappropriate breast secretion and/or elevated serum hPRL level.

Adenoma↗

Infundibuloneurohypophysitis presenting a large sellar-juxtasellar mass: case report.

BACKGROUND: Infundibuloneurohypophysitis (INH) is reported to be a self-limiting inflammatory disease involving neurohypophysis. The authors experienced a case of INH presenting a large mass compressing the brain stem. CASE PRESENTATION: The patient exhibited polyuria followed by left hemiparesis and dysarthria lasting a year. Magnetic resonance imaging showed a large sellar mass extending into the right cavernous sinus and prepontine cistern and compressing pons. Endocrinologically, diabetes insipidus was diagnosed and anterior pituitary function was almost normal. Microscopic examination of the surgical specimen obtained by a transsphenoidal route demonstrated diffuse infiltration of lymphoid cells with predominance of B cells over T cells and the granulation tissue. The patient underwent 40 Gy local radiation because of initial misinterpretation of histologic findings as malignant lymphoma and short-term corticostertoid administration. CONCLUSION: The mass gradually shrank and the patient has become neurologically intact in 6 months. At this moment, 67 months after the onset, the patient is free from disease and has no other lesion. INH seems to be a clinical entity possessing a wide spectrum from infundibular tumorlet to an aggressive sellar mass trespassing on surrounding structures.

Biopsy↗

Mucocele-like formation leading to neurological symptoms in prolactin-secreting pituitary adenomas under dopamine agonist therapy.

BACKGROUND: Mucocele-like formation associated with pituitary adenomas, to the best of our knowledge, has been paid little attention. We report three adult male patients with a mucocele-like formation that developed behind the tumor and led to neurological symptoms in prolactin-secreting pituitary adenomas (prolactinomas) under dopamine agonist therapy. CLINICAL PRESENTATION: Three adult male patients with prolactinomas developed hyperprolactinemia and new neurological symptoms during dopamine agonist treatment. In each case, the pathogenesis of these symptoms was due in part to a mass enlargement with development of a mucocele-like formation behind a prolactinoma. In our patients, a prolactinoma with a suprasellar extension originally filled the sphenoid sinus. When dopamine agonist therapy became ineffective, new symptoms, such as progressive visual impairment other than typical hemianopsia or headache, developed and mass enlargement was found on MRI. MRI demonstrated two different components: an enhancing prolactinoma and a nonenhancing mucocele-like formation behind the tumor. Two patients had compression of the optic nerves by a mass. Transnasal removal of mucoceles and adenomas led to resolution of the neurological symptoms. CONCLUSION: Early suspicion of a mucocele-like formation under dopamine agonist therapy for prolactinomas is important in order to avoid a delay in surgery, because a change in medical treatment will be ineffective.

Aged↗