https://www.thelancet.com/pdfs/journals/lancet/PIIS0140-6736(14)61375-1.pdf

Cushing's syndrome

Objective: The aim was to formulate practice guidelines for the diagnosis and treatment of hyperprolactinemia. Participants: The Task Force consisted of Endocrine Society-appointed experts, a methodologist, and a medical writer. Evidence: This evidence-based guideline was developed using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system to describe both the strength of recommendations and the quality of evidence. Consensus Process: One group meeting, several conference calls, and e-mail communications enabled consensus. Committees and members of The Endocrine Society, The European Society of Endocrinology, and The Pituitary Society reviewed and commented on preliminary drafts of these guidelines. Conclusions: Practice guidelines are presented for diagnosis and treatment of patients with elevated prolactin levels. These include evidence-based approaches to assessing the cause of hyperprolactinemia, treating drug-induced hyperprolactinemia, and managing prolactinomas in nonpregnant and pregnant subjects. Indications and side effects of therapeutic agents for treating prolactinomas are also presented. (J Clin Endocrinol Metab 96: 273–288, 2011)

Diagnosis and treatment of hyperprolactinemia: an Endocrine Society clinical practice guideline

Mood disorders and allostatic load.

Objective: The aim was to provide guidelines for evaluation, treatment, and genetic testing for multiple endocrine neoplasia type 1 (MEN1). Participants: The group, which comprised 10 experts, including physicians, surgeons, and geneticists from international centers, received no corporate funding or remuneration. Process: Guidelines were developed by reviews of peer-reviewed publications; a draft was prepared, reviewed, and rigorously revised at several stages; and agreed-upon revisions were incorporated. Conclusions: MEN1 is an autosomal dominant disorder that is due to mutations in the tumor suppressor gene MEN1, which encodes a 610-amino acid protein, menin. Thus, the finding of MEN1 in a patient has important implications for family members because first-degree relatives have a 50% risk of developing the disease and can often be identified by MEN1 mutational analysis. MEN1 is characterized by the occurrence of parathyroid, pancreatic islet, and anterior pituitary tumors. Some patients may also develo...

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Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1)

OBJECTIVE:The primary objectives of this report were, first, to determine the number and incidence of complications of transsphenoidal surgery performed by a cross-section of neurosurgeons in the United States and, second, to ascertain the influence of the surgeon's experience with the procedure on

Complications of Transsphenoidal Surgery: Results of a National Survey, Review of the Literature, and Personal Experience

To assess the long-term prognosis for women with prolactinoma after selective transsphenoidal adenomectomy, we followed 44 patients for 62±15 years Group 1 (28 patients) had microprolactinomas, and Group 2 (16 patients) had macroprolactinomas After surgery, normal plasma prolactin levels, resumption of menses, and cessation of galactorrhea were observed in 24 Group 1 patients (85 per cent) and 5 Group 2 patients (31 per cent) Hyperprolactinemia recurred in 12 of the 24 Group 1 patients and in 4 of the 5 Group 2 patients after 4±13 and 25±16 years of remission, respectively There was no radiologic evidence of tumor recurrence in any patient, and no relation was found between the occurrence of pregnancy after surgery and the recurrence of hyperprolactinemia Clinical and biologic features before surgery could not predict the long-term outcome However, the immediate postoperative level of plasma prolactin was significantly lower in patients in whom normal prolactinemia (64±11 ng per mill

Recurrence of hyperprolactinemia after selective transsphenoidal adenomectomy in women with prolactinoma.

From 1962 to 1987, 126 patients underwent trans-sphenoidal surgery for primary treatment of pituitary adenomas unassociated with clinical or biochemical evidence of hormonal overproduction. There were 73 male and 53 female patients (mean age, 50 +/- 12 years). Before surgery, 56% of the patients (70 of 124) had headaches, 74% (94 of 126) had deterioration of vision, and 12% (15 of 126) had ophthalmoplegia. Endocrine evaluation revealed the presence of hypogonadism in 75% (87 of 115), adrenal insufficiency in 36% (46 of 126), and hypothyroidism in 18% (21 of 122). Plasma prolactin was increased in 65% (56 of 86) with a mean level of 39 +/- 14 micrograms/l (normal, 3 to 20 micrograms/l). Radiologic enlargement of the sella turcica was documented in all cases: 67% (84 of 126) had enclosed and 33% (42 of 126) had invasive adenomas. After surgery, vision was normalized or improved in 75% (71 of 94) of the patients. Thyroid, adrenal, and gonadal functions were improved in 14% (three of 22), 41% (19 of 46), 11% (ten of 87), were unchanged in 82% (100 of 122), 77% (97 of 126), 89% (102 of 115), and worsened in 15% (19 of 22), 8% (ten of 126), 3% (102 of 115), respectively. Permanent diabetes insipidus occurred in 5% (seven of 126). Two patients died during the immediate postoperative period. The recurrence rate in patients with a mean follow-up of 6.4 +/- 4.2 years was 21% (15 of 71). These data indicate that trans-sphenoidal microsurgery is an effective and safe initial treatment for patients with nonsecreting pituitary adenoma and may reverse hypopituitarism.

The clinical and endocrine outcome to trans-sphenoidal microsurgery of nonsecreting pituitary adenomas

Chronic exposure to elevated glucocorticoid levels in Cushing's syndrome (CS), is associated with deficits in cognitive function and in emotion. The hippocampus plays a crucial role in the behavioral manifestations of the syndrome as it is richest in glucocorticoid receptors and is thus particularly vulnerable to glucocorticoid excess. The wide distribution of glucocorticoid receptors throughout the cerebral cortex, however, suggests that several cognitive functions can also be affected by the dysregulation of glucocorticoids. In this study, we investigated how an excess of glucocorticoid hormones affects cognitive processes. Nineteen patients with chronic hypercortisolemia due to CS were compared to healthy controls matched for age, sex, education, and occupation in tests of processing of visual and spatial information, memory, reasoning and concept formation, language and verbal functions, and attention. Multivariate and univariate analyses revealed overall differences in tests of treatment of visual and spatial information, reasoning and concept formation as well as in verbal and language performance, with poorer performance from CS patients. Differences were also observed in nonverbal aspects of memory and in attention tasks. The results suggest that chronic exposure to elevated levels of cortisol is associated with deficits in several areas of cognition, particularly those involving processing of selective attention and visual components. This study also shows that hormones play an important role in the modulation of cognitive function and that their influence on cerebral structure and function merits closer scrutiny. (JINS, 2000, 6, 20–29.)

Cognitive decline in patients with Cushing's syndrome.

Over 15 yr, 24 patients underwent transsphenoidal pituitary surgery for Cushing's disease with a median followup of 12 months. Cures included 7 patients with normal sella turcicas (6 microadenomas), 6 patients with focal depressions (6 microadenomas) of the sella (grade I), and 3 patients (3 adenomas) with enlarged sellas (grade II). Three patients with sella destruction (grades III and IV), 2 with normal sellas, and 1 with focal sella depression (grade I) were not cured. Two apparent cures (microadenomas) recurred. Tumor histology revealed 19 basophilic adenomas; electron microscopy (14 tumors) and immunochemical studies (10 tumors) revealed only ACTH cells. Circadian rhythm returned in 6 cured patients. Impotence (in 2), amenorrhea (in 7), and galactorrhea (in 3) resolved in affected cured patients. The major surgical complication was hemorrhage at the operative site (3 patients). Transsphenoidal pituitary surgery is a valuable method for managing Cushing's disease in many patients.

M. Somma

Papers

Recurrence of hyperprolactinemia after selective transsphenoidal adenomectomy in women with prolactinoma.

The clinical and endocrine outcome to trans-sphenoidal microsurgery of nonsecreting pituitary adenomas

Cognitive decline in patients with Cushing's syndrome.

Cushing' Disease: Management by Transsphenoidal Pituitary Microsurgery*

Prolactin secretion in sixty-five patients with galactorrhea.