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BACKGROUND Perioperative steroid protocols for patients undergoing transsphenoidal surgery (TSS) for pituitary pathology vary by institution. OBJECTIVE To assess the safety of withholding glucocorticoids in patients undergoing TSS. METHODS Patients with an intact hypothalamic-pituitary-adrenal (HPA) axis undergoing TSS for a pituitary tumor at the same academic institution between 2012 and 2015 were randomized to either receive 100 mg of intravenous hydrocortisone followed by 0.5 mg of intravenous dexamethasone every 6 h for 4 doses (STER, n = 23) or to undergo surgery without steroids (NOSTER, n = 20). Postoperative cortisol levels were then used to determine the need for glucocorticoids after surgery. Data regarding postoperative cortisol levels, hospital stay length, and complications were collected. RESULTS Mean postoperative 8 am cortisol levels were higher in the NOSTER group compared to the STER group (745 ± 359 nmol/L and 386 ± 193 nmol/L, respectively, P = .001) and more patients were discharged on glucocorticoids in the STER group (42% vs 12%, P = .07). There was no difference in the incidence of postoperative complications, including hyperglycemia, diabetes insipidus, or permanent adrenal insufficiency. Permanent adrenal insufficiency occurred in 8% of patients. CONCLUSION Perioperative steroids can be safely withheld in patients with an intact HPA axis undergoing TSS. Although administration of perioperative glucocorticoids does not appear to increase the risk of complications, it may interfere with assessment of the HPA axis after surgery.

Introduction Prior reports of Langerhans cell histiocytosis (LCH) suggest that isolated skin involvement is rare and often progresses to systemic disease. More rapid access to pediatric subspecialty care has likely led to more frequent representation of this condition. The purpose of this study is to characterize the natural history of skin-limited LCH in an era of increased access to pediatric subspecialty care. Materials and methods A retrospective chart review was performed on all patients newly diagnosed with LCH between 2001 and 2012 at the Children's Hospital of Wisconsin. Extensive review of laboratory, physical examination, and imaging reports was performed and data collected for patients with biopsy-proven skin LCH. Results Sixteen individuals with skin-limited LCH were identified. The median age at onset of skin eruption was birth (range, birth to 6 mo), and median duration of follow-up was 19.5 months (range, 2 wk to 10 y) from diagnosis. One patient (6%) developed pituitary disease and 1 patient (6%) had refractory skin involvement. All others experienced complete resolution. For patients without progressive or refractory disease, resolution of skin findings occurred within 7 months from onset. Discussion Progression of skin-limited to multisystem LCH likely may be less frequent than previously described.

Hypothalamic-pituitary (HP) sarcoidosis has 2 main endocrine manifestations: diabetes insipidus and hyperprolactinemia. We conducted the current study to investigate pituitary dysfunction and perform imaging of the HP area in patients both immediately following diagnosis and after treatment. The study included 6 men and 3 women, with a mean age of 30 years at the onset of sarcoidosis. All patients had both hormonal and magnetic resonance imaging (MRI) HP disorders. All patients had anterior pituitary dysfunction, 7 of them with associated diabetes insipidus. Nine patients had gonadotropin deficiency and 3 had hyperprolactinemia. MRI revealed infundibulum involvement in 5 patients, pituitary stalk thickness abnormality in 5, and involvement of the pituitary gland in 2, associated with other parenchymal brain or spinal cord lesions in 6 patients. All patients had multiple localizations of sarcoidosis, and 5 had histologically confirmed sinonasal localizations. Mean follow-up of the HP disorder was 7.5 years. All patients received prednisone. There was no correlation between the number of hormonal dysfunctions and the area of the HP axis involved as assessed by MRI. Although corticoid treatment was associated with a reduction of radiologic lesions, only 2 patients had partial recovery of hormonal deficiency. In conclusion, hormonal deficiencies associated with HP sarcoidosis frequently include hypogonadism (all patients) and to a lesser degree diabetes insipidus (7 of 9 patients). MRI abnormalities improved or disappeared in 7 cases under corticosteroid treatment, but most endocrine defects were irreversible despite regression of the granulomatous process. Most cases presented with multivisceral localizations and an abnormally high proportion of sinonasal localizations.

Imaging often plays an important role in the management of patients who have been surgically treated for pituitary disease. The primary goals of the radiologist are to recognize normal postoperative findings, to distinguish iatrogenic changes in the sella and surrounding regions from residual tumor, and to detect surgical or therapy-related complications when apparent on imaging. Accurate assessment of the postoperative pituitary region requires an understanding of the normal sellar anatomy and of the common neurosurgical techniques applied to this region and familiarity with the appearance of this region in both the acute postoperative setting and also upon subsequent subacute and later examinations.

Patients with hypopituitarism develop a phenotype similar to metabolic syndrome with central obesity and diabetes. Similarly, patients with hypothalamic damage may develop central obesity, insulin resistance, and hyperphagia. We sought to examine the clinical associations between hypopituitarism, hypothalamic dysfunction, and nonalcoholic fatty liver disease (NAFLD). A case series of patients seen at our institution with diagnoses of hypopituitarism, hypothalamic obesity, or craniopharyngioma and NAFLD was undertaken. Clinical, laboratory, and liver biopsy features were reviewed. Twenty-one patients were identified. NAFLD was diagnosed 6.4 +/- 7.5 years (median 3 years) after the diagnosis of hypothalamic/pituitary dysfunction. Mean gain in body mass index (BMI) between diagnoses of hypothalamic/pituitary disease and NAFLD was 11.3 +/- 8.9 kg/m(2) at an average yearly rate of 2.2 +/- 2.2 kg/m(2). The majority of patients developed elevated glucose levels and dyslipidemia by time of diagnosis of NAFLD. Of the 10 patients biopsied, six were cirrhotic, two had nonalcoholic steatohepatitis (NASH) with fibrosis, and two had simple steatosis. Long-term follow-up of 66 +/- 33 months (range 12-120) was available for 18 patients. Two required liver transplantation. Six patients died, two from liver related causes. In conclusion, patients with hypothalamic and/or pituitary disease are at risk of excessive weight gain, impaired glucose tolerance, and dyslipidemia with subsequent development of NAFLD. This group has a high prevalence of cirrhosis placing them at risk for liver-related death. The novel evidence that hypothalamic/pituitary dysfunction may be accompanied by progressive NAFLD has important implications for the work-up and management of patients with hypothalamic/pituitary disease.

The laparoscopic approach to the adrenal gland was first reported in 1992. Since then, more than 400 studies have been published, comprising hundreds of adults. There are few reports of laparoscopic adrenalectomy in the pediatric population. We reviewed our experience with laparoscopic adrenal surgery in children.Laparoscopic adrenalectomy was performed in 8 girls and 5 boys 15 years or younger (mean age 6.3) between December 1994 and August 2001. The clinical diagnosis before surgery was virilizing tumor in 5 cases, nonfunctioning solid adrenal tumor in 3, Cushing's syndrome in 2, pheochromocytoma in 2 and Cushing's pituitary disease in 1. Unilateral adrenal lesions were 15 to 80 mm. at the longest axis (mean 41.4) on computerized tomography.Two of the 13 procedures (15.4%) were converted to open surgery. No deaths occurred. No patients presented with postoperative complications. Average operative time in unilateral nonconverted procedures was 107 minutes (range 25 to 195). Blood transfusion was required in 1 case (7.7%). Average hospital stay was 5.5 days (range 1 to 17). Average postoperative followup was 16 months (range 1 to 60). The final clinicopathological diagnosis was virilization in 4 cases, Cushing's syndrome in 2, pheochromocytoma in 2, neuroblastoma in 2, Cushing's pituitary disease in 1, teratoma in 1 and primary carcinoma in 1.Laparoscopic adrenalectomy is feasible and shows positive results in select pediatric patients.

Aim: The aim is to describe a very rare case of idiopathic granulomatous hypophysitis presenting as panhypopituitarism on the background of chronic renal failure and type 2 diabetes mellitus. The patient presented with fatigue and episodes of self-terminated hypoglycemia (while on sulfonylurea) as the sole manifestation of panhypopituitarism. The case also highlights the difficulties of identifying patients with hypophysitis in the setting of chronic renal failure. Results: We describe a middle-aged man who presented to us with the sole complaints of early morning fatigue and hypoglycemia (while on glimepiride), with a background history of type 2 diabetes and chronic renal failure. Before visiting our practice, he was repeatedly evaluated for decompensation of renal and cardiovascular disease without much success. When we saw the patient there was no obvious evidence of any endocrine disturbance, especially as the blood pressure was high without any postural drop. It was only when the patient persistently complained of an impaired quality of life due to the fatigue that we decided to chase an endocrine cause for the same. On endocrine evaluation, we diagnosed panhypopituitarism secondary to idiopathic granulomatous hypophysitis. Conclusion: Idiopathic granulomatous hypophysitis is a very rare form of inflammatory pituitary disease. Fewer than 35 cases of biopsy-proven idiopathic granulomatous hypophysitis have been reported in medical literature. This is only the third case of biopsy-proven idiopathic granulomatous hypophysitis reported from India.

Pituitary dysfunction is a rare manifestation of granulomatosis with polyangiitis (GPA) (Wegener). The main aim of this multicenter retrospective study was to describe the characteristics and outcomes of pituitary manifestations in patients with GPA included in the French Vasculitis Study Group database. Among the 819 GPA patients included in the database, 9 (1.1%) had pituitary involvement. The median age at diagnosis of GPA and pituitary involvement was 46 and 50.8 years, respectively. Pituitary involvement was present at onset of GPA in 1 case and occurred later in 8 patients after a median follow up of 58.5 months. When pituitary dysfunction occurred, 8 patients had active disease at other sites including ENT (n = 6), eye (n = 4), or central nervous system (n = 3) involvement. The most common hormonal dysfunctions were diabetes insipidus (n = 7) and hypogonadism (n = 7). Magnetic resonance imaging was abnormal in 7 patients. The most common lesions were an enlargement of the pituitary gland, thickening of the pituitary stalk, and loss of posterior hypersignal on T1-weighed images. All patients were treated with corticosteroid therapy and 8 patients received immunosuppressive agents for the pituitary involvement, including cyclophosphamide (n = 3), rituximab (n = 2), and methotrexate (n = 3). After a median follow-up of 9.2 years, GPA was in complete remission in 7 patients, but 8 patients were still under hormone replacement therapy. Among the 5 patients who had a subsequent MRI, 2 had complete resolution of pituitary lesions.By combining our study and the literature review, the frequency of hypogonadism and diabetes insipidus, among the patients with pituitary dysfunction, can be estimated at 78% and 71% respectively. Despite a high rate of systemic disease remission on maintenance therapy, 86% of the patients had persistent pituitary dysfunction. The patients who recovered from pituitary dysfunction had all been treated by cyclophosphamide. Pituitary disease in GPA occurs mostly several months or years after diagnosis. There is no correlation between hormonal, radiologic, and systemic outcome. Although immunosuppressive drugs improve the systemic disease, hormonal deficiencies usually persist. It is therefore important to shorten diagnostic delays and treat these patients early in the course of disease before irreversible damage occur.

To the Editor: We read with interest the article by McLaughlin and colleagues1 about Pituitary Centers of Excellence (PCOEs), along with the outstanding comments published in the same issue of the journal. We agree with the authors that PCOE may be defined as a cohesive team of specialists (neurosurgeons, endocrinologists, otorynolaryngologists, neuro-ophthalmologists, neuroradiologists, neuropathologists, radiation oncologists) who promote collaboration and apply best practices to improve results and overall outcomes in patients with pituitary tumors and related disorders. Again, in accordance with McLaughlin and colleagues, the 3 primary missions to be fulfilled by the centers to be recognized as a PCOE are the following: providing multidisciplinary, optimal clinical care to patients with pituitary tumors and related disorders; providing residency, fellowship training, and/or continuing medical education and patient support; and contributing to research in the field of pituitary disorders.

Isolated adrenocorticotropin (ACTH) deficiency is a rare cause of secondary adrenocortical insufficiency. This review summarizes the clinical and laboratory features of 39 previously reported cases plus 4 new patients. The clinical manifestations of isolated ACTH deficiency are variable, nonspecific and similar to those seen in adrenocortical insufficiency of any cause. The diagnosis of isolated ACTH deficiency due to intrinsic pituitary disease is made unequivocally when all the following criteria are met: 1) low basal urinary 17-hydroxycorticosteroid (17-OHCS) levels with or without low basal plasma cortisol, 2) low or normal basal plasma ACTH, 3) stimulation of cortisol, 17-OHCS or both during prolonged ACTH administration, 4) lack of 17-OHCS elevation in response to metyrapone and 5) normal secretory indices of other pituitary hormones. Isolated ACTH deficiency secondary to suprapituitary (e.g., hypothalamic) dysfunction is also based upon the above criteria, but, in addition, is associated with stimulation of cortisol and ACTH secretion following vasopressin administration.

Elevated levels of serum prolactin in patients with pituitary tumors have been associated with hypogonadism, frequently manifested in men as impotence and/or loss of libido. The purpose of our study was to test the hypothesis that psychogenic and/or organic impotence may be a manifestation of a relative hyperprolactinemic state. We studied 30 men with the known diagnosis of impotence: 17 organic and 13 psychogenic. There were 11 normal, sexually active men used as controls. Serum prolactins were determined with the radioimmunoassay technique. There were no significant differences in serum prolactin levels among control, organic impotent and psychogenic impotent groups. Impotence does not seem to be related to hyperprolactinemia in men in the absence of pituitary disease.