Gregory Kaltsas

COMPARISONS IN THE EPIDEMIOLOGY, DIAGNOSTIC FEATURES AND CURE RATE BY TRANSSPHENOIDAL SURGERY BETWEEN PAEDIATRIC AND ADULT-ONSET CUSHING'S DISEASE

OBJECTIVE There are few published comparisons between paediatric and adult-onset Cushings disease (CD). We compare the epidemiology, diagnostic features and cure rate by transsphenoidal surgery (TSS) in these groups. DESIGN Retrospective review of patient databases in a single university hospital centre. PATIENTS Totally, 41 paediatric (mean age 12.3 ± 3.5 years; range 5.7-17.8) and 183 adult (mean age 40 ± 13 years; range 18.0-95.0) patients with CD were investigated. RESULTS Paediatric CD was characterised by male (63%) and adult CD by a female predominance (79%, P<0.0001). There were small but significant differences in clinical presentation. Biochemical features of CD were comparable except the serum cortisol increase during a CRH test: mean change (105%, n=39) in paediatric and (54%, n=123) in adult subjects (P<0.0001). Macroadenomas were more common in adult (15%, 28/183) than in paediatric (2%, 1/41, P=0.04) CD. Corticotroph microadenomas were more easily visualised by pituitary magnetic resonance imaging (MRI) in adult (76%, 50/66) compared with paediatric (55%, 21/38, P=0.045) CD with poorer concordance of imaging with surgical findings in children (P=0.058). The incidence of ACTH lateralisation by bilateral simultaneous inferior petrosal sinus sampling was comparable in paediatric (76%, 25/33) and adult (79%, 46/58; P=0.95) patients with good surgical concordance in both (82% paediatric and 79% adult). Cure rates by TSS were comparable, with a paediatric cure rate of 69%. CONCLUSION Several features of paediatric CD are distinct: increased frequency of prepubertal CD in males, the different clinical presentation, the decreased presence of macroadenomas and the frequent absence of radiological evidence of an adenoma on MRI.

The effect of growth hormone replacement therapy on adrenal androgen secretion in adult onset hypopituitarism

objective Growth hormone replacement therapy in GH‐deficient children is associated with enhanced adrenal androgen production, raising the possibility that GH might stimulate adrenocortical hormone secretion. This has not been extensively investigated in adults to date. GH is a potent modulator of the activity of the 11β‐hydroxysteroid dehydrogenase type 1 (11β‐HSD1) enzyme and by altering cortisol metabolism can affect the function of the hypothalamo–pituitary–adrenal (HPA) axis and therefore potentially of adrenal androgen secretion. This study examined the effects of GH replacement in GH‐deficient adults on adrenal androgen secretion.

Pancreatic Neuroendocrine Tumors Presenting with Hypercalcitonemia

ABSTRACT Objective: Pancreatic neuroendocrine tumors (pNETs) can be associated with discrete clinical syndromes due to the secretion of a variety of bioactive substances that are used to confirm the diagnosis and as potential tumor markers. Calcitonin (Ct) is mainly secreted by medullary thyroid carcinoma (MTC) but is also rarely secreted by pNETs, occasionally leading to diagnostic confusion. We report the diagnosis of 4 patients with metastatic pNETs and associated Ct secretion who were initially evaluated for thyroid pathology. Methods: We describe the histories of 4 patients who were referred for evaluation of thyroid diseases and proved to have metastatic pNETs and associated Ct secretion. One of the patients had erroneously undergone thyroidectomy as hypercalcitonemia was attributed to MTC. Results: Patients presented with variable Ct levels (4-fold up to 35-fold elevations), and all had thyroid nodules that were considered the cause of hypercalcitonemia. Ultrasonography and fine-needle aspiration c...

Somatostatin Receptor Expression in Gastrointestinal Tumors

Somatostatin (SST) receptors belong to the seven-transmembrane domain G-protein-coupled receptors superfamily and exist in five different subtypes. They are widely distributed in many tissues in the human body where they mediate several physiological actions of the native SST hormone. In addition, they are also overexpressed in many tumor cells especially in pituitary adenomas and neuroendocrine neoplasms of the gastrointestinal system. This fact has constituted the basis for development of highly specific and sensitive imaging modalities for in vivo visualization and selective irradiation of such tumors using radiolabeled SST analogues. However, heterogeneous distribution of the five known SST receptor subtypes between different tumor types, and even within the same tumor, can be challenging in the diagnostic approach and can produce considerable variability in responsiveness to therapies targeting these receptors. Therefore, detection of SST receptors and subtype profiling using in vivo and in vitro methods is increasingly gaining clinical attention.

Langerhans Cell Disease

Langerhans cell disease or Langerhans cell histiocytosis (LCH) is a rare disease of varied biologic behavior and course, resulting from the pathologic accumulation of cells that resemble the epidermal Langerhans cell. Although sometimes it may resolve spontaneously, it usually follows a chronic course and can either be localized to a few systems or progress to a multisystem disease, with considerable morbidity and mortality.