Incidental finding of a nasopharyngeal mass in an anterior pituitary insufficiency setting

Lara Delcour (1), Marie-Anne Labaisse (2), Carine Hinkeltz (3) Published in the journal : December 2018 Category : Endocrinology, Otorhinolaryngology

The differential diagnosis of nasopharyngeal masses is vast and mainly comprises three categories of lesions: benign masses corresponding to benign tumors and inflammatory lesions, malignant tumors, and congenital lesions. The age of the patient, clinical context, symptoms associated with the mass, as well as iconographic data are essential to orientate professionals towards the various possible etiologies. In a context of hypothalamic-pituitary axis malformation, and with reference to the embryological origins of the pituitary gland, the hypothesis of extracranial ectopic pituitary tissue is, although rare, an etiology that must be evoked when confronted to a nasopharyngeal mass. However, biopsy with an anatomopathological examination proved to be the only technique able to confirm the lesion’s histological origin.

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Schizophrenia and gender dysphoria: Is it only a question of thalamus?

Bernadette Ibrahim(1), Vinciane Corman(1) Published in the journal : June 2018 Category : Endocrinology

Schizophrenia is a mental disease characterized by a personality dislocation. Gender dysphoria refers to the intimate, constant, and irreversible conviction of belonging to the opposite sex versus the origi-nal one. For these two diseases, studies have reported a thalamic damage in two neighboring areas. We may wonder if these two areas of joint damage could be linked and thus explain this sexual identity disor-der in schizophrenic patients, thereby guiding us towards appropriate medical management. In this article, we illustrate the medical care of schizophrenic patients affected by gender dysphoria by considering the representative case of a schizophrenic male patient who is searching for his sexual identity with the feeling of an “atrophic clitoris”, along with an increase in adrenal androgens in labor-atory tests. The medical care for these patients proves complex, as it requires us to come up with the right ques-tions.

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Hyperprolactinemia in daily practice: prolactinomas are not so common

Dominique Maiter Published in the journal : March 2018 Category : Endocrinology

When confronted with patients suffering from moderate hyperprolactinemia (25-100μg/L) in daily practice, multiple causes should be evoked. If the condition is not associated with suggestive symptoms, it is most often caused by other conditions than a prolactinoma. These mainly include transient hyperprolactinemia that is not confirmed by subsequent hormonal testing, macroprolactinemia that must always be systematically ruled out; antidopaminergic or serotoninergic drugs leading to a sustained rise in prolactin concentrations (mainly neuroleptics and antiemetics), and disconnection hyperprolactinemia associated with other lesions of the hypothalamic-pituitary axis disrupting the dopaminergic inhibitory control on basal prolactin secretion. We have herein reviewed the different causes of hyperprolactinemia. Before embarking the patient on a long-term treatment with dopamine agonists, the diagnostic evaluation should always take into consideration these potential causes, in addition to a possible pituitary incidentaloma.

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An update on diagnosis and treatment of adult growth hormone deficiency

Orsalia Alexopoulou, Dominique Maiter Published in the journal : March 2018 Category : Endocrinology

Adult growth hormone deficiency (AGHD) is nowadays recognized as a distinct clinical entity and replacement therapy has become a standard practice. The benefits of GH treatment seem to outweigh its potential risks but issues concerning long term efficacy and safety are still a subject of debate. More research is needed in some key areas and it remains essential to monitor patients by means of longitudinal surveillance studies.

Key Words

Growth Hormone Deficiency, Hypopituitarism, GH deficient adults

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Clinically silent corticotroph tumor revealed by galactorrhea

Caroline Fobe (1), Etienne Marbaix (2), Thierry Gustin (3), Etienne Delgrange (4) Published in the journal : December 2017 Category : Endocrinology

Galactorrhea associated with a sellar mass results in a large differential diagnosis. We discuss the case of a female patient harboring an invasive macroadenoma upon MRI, clinically associated with galactorrhea and mild hyperprolactinemia. The discrepancy between the large tumor size and mild prolactinemia, as well as the rapid normalization of prolactinemia under cabergoline without associated tumor volume reduction were considered arguments pleading against the lactotroph tumor diagnosis. Despite the lack of hypercortisolism-related clinical symptoms, with normal free urinary cortisol levels and normal cortisol suppression under dexamethasone, a silent corticotroph adenoma was suspected preoperatively, owing to the mildly-elevated ACTH levels in contrast with normal cortisol levels.

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Use of synthetic glucocorticoids : undesirable effects in clinical practice

Dominique Maiter Published in the journal : March 2017 Category : Endocrinology

Multiple factors may account for the chronic toxicity of synthetic glucocorticoids (GCs), which are (too) widely prescribed in clinical practice. The relative potency of the GCs used, daily dose administered, duration of treatment, route and time of administration, as well as individual factors like age, genetic factors, or significant co-morbidity, such as renal or hepatic failure, determine the delay in the occurrence of potentially serious complications, including the suppression of the corticotropic axis. The concomitant use of drugs affecting the metabolism and/or the action of these glucocorticoids must also be considered. While the selected treatment strategy should take into account all these factors, it must also consider the desired effects and proofs of efficacy. Whenever possible, short-term treatment or non-systemic administration of glucocorticoids should be preferred. Iatrogenic Cushing's syndrome is characterized by specific complications, including posterior subcapsular cataract, benign intracranial hypertension, osteonecrosis of the femoral and humeral heads, tendon ruptures, pancreatitis or psychotic crisis. Suppression of the hypothalamus-pituitary-adrenal (HPA) axis can be assumed in patients receiving over 16mg of methylprednisolone per day (or equivalent dose) for at least 6 weeks and in those exhibiting clinical Cushing’s syndrome. On the contrary, this condition is rather unlikely in patients receiving nonparenteral corticosteroids for less than 3 weeks. In other situations, adrenal function must be assessed before considering glucocorticoid withdrawal. This comprises the measurement of morning plasma cortisol levels, in addition to a short corticotropin (ACTH) stimulation test. If the results confirm either complete or partial suppression of the HPA axis, treatment with hydrocortisone (20mg/day in the morning) should be administered until recovery of adrenocortical function.

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Intercellular communications in primary adrenal Cushing's syndrome : novel therapeutic targets for the management of hypercortisolism

Hervé Lefebvre Published in the journal : March 2017 Category : Endocrinology

Recent studies have demonstrated that most genetically-determined adrenal lesions responsible for Cushing's syndrome display abnormal cell differentiation resulting in the development of paracrine regulation loops that favor cortisol hypersecretion. In bilateral adrenal macronodular hyperplasia tissues, the causative gene mutations appear to lead to pseudo-gonadal differentiation of a subpopulation of adrenocortical cells, resulting in aberrant ACTH synthesis. In primary pigmented adrenocortical disease (PPNAD) and in some cortisol-secreting adenomas, activation of the protein kinase A (PKA) pathway is directly responsible for upregulation of the serotonergic signaling pathway . These original observations provide new insights into the pathophysiology of primary adrenal Cushing's syndrome. They also suggest that illicit intraadrenal paracrine regulatory mechanisms may be regarded as valuable targets for new pharmacological treatments of hypercortisolism. These original therapeutic approaches could represent valuable alternatives to adrenal surgery and the currently used anticortisolic drugs which are responsible for various side-effects.

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Premature pubarche : When should we be worried about ?

Véronique Beauloye Published in the journal : March 2017 Category : Endocrinology

The occurrence of early pubic hair often causes concern to both families and physicians. Whereas, in the majority of cases, this is only a normal variant called premature adrenarche, this condition may also be related to potentially serious diseases, such as congenital adrenal hyperplasia, adrenal tumors, or gonadal tumors, which must be correctly diagnosed and managed appropriately.

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Assessment of plasma cortisol levels : usefulness and diagnostic pitfalls

Damien Gruson Published in the journal : March 2017 Category : Endocrinology

Assessing cortisol levels is recommended in the event of suspected hypercorticism like Cushing's syndrome, as well as in the event of hypocorticism like adrenal insufficiency. Cortisol testing is therefore a major tool for clinicians, and especially for endocrinologists, with particular attention required for selecting the most appropriate cortisol measurement tool in relation to clinical expectations. Most of the current assays while being automated rely on immunoassays. It should, however, be noted that the standardization process for cortisol assays has not yet been fully completed, with still significant inter-method variations. The clinicobiological relationship thus appears paramount in order to best select the most appropriate cortisol assessment method.

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Diagnosis of primary bilateral macronodular adrenal hyperplasia and evaluation of aberrant receptors. A case report.

Corinne Jonas, Christophe Hommel, Julian Donckier Published in the journal : May 2015 Category : Endocrinology

Primary bilateral macronodular adrenal hyperplasia is a rare condition that may be responsible for Cushing's syndrome. We report herein the case of a patient presenting with bilateral adrenal incidentalomas causing subclinical hypercorticism. Aberrant receptors were searched for, suggesting the presence of vasopressin and serotonin receptors. As there is currently no specific treatment available to block these receptors, it has been proposed to follow-up the patient who did not present any hypercorticism-related complication.

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