Atypical thymic carcinoid in multiple endocrine neoplasia type 1 syndrome.

An asymptomatic, non-smoker patient carrier of a multiple endocrine neoplasia syndrome type 1 (MEN1) mutation was diagnosed with invasive atypical thymic carcinoid tumor. After surgical treatment the tumor reappeared albeit without metastasis. Thymic carcinoid is a well-known cause of mortality in MEN1, and usually metastatic disease is present at diagnosis. Male sex, smoking, and previous hyperparathyroidism probably play a role in the pathogenesis of this neoplasia.

Effect of acute ghrelin administration on glycaemia and insulin levels in obese patients.

OBJECTIVES: Ghrelin is a 28-amino-acid peptide, predominantly produced by the stomach. There are several studies that suggest the importance of ghrelin in obesity. However, the pancreatic endocrine response to ghrelin in obesity is unclear at present. The aim of this study was to clarify whether ghrelin administration influences glucose and insulin levels in obese patients. PATIENTS AND METHODS: Six obese female patients (31 +/- 3.4 year) with a BMI of 36.1 +/- 7.7 kg/m(2) were studied. Three tests were done: placebo, ghrelin (1 microg/kg, intravenously) and growth hormone-releasing hormone (GHRH; 1 microg/kg, iv) plus ghrelin (1 microg/kg, iv). Blood samples were taken at appropriate intervals for determination of glucose and insulin. Statistical analyses were performed by Wilcoxon and Mann-Whitney tests. RESULTS: Glucose (mean peak, mmol/l) level after placebo administration was 4.9 +/- 0.2. Glucose level after the administration of ghrelin was 5.1 +/- 0.2, not significantly different from the response after placebo (p = NS). Glucose level after the administration of ghrelin plus GHRH was 5.1 +/- 0.2, not significantly different from placebo (p = NS). Insulin (mean peak, mU/l) level after placebo administration was 16.1 +/- 6.1. Insulin level after the administration of ghrelin was 12.3 +/- 1.6, not significantly different from placebo (p = NS). Insulin level after the administration of ghrelin plus GHRH was 11.1 +/- 2.7, not significantly different from the response after placebo (p = NS). CONCLUSIONS: In female obese patients, we did not find significant differences in glucose or insulin levels following ghrelin or GHRH plus ghrelin administration.

Acromegaly due to a growth hormone-releasing hormone-secreting intracranial gangliocytoma.

In more than 95% of cases acromegaly is due to GH hypersecretion by a pituitary adenoma. GHRH hypersecretion accounts for about 0.5% of cases of acromegaly. Intracranial GHRH-secreting tumors are extremely rare and only a few well-documented cases have been reported. The clinical features of acromegaly due to intracranial GHRH-secreting tumor are indistinguishable from those of other patients with "classical acromegaly". In cases of intrasellar gangliocytomas, not even radiological findings help to make the correct diagnosis, which can only be made with the hystological study. We present the case of a woman with acromegaly; the magnetic resonance demonstrated a 2x1.8x1.2 cm mass in the jugum sphenoidalis region, associated with a partial empty sella. There was a partial response to high-dose lanreotide therapy, so surgical treatment was decided, although only part of the tumor could be removed. Histopathological diagnosis was consistent with gangliocytoma, and immunostaining in the ganglionic cells was positive for GHRH. After surgery, hormone hypersecretion persisted, so medical treatment was reintroduced. In summary, we report a well-documented case of an intracranial GHRH-secreting gangliocytoma, an exceedingly rare cause of acromegaly. Clinical and biochemical data did not allow to make the correct diagnosis, which was only made on the pathological study. This case underscores that acromegaly can be due to causes other than a GH-secreting adenoma, and underlines that finding an image not typical of a pituitary adenoma should raise the suspicion that an unusual cause subsides the acromegaly.

[Neuroendocrine disturbances in obesity]. / Alteraciones neuroendocrinas en la obesidad.

Obesity is associated with different disturbances in endocrine function. Both spontaneous growth hormone (GH) secretion and its response to several stimuli have shown to be reduced in obese patients. The GH responses to GH-releasing hormone and other challenges by pyridostigmine suggest that the reduction in GH secretion is related to an increased somatostatinergic tone. Other experiments point to a down-regulation of somatostatin receptors in the somatotroph cell. Ghrelin administration is followed by a massive GH release, but the possibility that ghrelin or GHRH deficiency are the cause of GH deficiency in obesity is unlikely. The increase in free fatty acids in obesity might be related to GH reduction, since acipimox administration is able to reverse GH secretion. In women, abdominal obesity is associated with hyperandrogenism and low sex hormone-binding globulin levels. Obese men have low testosterone and gonadotrophin concentrations, specially in cases of morbid obesity. An increase in hypothalamic-pituitary-adrenal axis activity and some resistance to dexamethasone suppression have been described in abdominal obesity. This effect may be due to neuroendocrine alterations related to a genetic origin. Adrenal hyperfunction may favour cardiovascular and metabolic complications. There are no disturbances in thyroid function. Sometimes a reduction in prolactin response to several stimuli has been reported. This effect may be due to hyperinsulinaemia or to disturbances in the dopaminergic tone.

[Central diabetes insipidus secondary to infundibuloneurohypophysitis]. / Diabetes insípida central secundaria a infundibuloneurohipofisitis.

In up to a third of cases, central diabetes insipidus (DIC) is idiopathic although the percentage varies in different series. Since antibodies against magnicellular neurons were detected in some patients, a possible autoimmune basis for certain cases of apparently idiopathic DIC was speculated. Lymphocytic infundibuloneurohypophysitis, an inflammatory process that affects the infundibulum, pituitary stalk and neurohypophysis with distinctive radiologic, histologic and evolutive characteristics, has recently been described as a cause of central diabetes insipidus. We describe a patient in whom the clinical and radiologic characteristics suggest the diagnosis of DIC secondary to infundibuloneurohyphysitis.