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Introduction Craniopharyngiomas are a big challenge in the neurosurgical field. Because these lesions involve important systems, surgeons must weigh the risks of aggressive resection against the long-term challenges of recurrence. We present the outcomes of our patients based on clinical results, degree of resection, recurrence and disease-free survival. Materials and methods We reviewed medical records in all patients who had undergone surgical resection for craniopharyngioma at (Institution’s name) between 2007 and 2019. We considered ophthalmological examinations, imaging studies, endocrinological studies and surgical complications . Radical resections were planned in all of the patients. To help choose the correct surgical approach, craniopharyngiomas were classified based on tumour location. Results Thirty cases of craniopharyngioma were analysed. 12.5% were classified as intrasellar, 12.5% as prechiasmatic, 43.75% as retrochiasmatic, and 31.25% as intraventricular. Overall, 38 cases involved a transcranial surgery (15 orbitozygomatic approach; 19 pterional approach and 4 transcallosal approach), 7 involved a transsphenoidal approach, 2 microscopic transnasal approach and one ventricular endoscopy for emptying the craniopharyngioma cyst. Gross-total resection was achieved in 43.7% and near-total resection (more than 90%) in 25%. The mean follow-up period after resection was 4.7 years. Tumour recurrence occurred in 48%, with an average of 42.7 disease-free months. Conclusion Total tumour resection is the best treatment for craniopharyngioma. Due to its high morbidity and mortality, a multidisciplinary team is necessary for the management of these tumours.

Resumen Introduccion Los craneofaringiomas son un gran desafio en el campo neuroquirurgico. Debido a que estas lesiones involucran estructuras importantes, los cirujanos deben sopesar los riesgos de una reseccion agresiva frente al riesgo de recurrencia a largo plazo. Presentamos los resultados de nuestra serie de pacientes en funcion de los resultados clinicos, el grado de reseccion, la recurrencia y la supervivencia libre de enfermedad. Materiales y metodos Se revisaron las historias clinicas de todos los pacientes que se sometieron a una reseccion quirurgica por craneofaringioma en el (Hospital Italiano de Buenos Aires) entre 2007 y 2019. Se consideraron examenes oftalmologicos, estudios de imagenes, estudios endocrinologicos y complicaciones quirurgicas. En todos los pacientes se planificaron resecciones totales. Para la correcta eleccion del abordaje quirurgico, los craneofaringiomas se clasificaron segun su ubicacion. Resultados Se analizaron treinta pacientes con diagnostico de craneofaringioma. El 12,5% se clasifico como intraselar, el 12,5% como prequiasmatico, el 43,75% como retroquiasmatico y el 31,25% como intraventricular. En total, 38 casos incluyeron una cirugia transcraneal (15 abordaje orbitocigomatico; 19 abordaje pterional y 4 abordaje transcalloso), siete incluyeron abordaje transeptoesfenoidal, dos abordajes transnasales microscopicos y una endoscopia ventricular para vaciar el quiste del craneofraingioma. La reseccion total se logro en el 43,7% de los pacientes y la reseccion casi total (mayor al 90%) en el 25%. El periodo de seguimiento promedio, despues de la reseccion, fue de 4,7 anos. La recurrencia tumoral ocurrio en el 48%, con un promedio de meses libres de enfermedad de 42,7. Conclusion La reseccion tumoral total es el mejor tratamiento para los craneofaringiomas. Debido a sus altas morbilidad y mortalidad, se necesita un equipo multidisciplinario para el manejo de estos tumores.

Background Acromegaly is associated with higher morbidity and mortality mainly due to cardiovascular disease. Data on the incidence and evolution of thyroid cancer in acromegaly are controversial. Our objective was to describe the characteristics of a group of acromegalic patients with differentiated thyroid carcinoma (DTC) and analyze their evolution. Methods This is a retrospective multicenter study of 24 acromegalic patients with DTC. The AJCC Staging System 8th Edition was used for TNM staging, and the initial risk of recurrence (RR), initial response and response at the end of follow-up (RFU) were defined according to the 2015 ATA Guidelines. As a control group, 92 patients with DTC without acromegaly were randomly included. Statistical analyses were done using SPSS Statistics 20.0. Results Median age of patients at diagnosis of acromegaly was 49.5 years (range 12–69). The median delay in diagnosis of acromegaly was 3 years (range 0.5–23). Mean baseline IGF-1 level was 2.9 ± 1.1 ULN. Median age at DTC diagnosis was 51.5 years (18–69). At the moment of diagnosis of DTC, 58.3% of the patients had active acromegaly. Median time from DTC diagnosis to acromegaly control was 1.25 years (0.5–7). Mean DTC tumor diameter of the biggest lesion was 14.6 ± 9.2 mm, being multifocal in 37.5%. All tumors were papillary carcinomas, two cases being of an aggressive variety. Lymph node dissection was performed in 8 out of 24 patients and 62.5% had metastases. Only one patient had distant metastases. Radioiodine ablation was given to 87.5% of patients. Nineteen patients (79%) were stage I, four (17%) stage II and one (4%) stage IVb. Initial RR was low in 87% (21/24), intermediate in 9% (2/24) and high in 4% (1/24) patient. RFU was: 83% (19/23) patients with no evidence of disease, 9% (2/23) with indeterminate response, 4% (1/23) with biochemical incomplete response and 4% (1/23) with structural incomplete response, at a median time of FU of 36.5 months. When comparing RFU between acromegalics and controls no statistically significant differences were found. Conclusions Patients with acromegaly and DTC mostly had a low initial RR. When compared with the control group, we found that DTC patients with acromegaly did not have a worse evolution.

Rathke's cleft cyst (RCC) are a slow-growing, benign, cystic lesions that arises from the remnants of the primitive ectoderm and Rathke's pouch. They present a common origin with pituitary adenomas (PA), however, the concomitant presentation of a PA and a RCC rarely occur. We present a case of a 41-year-old female with Cushing's disease. Magnetic resonance imaging (MRI) showed two synchronic lesions in the sellar region. An endoscopic transnasal approach was performed, with complete excision of both. The histological studies revealed an ACTH secreting PA and a RCC. The patient presented clinical and endocrinological remission six months after surgery. With the presence of cystic lesion at the sellar region, and the concomitant finding of a pituitary adenoma, RCC should be considered.El quiste de la bolsa de Rathke (QBR) es una entidad benigna de crecimiento lento que proviene del remanente del ectodermo primitivo. Presenta un origen común con los adenomas hipofisarios (AH), sin embargo, la presentación sincrónica de un AH y un QBR es infrecuente. Presentamos el caso de una mujer de 41 años con enfermedad de Cushing. Se realizó resonancia magnética con el hallazgo de dos lesiones en región selar. Se hizo un abordaje transnasal endoscópico, con exéresis completa de ambas. El informe de anatomía patológica reveló un AH corticotropo y un QBR. Tuvo una remisión clínica analítica endocrinológica a los seis meses postquirúrgicos. Ante el hallazgo de una imagen quística a nivel selar concomitante con un adenoma hipofisario, debe ser considerada la posibilidad diagnóstica de un QBR.

Objective To describe the long term safety and efficacy of pegvisomant (PEGV), and the predictors of treatment response in patients with acromegaly in the real life setting. Subjects and methods We retrospectively reviewed the clinical, hormonal and radiological data of acromegalic patients treated with PEGV in 17 Argentine centers. Results Seventy-five patients (age range 22-77, 51 females) with acromegaly have been treated with PEGV for up to 118 months (median 27 months). Before PEGV, 97.3% of patients had been treated with medical therapy, surgery and/or radiotherapy, two patients had no previous treatment. At that time, all patients had an IGF-1 above the upper normal limit (ULN) (mean 2.4 x ULN ± 0.98, range 1.25-7). At diagnosis of acromegaly 84% presented macroadenomas, prior to PEGV only 23,5% of patients remained with tumor remnant > 1 cm, the remaining showed normal or less than 1 cm images. Disease control (IGF-1 ≤ 1.2 x ULN) was achieved in 62.9% of patients with a mean dose of 11.8 mg/day. Thirty-four patients (45%) received PEGV monotherapy, while 41 (55%) received combined therapy with either somatostatin analogues and/or cabergoline. Adverse events related to PEGV were: local injection site reaction in 5.3%, elevated liver enzymes in 9.3%, and tumor size growth in 9.8%. Pre-PEGV IGF-I level was the only predictor of treatment response: 2.1 x ULN vs 2.8 x ULN in controlled and uncontrolled patients respectively (p < 0.001). Conclusion this long term experience indicates PEGV treatment was highly effective and safe in our series of Argentine patients with acromegaly refractory to standard therapies. Arch Endocrinol Metab. 2019;63(4):320-7

Background: Craniopharyngioma (CP) is a rare tumor in the elderly whose clinical features and prognosis are not well known in this population. Aim: To evaluate the clinicopathological features and therapeutic outcomes of CP diagnosed in the elderly. Patients and Methods: This was a retrospective, multicenter, national study of CP patients diagnosed over the age of 65 years and surgically treated. Results: From a total of 384 adult CP patients, we selected 53 (13.8%) patients (27 women [50.9%], mean age 72.3 ± 5.1 years [range 65–83 years]) diagnosed after the age of 65 years. The most common clinical symptoms were visual field defects (71.2%) followed by headache (45.3%). The maximum tumor diameter was 2.9 ± 1.1 cm. In most patients, the tumor was suprasellar (96.2%) and mixed (solid-cystic) (58.5%). The surgical approach most commonly used was transcranial surgery (52.8%), and more than half of the patients (54.7%) underwent subtotal resection (STR). Adamantinomatous CP and papillary CP were present in 51 and 45.1%, respectively, with mixed forms in the remaining. Surgery was accompanied by an improvement in visual field defects and in headaches; however, pituitary hormonal hypofunction increased, mainly at the expense of an increase in the prevalence of diabetes insipidus (DI) (from 3.9 to 69.2%). Near-total resection (NTR) was associated with a higher prevalence of DI compared with subtotal resection (87.5 vs. 53.6%, p = 0.008). Patients were followed for 46.7 ± 40.8 months. The mortality rate was 39.6% with a median survival time of 88 (95% CI: 57–118) months. DI at last visit was associated with a lower survival. Conclusion: CP diagnosed in the elderly shows a similar distribution by sex and histologic forms than that diagnosed at younger ages. At presentation, visual field alterations and headaches are the main clinical symptoms which improve substantially with surgery. However, surgery, mainly NTR, is accompanied by worsening of pituitary function, especially DI, which seems to be a predictor of mortality in this population.

Current international guidelines recommend colonoscopy in patients with acromegaly at the time of diagnosis, even though the risk of developing colorectal neoplasm is still controversial. The main objective of this Argentine multicenter study was to analyze through screening colonoscopy the presence of advanced neoplastic lesions considered as precancerous, in patients with acromegaly compared to a control group. This is a case–control retrospective study. Full length colonoscopy of 70 acromegalic patients and 128 control subjects were studied. Polyps were classified into non pre-cancerous lesions and advance neoplastic lesions which included advanced adenomas (preneoplastic) and colorectal carcinomas. Thirty three out of 70 acromegalic patients and 32 out of 128 subjects controls presented polyps in the colonoscopy [47.1% vs 25%, p = 0.002, OR 2.68]. Non precancerous polyps were found in 11 (15.7%) and 23 (17.9%) (p = 0.690), while advanced neoplastic lesions were found in 22 (31.4%) and 9 (7.0%) (p = 0,0001 – OR: 6.06) patients and controls respectively. Advanced adenomas and colorectal carcinomas were found in 18 (27.3%) and 9 (7.0%) (p = 0,0006—OR: 4,57), and 4 (5.7%) and 0 (0.0%) p = 0.0063) of patients and controls respectively. The presence of insulin resistance was the only statistically significant associated factor among acromegalic patients with and without colonic polyps. Our findings show an increased risk of preneoplastic colonic lesions and colorectal carcinoma in patients with chronic and sustained GH excess compared to a control group. This supports the recommendation to perform screening colonoscopy at diagnosis of acromegaly.

Resumen Introduccion El riesgo de desarrollar neoplasias colonicas en pacientes acromegalicos y su relacion directa con los niveles elevados de GH/IGF-1 no estan bien establecidos y continuan siendo motivo de controversia en la literatura mundial. El objetivo de este trabajo fue evaluar el riesgo de desarrollar lesiones neoplasicas avanzadas (LNA) (adenomas mayores a 1 cm, componente velloso mayor del 75% y/o displasia de alto grado), en pacientes con acromegalia, comparado con un grupo control. Materiales y metodos Estudio multicentrico caso-control retrospectivo. Ciento treinta y siete pacientes con acromegalia que realizaron videocolonoscopia (VCC) fueron incluidos inicialmente, aunque solo 69 cumplieron criterios de inclusion. Sesenta y dos controles fueron obtenidos: por cada caso (paciente con acromegalia) 2 «controles» fueron seleccionados aleatorizadamente e igualados por edad y sexo. El riesgo se expreso en odds ratio (OR) y su correspondiente intervalo de confianza (IC) del 95%. La significacion estadistica fue considerada una p Resultados De los 69 pacientes con VCC completa y datos adecuados para su analisis, 28 presentaron VCC positiva con hallazgos de polipos (40%) y 41 VCC negativa o normal (60%). Dentro del grupo con VCC positiva, 14 presentaron LNA (20%) y solo un paciente presento diagnostico de cancer colorrectal. Para el analisis caso-control se incluyo a 31 pacientes frente al grupo control (n = 62) que cumplieron con los criterios de inclusion. La presencia de polipos colonicos, adenomas y LNA en los pacientes con acromegalia fue de 19/31 (61,9%), 14/31 (45,16%) y 10/31 (32,25%), y en el grupo control de 18/62 (29,03%), 11/62 (17,74%) y 4/62 (6,45%), respectivamente. El riesgo de adenomas y LNA fue mayor en el grupo de acromegalia en comparacion con el grupo control, siendo ambos resultados estadisticamente significativos: adenomas OR 2,54 (IC 1,22-5,25) p = 0,005, LNA OR: 7,3 (2,4-25), p = 0,00. Conclusion La acromegalia se asocia a un mayor riesgo de lesiones colonicas preneoplasicas. Este hallazgo justifica el cribado con VCC al diagnostico en pacientes con acromegalia.

Introduction: Pituitary Metastases (MTS) are infrequently seen in clinical practice. The incidence ranges from 0.14 to 28%. Breast and lung cancer are the primary sites that most frequently metastasize to sellar region, between the sixth and seventh decades of life. Most cases are diagnosed in patients with advanced malignant disease, however, in 20-30%, symptoms of pituitary involvement can precede the diagnosis of the primary tumor. Objectives: To evaluate symptoms at presentation, hormonal, radiological and histological findings, management and outcome of a series of patients with pituitary MTS. Patients and methods: medical records of 16 patients from eight Endocrine Centers were reviewed. Ten patients had histological confirmation of the pituitary MTS, 6 were not operated, being the diagnostic criteria the presence of sellar mass associated with diabetes insipidus (DI) and / or sudden-onset of neuro-ophthalmological symptoms in patients with confirmed primary neoplasia. Results: The median age was 54 years (range 35-70), 9 women (56.2%). The sites of the primary tumor were: 7 lung (44%), 5 breast (32%), 1 follicular thyroid carcinoma (6%), 1 Hodgkin lymphoma (6%), 1 poorly differentiated carcinoma (6%), and 1 clear cell renal carcinoma (6%). The median time between the diagnosis of the primary neoplasm and the occurrence of the pituitary MTS was 12 months (range: 3-120). In 9 patients (56.2%), the diagnosis of the primary neoplasm was made after the finding of the symptomatic sellar mass. DI was found in 14 patients (87.5%), adenohypophyseal deficit in 12 (75%), visual disorders in 10 (62.5 %), headache in 6 (37.5%) and cranial nerve deficits in 6 (37.5%). In 68.7% (11 patients), other MTS were detected. Fifteen patients were evaluated by MRI and one by CT: 13 (81.3%) harbored supra / parasellar masses, and the remaining 3 had lesions limited to the pituitary gland, with stalk thickening and lack of spontaneous neurohypophysis hyperintensity in 2 of them. In all cases diffuse gadolinium uptake was present. Fourteen patients died (87.5%), with a median survival time of 6,5 months (range: 1-36); the remaining 2 are still alive with a follow-up period of 4 and 12 months respectively. Conclusions: In this series of 16 patients with pituitary MTS, the most frequent primary neoplasms were lung and breast. Median age was lower than in published series. DI was the most common condition at presentation, followed by hypopituitarism and visual disorders. The short survival was related to the extent of the disease at the time of diagnosis. In more than half of the cases the diagnosis of primary neoplasia was made through the symptomatic pituitary mass. In the presence of a pituitary lesion with diffuse gadolinium uptake, associated with DI and / or acute visual deficit, pituitary MTS should be suspected even in patients without a history of oncological disease.

Objetive The aim was to assess the evolution of tumor size and prolactin (PRL) levels in patients with micro and macroprolactinomas diagnosed and treated with dopamine agonists during fertile age, and the effects of suspension of drugs after menopause. Retrospective study, 29 patients with prolactinomas, 22 microadenomas and 7 macroadenomas, diagnosed during their fertile age were studied in their menopause; treatment was stopped in this period. Age at menopause was 49 ± 3.6 years. The average time of treatment was 135 ± 79 months. The time of follow-up after treatment suspension was 4 to 192 months. Results Pre-treatment PRL levels in micro and macroadenomas were 119 ± 57 ng/mL and 258 ± 225 ng/mL, respectively. During menopause after treatment suspension, and at the latest follow-up: in microadenomas PRL levels were 23 ± 13 ng/mL and 16 ± 5.7 ng/mL, respectively; in macroadenomas, PRL levels were 20 ± 6.6 ng/mL 5t5and 25 ± 18 ng/mL, respectively. In menopause after treatment suspension, the microadenomas had disappeared in 9/22 and had decreased in 13/22. In the group of patients whose tumor had decreased, in the latest follow-up, tumors disappeared in 7/13 and remained unchanged in 6/13. In macroadenomas, after treatment suspension 3/7 had disappeared, 3/7 decreased and 1/7 remained unchanged. In the latest control in the 3 patients whose tumor decreased, disappeared in 1/3, decreased in 1/3 and there was no change in the remaining. Conclusions Normal PRL levels and sustained reduction or disappearance of adenomas were achieved in most of patients, probably due to the decrease of estrogen levels. Dopamine agonists might be stopped after menopause in patients with prolactinomas.

The aim of our study was to evaluate two different methodologies in IGF-I levels determination, its correlation with GH nadir in OGTT

The aim of the study is to assess the rate of any potential adverse effects on women who became pregnant under cabergoline (CAB) treatment and to evaluate any effects on the embryo-fetal development and on children who were born from mothers exposed to CAB in early weeks of gestation. Observational, retrospective and multicenter study on 103 pregnancies in 90 women with hyperprolactinemia. All patients were under CAB at conception. Serum prolactin at baseline was between 30 and 1921 ng/ml. Duration of therapy before pregnancy ranged from 1 to 120 months and doses ranged from 0.125 to 5 mg/week. Fetal exposure ranged from 3 to 25 weeks, 96.9% of patients received CAB during the first trimester of pregnancy and the rest until the second one. No significant complications during pregnancy were found. Seven women (7.2%) had spontaneous abortions. Preterm deliveries were recorded in eight (8.8%), only one with low weight for gestational age. Neonatal abnormalities were observed in 3 (3.6%): 1 major (Down syndrome) and 2 minor malformations (umbilical and inguinal hernia). We were able to asses the children's development in 61. Two had epilepsy and two had Pervasive Developmental Disorder (PDD). No significantly higher frequency of complications was found in pregnancies and/or offspring exposed to CAB than in the normal population. We registered 2 abnormalities in the development of the children: epilepsy and PDD. Larger series of patients are needed to assess the safety of this drug during pregnancy.

The aim of this study was to evaluate the changes in bone metabolism in breastfeeding women (BF). We selected 30 primiparous women and compared them to 31 nulliparous women. We assessed bone mineral density (BMD) in the lumbar spine (LS), femoral neck (FN) and trochanter (TROC), biochemical parameters of bone turnover and hormone and cytokine levels at the puerperium, 6 months and 12 months after delivery. A trend to lower BMD of LS was seen at initial evaluation in BF. BMD in LS, FN, and TROC were increased 12 months after delivery. Baseline body mass index was higher in puerperal women (p = 0.02) and correlated with an increased FN and TROC BMD one year post delivery (p = 0.001 and p = 0.003). An increase in bone remodeling markers, and lower urinary calcium was observed; after 12 months these values normalized. Prolactin, parathormone related peptide (PTHrP) and IL-6 were enhanced during the first six months of breastfeeding. We conclude that calcium for breastfeeding was obtained by transient mobilization of calcium deposits from the trabecular bone, and urinary calcium sparing induced by calciotrophic hormones and cytokines. Body weigth is an important factor in proximal femur BMD.El objetivo de este estudio fue evaluar los cambios que suceden en el metabolismo óseo de mujeres que amamantan. Se seleccionaron 30 mujeres primíparas y se compararon con 30 mujeres nulíparas como grupo control. Se evaluó la densidad mineral ósea (DMO) del raquis lumbar (RL), cuello femoral (CF) y trocánter (TROC), parámetros del remodelado óseo, hormonas y citoquinas. Estos parámetros se midieron en el puerperio inmediato, y a los 6 y 12 meses post-parto. La DMO del RL de la mujeres primíparas evidenciaron una tendencia a menores valores al comienzo de la lactancia comparadas con las mujeres controles, y se observó un incremento significativo de la DMO a los 12 meses, alcanzando valores similares al grupo control. La DMO en CF y del TROC aumentó significativamente a los 12 meses post parto. El índice de masa corporal basal fue mayor en el grupo de primíparas en el puerperio inmediato (p = 0.02) y correlacionó con el incremento observado en la DMO CF y del TROC al año del post-parto (p = 0.001 y p = 0.003, respectivamente). Un alto remodelado óseo y descenso en el calcio urinario se observaron en el puerperio inmediato, y tanto los marcadores óseos como la calciuria se normalizaron a los 12 meses post-parto. Prolactina, interleuquina-6 y el PTHrP aumentaron significativamente en los primeros 6 meses. Concluimos que el calcio de la leche materna proviene de la movilización de los depósitos cálcicos del hueso trabecular y del ahorro de la pérdida renal inducido por las hormonas calcitrópicas y las citoquinas involucradas en el metabolismo óseo. El peso corporal es un factor de importancia en el incremento de DMO del fémur proximal.

Summary IgG4-related hypophysitis is a recently described entity belonging to the group of IgG4-related diseases. Many other organs can also be affected, and it is more common in older men. To date, 32 cases of IgG4-related hypophysitis have been reported in the literature, 11 of which included confirmatory tissue biopsy and the majority affecting multiple organs. The aim of this report is to present two cases of biopsy-proven IgG4-related hypophysitis occurring in two young female patients with no evidence of involvement of other organs at the time of diagnosis. Learning points IgG4-related hypophysitis belongs to the group of IgG4-related diseases, and is a fibro-inflammatory condition characterized by dense lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells and storiform fibrosis. It is more common in older men, but young women may also present this type of hypophysitis. Although involvement of other organs is frequent, isolated pituitary disease is possible. Frequent clinical manifestations include anterior hypopituitarism and/or diabetes insipidus. The diagnosis may be confirmed with any of the following criteria: a pituitary biopsy with lymphoplasmacytic infiltrates, with more than ten IgG4-positive cells; a sellar mass and/or thickened pituitary stalk and a biopsy-proven involvement of another organ; a sellar mass and/or thickened pituitary stalk and IgG4 serum levels >140 mg/dl and sellar mass reduction and symptom improvement after corticosteroid treatment. Glucocorticoids are recommended as first-line therapy.

Adrenocorticotropin (ACTH) independent bilateral macronodular adrenal hyperplasia (AIMAH) is a rare cause of Cushing´s syndrome, characterized by bilateral adrenal lesions and excess cortisol production despite ACTH suppression. Cortisol synthesis is produced in response to abnormal activation of G-protein- coupled receptors, such as gastric inhibitory peptide, vasopressin, beta adrenergic agonists, LH/hCG and serotonin receptors. The aim of this study was to analyze the expression of glucagon receptors in adrenal glands from an AIMAH patient. A patient with ACTH-independent Cushing´s syndrome and bilateral macronodular adrenal hyperplasia was screened for altered activation of adrenal receptors by physiological (mixed meal) and pharmacological (gonadotrophin releasing hormone, ACTH and glucagon) tests. The results showed abnormally high levels of serum cortisol after stimulation with glucagon. Hypercortisolism was successfully managed with ketoconazole treatment. Interestingly, a 4-month treatment with a somatostatin analogue (octreotide) was also able to reduce cortisol secretion. Finally, Cushing's syndrome was cured after bilateral adrenalectomy. Abnormal mRNA expression for glucagon receptor in the patient´s adrenal glands was observed by Real-Time PCR procedure. These results strongly suggest that the mechanism of AIMAH causing Cushing´s syndrome in this case involves the illicit activation of adrenal glucagon receptors. This is the first case reported of AIMAH associated with ectopic glucagon receptors.La hiperplasia adrenal macronodular bilateral independiente de ACTH (HAMIA) es una causa infrecuente de Síndrome de Cushing, caracterizada por lesiones adrenales bilaterales, hipercortisolismo y ACTH plasmática suprimida. La síntesis de cortisol estaría regulada a través de ligandos de receptores asociados a proteína G que se expresan en forma aberrante en la corteza de las glándulas adrenales. El objetivo de este trabajo es analizar la presencia del receptor de glucagón en tejido adrenal de un paciente con diagnóstico de Síndrome de Cushing asociado a hiperplasia adrenal bilateral independiente de ACTH. Se realizaron tests de estímulos fisiológicos y farmacológicos para evaluar la respuesta en la secreción de cortisol. Como resultado se observó respuesta significativa del cortisol posterior al estímulo con glucagón. El paciente presentó buena evolución clínica y bioquímica al tratamiento con ketoconazol. La administración del análogo de somatostatina (octreotide) redujo los niveles de cortisol. Finalmente, la curación se logró posteriormente a la adrenalectomía bilateral. Mediante el estudio de PCR en Tiempo Real se halló la presencia del receptor de glucagón en tejido adrenal del paciente. Según nuestro conocimiento, es el primer paciente descripto de HAMIA vinculado a la expresión ilícita de receptores de glucagón.

The term primary empty sella (PES) makes reference to the herniation of the subarachnoid space within the sella turcica in patients with no history of pituitary tumor, surgery or radiotherapy. To retrospectively assess clinical features, radiological findings and the biochemical endocrine function from the records of 175 patients with a diagnosis of PES. One hundred seventy-five patients (150 females) were studied. The mean age at diagnosis was 48.2 ± 14 year. Most diagnoses were made by magnetic resonance imaging (n = 172). In most patients, the pituitary function was assessed by basal pituitary hormones measurements. Pituitary scans were ordered for different reasons: headache (33.1 %), endocrine disorders (30.6 %), neurological symptoms (12.5 %), visual disturbances (8.75 %), abnormalities on sella turcica radiograph (8.75 %) and others (6.25 %). Multiple pregnancies were observed in 58.3 % of women; headaches, obesity, and hypertension were found in 59.4, 49.5, and 27.3 % of the studied population, respectively. Mild hyperprolactinemia (

Prolactinomas in men are usually macroprolactinomas and other investigators have attributed bigger size of tumors in men to delay in diagnosis. A retrospective study of 71 macroadenomas (42 men) was carried out. Parameters studied were age, signs and symptoms at presentation, time of onset of symptoms, basal prolactin, estradiol, and total testosterone levels, tumor size and Ki 67 expression in tumor tissue. Male patients were older. Visual defects were significantly more prevalent in men. Hardy 4 stage tumors were found only in men. We found no significant correlation between tumor size and the patients age nor between tumor size and the onset of symptoms. Whereas basal E2 levels (21.2+/-12.9 vs. 33.3+/-43.3 pg/ml, p=n.s.) were very similar in male and female patients, testosterone levels were significantly higher in men (0.6+/-0.5 vs. 1.8+/-1.2 ng/ml, p=0.02). The rate of cell proliferation represented by Ki 67 was significantly higher in tumors in men (3.5+/-1.2 vs. 1.5+/-0.5%, p=0.0001). This is the first study focused in macroprolactinomas that shows that they are clinically and biologically more aggressive in men. Hypogonadism in men could appear later in the progression of prolactinomas and this might explain why men were older at the time of diagnosis. Furthermore, testosterone could be a source for E2 in situ aromatization giving male tumors an advantage in cell proliferation.

Prolactinomas in men are usually macroprolactinomas and other investigators have attributed bigger size of tumors in men to delay in diagnosis. A retrospective study of 71 macroadenomas (42 men) was carried out. Parameters studied were age, signs and symptoms at presentation, time of onset of symptoms, basal prolactin, estradiol, and total testosterone levels, tumor size and Ki 67 expression in tumor tissue. Male patients were older. Visual defects were significantly more prevalent in men. Hardy 4 stage tumors were found only in men. We found no significant correlation between tumor size and the patients age nor between tumor size and the onset of symptoms. Whereas basal E2 levels (21.2+/-12.9 vs. 33.3+/-43.3 pg/ml, p=n.s.) were very similar in male and female patients, testosterone levels were significantly higher in men (0.6+/-0.5 vs. 1.8+/-1.2 ng/ml, p=0.02). The rate of cell proliferation represented by Ki 67 was significantly higher in tumors in men (3.5+/-1.2 vs. 1.5+/-0.5%, p=0.0001). This is the first study focused in macroprolactinomas that shows that they are clinically and biologically more aggressive in men. Hypogonadism in men could appear later in the progression of prolactinomas and this might explain why men were older at the time of diagnosis. Furthermore, testosterone could be a source for E2 in situ aromatization giving male tumors an advantage in cell proliferation.

Ectopic acromegaly represents less than 1% of the reported cases of acromegaly. Although clinical improvement is common after treatment with somatostatin (SMS) analogs, the biochemical response and tumor size of the growth hormone-releasing hormone (GHRH)-producing tumor and its metastases are less predictable. Subject A 36-year-old male was referred because of a 3-year history of acromegaly related symptoms. He had undergone lung surgery in 1987 for a "benign" carcinoid tumor. Endocrine evaluation confirmed acromegaly Plasma IGF-1: 984 ng/ml (63-380), GH: 49.8 ng/ml (