RESUMO
Pituitary diseases such as acromegaly and Cushing's disease require surgical or medical therapy. In some functioning pituitary tumors, a spontaneous remission of hormonal hypersecretion is observed, mainly associated to apoplexy or pituitary infarction. We report the evolution of two female patients older than 70 years at the time of diagnosis, with multiple comorbidities. In case 1, acromegaly was diagnosed at 74 years of age. Sellar CT scan showed a 10-mm adenoma. During her follow-up, IGF1 levels remained normal. Nine years later, a magnetic resonance (MR) showed a 7-mm adenoma. In case 2, clinical and biochemical diagnosis of Cushing's disease was done being 71 years old. Sellar MR showed a 6-mm adenoma. Three years later, urinary cortisol normalized with no changes in adenoma at MR. Seven years later, she remains without clinical or biochemical signs of hypercortisolism. In both cases, no signs of hemorrhage were observed at MR.
Assuntos
Acromegalia/diagnóstico por imagem , Adenoma/diagnóstico por imagem , Hipersecreção Hipofisária de ACTH/diagnóstico por imagem , Neoplasias Hipofisárias/diagnóstico por imagem , Acromegalia/fisiopatologia , Adenoma/fisiopatologia , Idoso , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Hipersecreção Hipofisária de ACTH/fisiopatologia , Apoplexia Hipofisária , Neoplasias Hipofisárias/fisiopatologia , Remissão EspontâneaRESUMO
RESUMEN Los pacientes con adenomas hipofisarios constituyen una población heterogénea y requieren un enfoque individualizado. El objetivo de nuestro trabajo fue analizar nuestra población con adenomas hipofisarios no funcionantes (ACNF) y evaluar factores pronóstico de crecimiento (como el Ki-67) que ayuden en la toma de decisiones. Se realizó un análisis retrospectivo de 202 pacientes, incluyendo evaluación basal, enfoque terapéutico y evolución tumoral en 2 grupos: pacientes con conducta expectante (n = 69) y pacientes con cirugía (n = 133). La serie tuvo 55% de pacientes mujeres y la edad media al diagnóstico fue de 49 años. Los motivos de consulta más frecuentes fueron incidentaloma hipofisario y alteraciones visuales. Radiológicamente, 83% fueron macroadenomas, 77% invasivos y 55% mostraron compromiso visual. Entre los adenomas invasores, el 53% tenían disfunción hipofisaria, siendo el hipogonadismo el hallazgo más frecuente. El tratamiento inicial fue la cirugía en el 65,8% realizándose por vía transnasal en el 79% de los casos. Las complicaciones más frecuentes fueron diabetes insípida transitoria e hiponatremia, con mayor incidencia de diabetes insípida permanente en la cirugía transcraneal. La inmunohistoquímica mostró gonatropinomas en el 43,4% de los casos y fue negativa en el 37,7%. Doce adenomas tuvieron índice de proliferación Ki-67 ≥3%. Luego de la cirugía 56,8% de los pacientes mejoraron el campo visual, 22,6% recuperó alguna función endocrina y 18,8% agregó un nuevo déficit. En pacientes no operados, se observó crecimiento tumoral en 5,6% de los adenomas Hardy 1-2 y en el 21% de los Hardy 3-4. Entre los adenomas operados, aquellos sin resto tumoral postoperatorio no presentaron recurrencia. De los tumores con remanente postoperatorio (78,6%) no irradiados, el 41,5% mostró recrecimiento lesional al seguimiento. Este porcentaje se eleva a 66,6% en aquellos con Ki-67 ≥3% y disminuye a 12% en los que recibieron radioterapia.
ABSTRACT Patients with pituitary adenomas are a heterogeneous population and require an individualized approach. The aim of our study was to analyze our population of patients with nonfunctioning pituitary adenomas (NFA) and to evaluate prognostic growth factors (such as Ki-67) that help in decision making. A retrospective analysis of 202 patients, including baseline assessment, therapeutic approach and tumor evolution was performed in 2 groups: expectant management (n = 69) and surgery (n = 133). The mean age at diagnosis was 49 years, 55% women. The most frequent reasons for consultation were pituitary incidentaloma and visual impairment. Eighty three percent were macroadenomas, 77% invasive, and 55% with visual impairment. Among the invasive adenomas, 53% had pituitary dysfunction, with hypogonadism being the most frequent finding. The initial treatment was surgery in 65.8%, 79% of them through transnasal approach. The most frequent complications were transient diabetes insipidus and hyponatremia, with a higher incidence of permanent diabetes insipidus in transcranial surgery. The immunohistochemistry showed: 43.4% gonadotropinomas, 37.7% negative. Twelve adenomas had proliferation index Ki-67 ≥3%. After surgery, 56.8% improved the visual fields, 22.6% recovered some endocrine function and 18.8% added a new deficit. In non-operated patients, tumor growth was observed in 5.6% of the Hardy 1-2 adenomas and 21% of the Hardy 3-4 adenomas. Among the operated adenomas, those without postoperative tumor residue did not present recurrence. In tumors with non-irradiated postoperative remnant (78.6%), 41.5% increased. This percentage rises to 66.6% in those with Ki-67 ≥3%, and decreases to 12% in those who received radiotherapy.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Neoplasias Hipofisárias/complicações , Neoplasias Hipofisárias/fisiopatologia , Adenoma/complicações , Neoplasias Hipofisárias/cirurgia , Prognóstico , Adenoma/radioterapia , Tomada de Decisões , Proliferação de CélulasRESUMO
Introducción: La cirugía endoscópica transesfenoidal en la exéresis de patología hipofisaria ha sido ratificada dentro de la mejores opciones para conseguir abordajes exitosos. Para cerrar la brecha ósea, se puede utilizar el colgajo nasoseptal, el cual lograría un cierre adecuado y seguro, evitando complicaciones como persistencia de fístulas de líquido cefalorraquídeo. Dentro de los probables efectos secundarios de este colgajo se señala la presencia de anosmia o hiposmia. Objetivo: Nuestro objetivo es mostrar los resultados obtenidos empleando la técnica del colgajo nasoseptal y la evaluación del olfato posoperatorio. Material y método: Incluimos 14 pacientes con diagnóstico de adenoma hipofisiario intervenidos en nuestro servicio entre diciembre 2014 a diciembre 2015. Se evaluaron diversos parámetros entre otros, olfatometría pre y posoperatorias. A todos se les realizó la técnica endoscópica transesfenoidal. Efectuando la disección del colgajo con la técnica tipo Hadad. La exéresis tumoral fue realizada por neurocirujano, se cubrió la osteotomía con este colgajo. Resultados: De los 14 pacientes, 13 llegaron al estado olfativo preoperatorio. 1 paciente se mantuvo hipósmico mantenidamente. En nuestro estudio, no hemos encontrado deterioro significativo en la calidad del sentido del olfato. Así como tampoco observamos otras complicaciones. Conclusiones: Esta experiencia aún inicial, estimamos que podría ya esbozar una tendencia de mantención de la función olfativa en este tipo de cirugía.
Introduction: Transsphenoidal endoscopic surgery resection of pituitary pathology has been ratified within the best options for successful approaches. To close the gap bone, you can use the nasoseptal flap, which achieved an adequate and secure closure, preventing complications such as persistent cerebrospinal fluid leaks. Among possible side effects of this flap anosmia-hyposmia noted. Aim: Our goal is to show the results obtained using the technique of nasoseptal flap and postoperative evaluation of smell. Material and method: We included 14 patients with a diagnosis of pituitary adenoma surgery in our department between December 2014 to December 2015. Various parameters were evaluated pre and post operative, specially olfactory function. All underwent transsphenoidal endoscopic technique. Performing dissection flap with the technical type Hadad. The tumor resection was performed by neurosurgeon, osteotomy covered with this flap. Results: Of the 14 patients, 13 reached the olfactory preoperative state. 1 patient remained with hyposmia. In our study, we found no significant deterioration in the quality of the sense of smell. Not other complications were found. Conclusions: This is an initial experience, and we estimate that could outline a trend of maintaining olfactory function in this surgery approach.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Adenoma/cirurgia , Endoscopia/métodos , Neoplasias Hipofisárias/cirurgia , Seio Esfenoidal/cirurgia , Adenoma/fisiopatologia , Epidemiologia Descritiva , Septo Nasal/irrigação sanguínea , Neoplasias Hipofisárias/fisiopatologia , Olfato/fisiologia , Retalhos Cirúrgicos , Resultado do TratamentoRESUMO
Introducción. los craneofaringiomas son malformaciones histológicamente benignas entre el hipotálamo y la hipófisis que pueden afectar la secreción hormonal clave en la regulación endócrina y modulación de la saciedad. Si bien se trata de una enfermedad relativamente benigna, la combinación de obesidad grave de origen hipotalámico y las comorbilidades asociadas a ella disminuyen la calidad de vida. Objetivo. Evaluar el estado nutricional de pacientes operados de craneofaringioma infantil. Población y métodos. Pacientes menores de 21 años al momento del estudio que requirieron cirugía por craneofaringioma en el Hospital de Pediatría Garrahan y que firmaron el consentimiento informado. Se realizó la evaluación antropométrica, composición corporal con impedanciometría, gasto energético con calorimetría indirecta e ingesta energética. Se determinó la resistencia a la insulina y la dislipidemia. Resultados. Se incluyeron 39 pacientes; 41%, normopeso y 59%, obesos. El 68% de los pacientes presentó distribución grasa central; 40%, resistencia a la insulina; y 32%, dislipidemia. No se encontraron diferencias significativas en la presencia de resistencia a la insulina, dislipidemia, gasto energético en reposo ni en la ingesta entre obesos vs. normopeso. El 77% de los pacientes obesos presentó bajo gasto energético independiente del porcentaje de masa magra (62 ± 2,7% vs. 61,2 ± 1,8% de gasto energético en reposo normal vs. bajo; p 0,8). Conclusiones. El 59% de la población estudiada presentó obesidad. No se encontraron diferencias significativas en complicaciones metabólicas entre pacientes obesos y normopeso. Se observó menor gasto energético independiente del porcentaje de masa magra y similar ingesta energética.
Introduction. Craniopharyngiomas are histologically benign malformations located between the pituitary and hypothalamus that may affect key hormone secretion for endocrine regulation and satiety modulation. Although this is a relatively benign disease, the combination of severe hypothalamic obesity and associated comorbidities results in a reduced quality of life. Objective. To assess the nutritional status of patients after craniopharyngioma surgery. population and Methods. Patients younger than 21 years old at the time of the study who required craniopharyngioma surgery at Hospital de Pediatria Garrahan and who signed an informed consent. Anthropometric characteristics, body composition by impedance analysis, energy expenditure by indirect calorimetry and energy intake were assessed. Insulin resistance and dyslipemia were estimated. Results. A total of 39 patients were included; 41% had a normal weight and 59% were obese. Overall, 68% of patients had a central fat distribution; 40% had insulin resistance; and 32%, dyslipemia. No significant differences were observed in terms of insulin resistance, dyslipemia, energy expenditure at rest, or energy intake between normal weight and obese patients. Among obese patients, 77% had a low energy expenditure, regardless of their percentage of lean body mass (62 ± 2.7% versus 61.2 ± 1.8% of normal versus low energy expenditure at rest; p = 0.8). Conclusions. A total of 59% of the studied population was obese. No significant differences were observedinterms ofmetabolic complications between normal weight and obese patients. A lower energy expenditure was observed, regardless of the lean body mass percentage and a similar energy intake.
Assuntos
Humanos , Pré-Escolar , Criança , Adolescente , Adulto Jovem , Neoplasias Hipofisárias/cirurgia , Neoplasias Hipofisárias/fisiopatologia , Estado Nutricional , Craniofaringioma/cirurgia , Craniofaringioma/fisiopatologia , Avaliação Nutricional , Estudos ProspectivosRESUMO
INTRODUCTION: Craniopharyngiomas are histologically benign malformations located between the pituitary and hypothalamus that may affect key hormone secretion for endocrine regulation and satiety modulation. Although this is a relatively benign disease, the combination of severe hypothalamic obesity and associated comorbidities results in a reduced quality of life. OBJECTIVE: To assess the nutritional status of patients after craniopharyngioma surgery. POPULATION AND METHODS: Patients younger than 21 years old at the time of the study who required craniopharyngioma surgery at Hospital de Pediatr.a Garrahan and who signed an informed consent. Anthropometric characteristics, body composition by impedance analysis, energy expenditure by indirect calorimetry and energy intake were assessed. Insulin resistance and dyslipemia were estimated. RESULTS: A total of 39 patients were included; 41% had a normal weight and 59% were obese. Overall, 68% of patients had a central fat distribution; 40% had insulin resistance; and 32%, dyslipemia. No significant differences were observed in terms of insulin resistance, dyslipemia, energy expenditure at rest, or energy intake between normal weight and obese patients. Among obese patients, 77% had a low energy expenditure, regardless of their percentage of lean body mass (62 Å} 2.7% versus 61.2 Å} 1.8% of normal versus low energy expenditure at rest; p = 0.8). CONCLUSIONS: A total of 59% of the studied population was obese. No significant differences were observed in terms of metabolic complications between normal weight and obese patients. A lower energy expenditure was observed, regardless of the lean body mass percentage and a similar energy intake.
Introducción. los craneofaringiomas son malformaciones histológicamente benignas entre el hipotálamo y la hipófisis que pueden afectar la secreción hormonal clave en la regulación endócrina y modulación de la saciedad. Si bien se trata de una enfermedad relativamente benigna, la combinación de obesidad grave de origen hipotalámico y las comorbilidades asociadas a ella disminuyen la calidad de vida. Objetivo. Evaluar el estado nutricional de pacientes operados de craneofaringioma infantil. Población y métodos. Pacientes menores de 21 años al momento del estudio que requirieron cirugía por craneofaringioma en el Hospital de Pediatría Garrahan y que firmaron el consentimiento informado. Se realizó la evaluación antropométrica, composición corporal con impedanciometría, gasto energético con calorimetría indirecta e ingesta energética. Se determinó la resistencia a la insulina y la dislipidemia. Resultados. Se incluyeron 39 pacientes; 41%, normopeso y 59%, obesos. El 68% de los pacientes presentó distribución grasa central; 40%, resistencia a la insulina; y 32%, dislipidemia. No se encontraron diferencias significativas en la presencia de resistencia a la insulina, dislipidemia, gasto energético en reposo ni en la ingesta entre obesos vs. normopeso. El 77% de los pacientes obesos presentó bajo gasto energético independiente del porcentaje de masa magra (62 ± 2,7% vs. 61,2 ± 1,8% de gasto energético en reposo normal vs. bajo; p 0,8). Conclusiones. El 59% de la población estudiada presentó obesidad. No se encontraron diferencias significativas en complicaciones metabólicas entre pacientes obesos y normopeso. Se observó menor gasto energético independiente del porcentaje de masa magra y similar ingesta energética.
Assuntos
Craniofaringioma , Estado Nutricional , Neoplasias Hipofisárias , Adolescente , Criança , Pré-Escolar , Craniofaringioma/fisiopatologia , Craniofaringioma/cirurgia , Feminino , Hospitais , Humanos , Masculino , Avaliação Nutricional , Neoplasias Hipofisárias/fisiopatologia , Neoplasias Hipofisárias/cirurgia , Estudos Prospectivos , Adulto JovemRESUMO
Pituitary apoplexy is characterized by sudden increase in pituitary gland volume secondary to ischemia and/or necrosis, usually in a pituitary adenoma. Most cases occur during the 5th decade of life, predominantly in males and in previously unknown clinically non-functioning pituitary adenomas. There are some predisposing factors as arterial hypertension, anticoagulant therapy and major surgery. Clinical picture comprises headache, visual impairment, cranial nerve palsies and hypopituitarism. Most cases improve with both surgical and expectant management and the best approach in the acute phase is still controversial. Surgery, usually by transsphenoidal route, is indicated if consciousness and/or vision are impaired, despite glucocorticoid replacement and electrolyte support. Pituitary function is impaired in most patients before apoplexy and ACTH deficiency is common, which makes glucocorticoid replacement needed in most cases. Pituitary deficiencies, once established, usually do not recover, regardless the treatment. Sellar imaging and endocrinological function must be periodic reevaluated.
Assuntos
Apoplexia Hipofisária/diagnóstico , Apoplexia Hipofisária/fisiopatologia , Apoplexia Hipofisária/terapia , Doença Aguda , Adenoma/diagnóstico , Adenoma/fisiopatologia , Adenoma/terapia , Gerenciamento Clínico , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/fisiopatologia , Neoplasias Hipofisárias/terapia , Fatores de RiscoRESUMO
OBJECTIVE: To test memory performance and executive functions in patients with childhood craniopharyngioma and hypothalamic involvement. STUDY DESIGN: Using standardized neuropsychological tests, we compared cognitive performance in a group of 15 patients with childhood craniopharyngioma and known hypothalamic involvement and a group of 24 age- and intelligence-matched control subjects. In addition, we compared individual patients' results with normative data to detect abnormal performance in the clinically relevant range. Within the patient group, we further tested whether the grade of hypothalamic involvement had an impact on cognitive performance and quality of life. RESULTS: Relative to healthy controls, the patients demonstrated significantly lower performance scores in tests of memory and executive functioning. On the individual performance level, delayed recall performance was severely impaired in one-third of the patients. Compared with patients with low-grade hypothalamic involvement, those with high-grade hypothalamic involvement showed worse performance in executive functions and reduced functional capabilities for daily life actions, indicating lower quality of life. CONCLUSION: Our findings demonstrate that hypothalamic involvement is related to impairments in memory and executive functioning in patients with childhood craniopharyngioma and indicate that a high grade of hypothalamic involvement is related to worse outcomes.
Assuntos
Craniofaringioma/fisiopatologia , Neoplasias Hipotalâmicas/fisiopatologia , Neoplasias Primárias Múltiplas/fisiopatologia , Neoplasias Hipofisárias/fisiopatologia , Adolescente , Criança , Feminino , Humanos , Masculino , Testes NeuropsicológicosRESUMO
Apoptosis, also known as programmed cell death, is a phenomenon in which different stimuli trigger cellular mechanisms that culminate in death, in the absence of inflammatory cell response. Two different activation pathways are known, the intrinsic pathway (or mitochondrial) and extrinsic (or death-receptor pathway), both pathways trigger enzymatic reactions that lead cells to break up and be phagocytized by neighboring cells. This process is a common occurrence in physiological and pathological states, participating in the control of cell proliferation, differentiation and remodeling of organs. In the early steps of pituitary gland formation, numerous apoptotic cells are detected in the separation of Rathke's pouch from the roof of oral ectoderm. In the distal part of the gland, which will form the adenohypophysis, the ratio of apoptosis was significantly lower. However, there is evidence that neoplastic pituitary cells undergo unbalance in genes that control apoptosis leading to uncontrolled cell growth. No direct evidence of apoptosis was found in the drugs used for tumors producing prolactin and growth hormone. In conclusion, an unbalancing in the apoptosis process is the boundary between development and tumor growth.
Assuntos
Apoptose/fisiologia , Hipófise/embriologia , Hipófise/fisiologia , Neoplasias Hipofisárias/fisiopatologia , Diferenciação Celular/fisiologia , Proliferação de Células , Transformação Celular Neoplásica/patologia , Humanos , Hipófise/patologia , Neoplasias Hipofisárias/patologia , Transdução de Sinais/fisiologiaRESUMO
OBJECTIVES: To determine the frequency of macroprolactinemia in a cohort of hyperprolactinemic women, describing 1) the association of macroprolactinemia with clinical variables and morphological changes in the pituitary gland and 2) clinical status and prolactin levels after 10 years of follow-up. DESIGN: Blood samples were obtained from 32 patients for hormonal assessment. Treatment with cabergoline or bromocriptine was interrupted 3 months before the determination of serum prolactin and macroprolactin. Macroprolactin was measured using the polyethylene glycol (PEG) precipitation method. Computed tomography was performed in all patients. RESULTS: The frequency of macroprolactinemia was 28.1%. In 19 patients prolactin remained elevated (persistent hyperprolactinemia). In 13, prolactin returned to normal (former hyperprolactinemia). Nine patients with PEG recovery between 40 and 50%, and the only two macroprolactinemic patients with previous hyperprolactinemia were excluded from the analysis of clinical outcomes. Only one of seven macroprolactinemic patients had an abnormal pituitary image (empty sella). None had galactorrhea. MAIN FINDINGS: Classic symptoms of hyperprolactinemia and abnormal imaging findings are not common in patients in whom macroprolactin is the predominant form of PRL. CONCLUSIONS: Women with hyperprolactinemia, especially if asymptomatic, should be routinely screened for macroprolactinemia. Macroprolactinemia remains stable in the long term.
Assuntos
Hiperprolactinemia/diagnóstico , Hiperprolactinemia/fisiopatologia , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/fisiopatologia , Prolactinoma/diagnóstico , Prolactinoma/fisiopatologia , Adulto , Estudos de Coortes , Feminino , Seguimentos , Galactorreia/diagnóstico , Galactorreia/fisiopatologia , Humanos , Pessoa de Meia-Idade , Adeno-Hipófise/fisiologia , Gravidez , Resultado da Gravidez , Prolactina/sangue , Fatores de Tempo , Adulto JovemRESUMO
The adaptation of GH(3) cells to different microenvironments is a consequence of a partial compromise with the tumor phenotype. A collagen type IV enriched microenvironment favors an invasive phenotype and increases the substrate adhesion capacity, whereas it decreases the phosphorylation of the regulatory myosin light chain and the aggregation capacity. In contrast, the higher internal tension and increased aggregation capacity induced by collagen type I/III are factors that reduce the invasion rate. Our results show, for the first time, the importance of collagen subtypes in determining the migratory strategy: collagen I/III favors mesenchymal-like motility, whereas collagen type IV induces an ameboid-type displacement. The reciprocal modulation of the myosin light chain kinase and the Rho-kinase determines the invasive capacity through changes in tissue cohesion, extracellular matrix affinity, regulatory myosin light chain phosphorylation and spatial distribution. The collagen subtype determines which of the mechano-transduction signaling pathways will regulate the tensional homeostasis and affect the invasion ability as well as the preferred migration strategy of the cells.