RESUMEN
Prolactinoma is frequently found not only in females but also in males with abnormal reproductive and/or sexual function. Patients typically complain about amenorrhea and infertility because of anovulation. Approximately 15% to 20% of cases of secondary amenorrhea are caused by prolactinemia. Galactorrhea may occur simultaneously, before or after menstrual disorders, and sometimes it may not be clinically obvious, or only detected by breast examination. We reported a case of a 14-year-old girl who presented primary amenorrhea accompanied by frequent headaches and blurred vision. Hormonal tests showed severe hyperprolactinemia (prolactin [PRL] concentration: 1,570 ng/ml). Further tests confirmed a mass in the pituitary with an extension to the left parasellar and suprasellar regions. Some parts of the sella turcica tumor were removed by transcranial surgery. During the follow-up, the clinicopathological examinations revealed the patient had hyperprolactinemia. Clinicians should be aware of the diagnostic and therapeutic problems regarding the management of hyperprolactinemia.
O prolactinoma é frequentemente encontrado não apenas em mulheres, mas também em homens com função reprodutiva e/ou sexual anormal. Os pacientes geralmente se queixam de amenorreia e infertilidade devido à anovulação. Aproximadamente 15% a 20% dos casos de amenorreia secundária são causados por prolactinoma. A galactorreia pode ocorrer simultaneamente, antes ou depois dos distúrbios menstruais, e às vezes pode não ser clinicamente óbvia ou ser detectada apenas pelo exame das mamas. Relatamos o caso de uma menina de 14 anos que apresentou amenorreia primária acompanhada de dores de cabeça frequentes e visão turva. Os testes hormonais mostraram hiperprolactinemia grave (concentração de prolactina [PRL]: 1.570 ng/ml). Outros exames confirmaram uma massa na hipófise com extensão para as regiões parasselar e suprasselar esquerda. Algumas partes do tumor da sela turca foram removidas por cirurgia transcraniana. Durante o acompanhamento, os exames clinicopatológicos revelaram que o paciente apresentava hiperprolactinemia. Os médicos devem estar cientes dos problemas diagnósticos e terapêuticos relativos ao manejo da hiperprolactinemia.
RESUMEN
The study aimed to evaluate the retinal ganglion cell structure using optical coherence tomography and the visual pathway function employing visual evoked potentials in the diagnosis and monitoring of patients with pituitary macroadenoma. A descriptive, cross-sectional, and longitudinal study (3 and 12 months follow-up) was conducted on forty-two patients. Thirty-five age-matched healthy controls were used in the cross-sectional one. Full neuro-ophthalmological evaluation (structural and functional) was carried out including global and segmented retinal nerve fiber layer/ganglion cell complex analysis and amplitude and latency of P100 component in the electrophysiology. Statistical data analysis was conducted with R version 3.6.3 and Python version 3.8. Associations were evaluated using Spearman's correlations. Amplitude sensitivities were 0.999, and bi-nasal sectors of ganglion cell complex thickness specificities were 0.999. This structural parameter had the highest diagnostic value (area under curve = 0.923). Significant associations were found between bi-nasal sectors with amplitude at 12' (rho > 0.7, p < 0.01) and median deviation of the visual field (rho > 0.5, p < 0.01) at 3 months. Pre-surgical values of bi-nasal sectors and amplitude can predict post-surgically median deviation and amplitude (Oz, 12') at 3 months with r 2 > 0.5. Bi-nasal sectors of ganglion cell complex and visual evoked potentials P100 amplitude are efficient biomarkers of visual pathway damage for pituitary macroadenoma patients' management. Pre-surgical values of the bi-nasal sector and visual evoked potentials' amplitude could help to predict the restoration of parvocellular pathway traffic after decompression.
RESUMEN
Chiasmal syndromes present mostly with visual problems, such as changes in visual fields, decreased visual acuity, or dyschromatopsia (and classically without pupillary reflex defects). The prototypical bitemporal hemianopia upon visual field testing can easily suggest chiasmal compression due to sellar/suprasellar involvement. However, because of the complexity of the decussation of fibres at the optic chiasm and the presence of anatomical variants, unpredictable visual fields defects can be detected in chiasmal diseases. In some patients, especially in those who have undergone neurosurgical procedures, visual field examination and neuroimaging may not completely reflect the classical pattern of chiasmal visual loss. We describe a novel semiological sign, reporting a patient in which a pupillary bitemporal hemihypokinesia was not accompanied by hemianopia, with the phenomenon being abolished by surgical resection of the causative pituitary macroadenoma. In addition, this finding was an important tool in making the diagnosis.
RESUMEN
PURPOSE: This study aimed to review the cases of giant adenomas that were operated on from 2012 to 2020 and to analyze the epidemiological profile, complications, and resection rates using the transseptal/transnasal endoscopy technique. We also compared the results with data from revised literature. METHODS: This is an observational, retrospective study includes a review of the medical records of 26 patients diagnosed with giant adenomas (diameter > 4 cm or volume >10 cm3) who underwent 28 surgical procedures. RESULTS: Of the 57 patients operated for pituitary adenoma, 26 (50.8%) had giant adenomas. The mean volume was 17.28 cm3 (95% confidence interval [CI], 8.3359-26.2241). Moreover, 64.28% of the patients were graded Knosp 3 (p < 0.0001). Most cases were of nonsecretory adenomas (88.46%). Visual impairment was present in 89.2% of the patients and hormonal deficit and headache affected 53.5% of them. The visual impairment improved in 60% of the patients. The most common surgical complication was cerebrospinal fluid fistula (10.71%). Tumor resection > 90% was achieved in 53.56% of the cases, and the mean resection rate was 78.36% (95% CI, 71.316-87.956). CONCLUSIONS: The endoscopic endonasal technique is a good treatment option for giant adenomas, showing satisfactory optic apparatus decompression rates and a low incidence of complications when performed by surgeons with expertise in this approach.
Asunto(s)
Adenoma , Neoplasias Hipofisarias , Adenoma/diagnóstico por imagen , Adenoma/epidemiología , Adenoma/cirugía , Brasil/epidemiología , Endoscopía/métodos , Humanos , Imagen por Resonancia Magnética , Neoplasias Hipofisarias/complicaciones , Neoplasias Hipofisarias/diagnóstico por imagen , Neoplasias Hipofisarias/cirugía , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
PURPOSE: RAS genes are among the most frequently mutated genes in cancer, where their mutation frequency varies according to the distinct RAS isoforms and tumour types. Despite occurring more prevalent in malignant tumours, RAS mutations were also observed in few benign tumours. Pituitary adenomas are examples of benign tumours which vary in size and aggressiveness. The present study was performed to investigate, via liquid biopsy and tissue analysis, the presence of K-RAS mutations in a pituitary macroadenoma. METHODS: Molecular analysis was performed to investigate K-RAS mutations using the droplet digital PCR (ddPCR) method by evaluating both plasma (liquid biopsy) and the solid tumour of a patient diagnosed with a giant clinically non-functioning pituitary tumour. RESULTS: The patient underwent surgical resection due to visual loss, and the histopathological analysis showed a gonadotrophic pituitary macroadenoma. The molecular analysis revealed the presence of mutant K-RAS both in the plasma and in the tumour tissue which, to our knowledge, has not been previously reported in the literature. CONCLUSION: Our findings highlight the exceptional capacity of the digital PCR in detecting low frequency mutations (below 1%), since we detected, for the first time, K-RAS mutations in pituitary macroadenoma. The potential impact of K-RAS mutations in these tumours should be further investigated.
Asunto(s)
Adenoma , Neoplasias Hipofisarias , Proteínas Proto-Oncogénicas p21(ras) , Adenoma/genética , Genes ras , Humanos , Mutación/genética , Neoplasias Hipofisarias/genética , Reacción en Cadena de la Polimerasa , Proteínas Proto-Oncogénicas p21(ras)/genéticaRESUMEN
Objetivo: determinar o perfil epidemiológico e clínico de pacientes com acromegalia no Estado de Alagoas. Métodos: trata-se de um estudo descritivo, observacional, que foi realizado na Farmácia de Medicamentos Excepcionais do Estado de Alagoas, abrangendo 12 pacientes com acromegalia dessa instituição, correspondendo a 85% dos acromegálicos do Estado de Alagoas. Resultados: dos 12 pacientes estudados, a média de idade foi de 50,8 +/- 12,1 anos e, no momento do diagnóstico, uma média de idade de 44,5 +/- 11,6 anos. Foram realizados exames de imagem em 91,6% dos pacientes, em que se evidenciou macroademona hipofisário no momento do diagnóstico. A octreotida foi o medicamento principal, utilizado em 92% dos pacientes. Conclusão: O estudo corrobora, em boa parte dos dados, a literatura atual e revela a necessidade de maior precocidade de diagnóstico e início de terapêutica adequada, evitando o surgimento de comorbidades e complicações típicas da doença. (AU)
Objective: to determine the clinical and epidemiological profile of patients with acromegaly in the state of Alagoas. Method: This is a descriptive and observational study, which was held at the Exceptional Drug Pharmacy of the State of Alagoas. This study included 12 patients with acromegaly of this institution, corresponding to 85% of acromegaly in the State of Alagoas. Results: The mean age of the patients was 50.8 +/- 12.1 year and the mean age at diagnosis was 44.5 +/- 11, 6 years. Medical imaging exams were performed in 91.7% of patients, showing pituitary macroadenoma at the moment of diagnosis. Octreotide was the main medication, used in 91,7% of patients. Conclusion: The study corroborates, in most of the data, the current literature and reveals the need for earlier diagnosis and appropriate therapy, which avoids the appearance of typical comorbidities and complications of this disease. (AU)
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Acromegalia , Hormona del Crecimiento , Adenoma Hipofisario Secretor de ACTHRESUMEN
Antecedentes: la apoplejía hipofisaria es un síndrome clínico agudo, potencialmente fatal, que acontece como complicación de un adenoma hipofisario; presenta una complejidad diagnóstica, y puede imitar numerosos procesos intracraneales. Objetivo: realizar la revisión bibliográfica del tema, y describir dos casos clínicos, dada la poca frecuencia de presentación de la enfermedad y su complejidad diagnóstica. Desarrollo: caso 1, paciente masculino, de 52 años con antecedentes patológicos de hipertensión arterial y macroadenoma hipofisario no funcionante. Durante su seguimiento por Neurocirugía se detectó hiperprolactinemia, por lo que se inició tratamiento con bromocriptina a dosis progresiva, lo cual desencadenó cuadro agudo no definido. Tres meses después presentó nuevo cuadro agudo, que se diagnosticó como apoplejía hipofisaria; caso 2, paciente masculino, de 49 años, con antecedente de hipertensión arterial con tratamiento irregular. Fue ingresado en servicio de terapia intermedia con diagnóstico presuntivo de meningoencefalitis, y por estudios complementarios se llega al diagnóstico de apoplejía hipofisaria. Ambos casos llevaron tratamiento quirúrgico, con evolución favorable. Conclusiones: la apoplejía hipofisaria es una enfermedad que se presenta con poca frecuencia, por lo tanto, es importante la sospecha diagnóstica para una temprana identificación y tratamiento de la enfermedad(AU)
Background: pituitary apoplexy is an acute clinical syndrome, potentially fatal, that occurs as a complication of a pituitary adenoma. It presents a diagnostic complexity, and it can mimic numerous intracranial processes. Objective: to carry out the bibliographic review of the subject, and to describe two clinical cases given the infrequency of presentation of the disease and its diagnostic complexity. Development: case 1, male patient, 52 years old with pathological history of arterial hypertension and non-functioning pituitary macroadenoma. During follow-up by Neurosurgery, hyperprolactinemia was detected, so treatment with bromocriptine was started with a progressive dose, which triggered undefined acute symptoms. Three months later he presented new acute symptoms, which was diagnosed as pituitary apoplexy. Case 2, male patient, 49 years old, with a history of arterial hypertension under irregular treatment. He was admitted in the intermediate therapy service with a presumptive diagnosis of meningoencephalitis, and by complementary studies the diagnosis of pituitary apoplexy was reached. Both cases led to surgical treatment, with favorable evolution. Conclusions: pituitary apoplexy is a disease that occurs infrequently, therefore, diagnostic suspicion is important for early identification and treatment of the disease(AU)
Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Bibliografías como Asunto , Apoplejia Hipofisaria/cirugía , Apoplejia Hipofisaria/diagnóstico , Hiperprolactinemia , Literatura de Revisión como AsuntoRESUMEN
Antecedentes: la apoplejía hipofisaria es un síndrome clínico agudo, potencialmente fatal, que acontece como complicación de un adenoma hipofisario; presenta una complejidad diagnóstica, y puede imitar numerosos procesos intracraneales. Objetivo: realizar la revisión bibliográfica del tema, y describir dos casos clínicos, dada la poca frecuencia de presentación de la enfermedad y su complejidad diagnóstica. Desarrollo: caso 1, paciente masculino, de 52 años con antecedentes patológicos de hipertensión arterial y macroadenoma hipofisario no funcionante. Durante su seguimiento por Neurocirugía se detectó hiperprolactinemia, por lo que se inició tratamiento con bromocriptina a dosis progresiva, lo cual desencadenó cuadro agudo no definido. Tres meses después presentó nuevo cuadro agudo, que se diagnosticó como apoplejía hipofisaria; caso 2, paciente masculino, de 49 años, con antecedente de hipertensión arterial con tratamiento irregular. Fue ingresado en servicio de terapia intermedia con diagnóstico presuntivo de meningoencefalitis, y por estudios complementarios se llega al diagnóstico de apoplejía hipofisaria. Ambos casos llevaron tratamiento quirúrgico, con evolución favorable. Conclusiones: la apoplejía hipofisaria es una enfermedad que se presenta con poca frecuencia, por lo tanto, es importante la sospecha diagnóstica para una temprana identificación y tratamiento de la enfermedad(AU)
Background: pituitary apoplexy is an acute clinical syndrome, potentially fatal, that occurs as a complication of a pituitary adenoma. It presents a diagnostic complexity, and it can mimic numerous intracranial processes. Objective: to carry out the bibliographic review of the subject, and to describe two clinical cases given the infrequency of presentation of the disease and its diagnostic complexity. Development: case 1, male patient, 52 years old with pathological history of arterial hypertension and non-functioning pituitary macroadenoma. During follow-up by Neurosurgery, hyperprolactinemia was detected, so treatment with bromocriptine was started with a progressive dose, which triggered undefined acute symptoms. Three months later he presented new acute symptoms, which was diagnosed as pituitary apoplexy. Case 2, male patient, 49 years old, with a history of arterial hypertension under irregular treatment. He was admitted in the intermediate therapy service with a presumptive diagnosis of meningoencephalitis, and by complementary studies the diagnosis of pituitary apoplexy was reached. Both cases led to surgical treatment, with favorable evolution. Conclusions: pituitary apoplexy is a disease that occurs infrequently, therefore, diagnostic suspicion is important for early identification and treatment of the disease(AU)
Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Bibliografías como Asunto , Apoplejia Hipofisaria/cirugía , Apoplejia Hipofisaria/diagnóstico , Hiperprolactinemia/tratamiento farmacológico , Literatura de Revisión como AsuntoAsunto(s)
Fármacos del Sistema Nervioso Central/uso terapéutico , Agonistas de Dopamina/efectos adversos , Fructosa/análogos & derivados , Trastornos Migrañosos/tratamiento farmacológico , Trastornos Migrañosos/etiología , Neoplasias Hipofisarias/complicaciones , Prolactinoma/complicaciones , Encéfalo/diagnóstico por imagen , Cabergolina , Niño , Agonistas de Dopamina/uso terapéutico , Ergolinas/efectos adversos , Ergolinas/uso terapéutico , Fructosa/uso terapéutico , Humanos , Masculino , Trastornos Migrañosos/diagnóstico por imagen , Trastornos Migrañosos/metabolismo , Neoplasias Hipofisarias/diagnóstico por imagen , Neoplasias Hipofisarias/tratamiento farmacológico , Neoplasias Hipofisarias/cirugía , Prolactinoma/diagnóstico por imagen , Prolactinoma/tratamiento farmacológico , Prolactinoma/cirugía , TopiramatoRESUMEN
PURPOSE: Pituitary macroadenomas (MACs) represent 10-30 % of Cushing's disease (CD) cases. The aim of this study was to report the clinical, laboratorial and imaging features and postsurgical outcomes of microadenoma (MIC) and MAC patients. METHODS: Retrospective study with 317 CD patients (median 32 years old, range 9-71 years) admitted between 1990 and 2014, 74 (23.3 %) of whom had MAC. RESULTS: Hirsutism, plethora facial, muscular weakness and muscular atrophy were more frequent in the MIC patients. Nephrolithiasis, osteopenia, hyperprolactinaemia and galactorrhoea were more prevalent in MAC patients. The morning serum cortisol (Fs), nocturnal salivary cortisol (NSC), nocturnal Fs (Fs 2400 h), low- and high-dose dexamethasone suppression test results and CRH and desmopressin test results were similar between the subgroups. MIC patients showed higher urinary cortisol at 24 h (UC), and MAC patients presented higher ACTH levels but lower Fs/ACTH, Fs 2400 h/ACTH, NSC/ACTH and UC/ACTH ratios. There were negative correlations of tumour size with Fs/ACTH, Fs 2400 h/ACTH, NSC/ACTH and UC/ACTH ratios. Overall, the postsurgical remission and recurrence rates were similar between MIC and MAC. However, patients in remission (MIC + MAC) showed smaller tumour diameters and a lower prevalence of invasion and extension on MRI. CONCLUSIONS: Despite exhibiting higher plasma ACTH levels, CD patients with MAC presented lower cortisol/ACTH ratios than did patients with MIC, with a negative correlation between tumour size and cortisol/ACTH ratios. The overall postsurgical remission and recurrence rates were similar between MIC and MAC patients, with those with larger and/or invasive tumours showing a lower remission rate.
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Adenoma/sangre , Hormona Adrenocorticotrópica/sangre , Hidrocortisona/sangre , Hipersecreción de la Hormona Adrenocorticotrópica Pituitaria (HACT)/complicaciones , Neoplasias Hipofisarias/sangre , Adenoma/etiología , Adenoma/patología , Adolescente , Adulto , Anciano , Niño , Ensayo de Inmunoadsorción Enzimática , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Hipersecreción de la Hormona Adrenocorticotrópica Pituitaria (HACT)/fisiopatología , Neoplasias Hipofisarias/etiología , Neoplasias Hipofisarias/patología , Estudios Retrospectivos , Adulto JovenRESUMEN
Objetivo: Resaltar las indicaciones y detalles técnicos en el abordaje fronto-órbito-cigomático en dos piezas en base a nuestra experiencia quirúrgica. Material y método: se realizó un estudio descriptivo retrospectivo analizando las historias clínicas de 18 pacientes intervenidos quirúrgicamente a través de un abordaje fronto-orbito-cigomático en dos piezas, entre junio de 2010 y junio de 2013, en nuestro servicio. Se obtuvieron datos epidemiológicos y los relacionados a las diferentes patologías alcanzadas mediante esta vía. Resultados: durante dicho período se realizaron 18 craneotomías fronto-orbito-cigomática en dos piezas. Fueron intervenidos 11 pacientes con aneurismas cerebrales, de los cuales 4 fueron aneurismas del segmento oftálmico de la arteria carótida interna, 3 de bifurcación alta de arteria carótida interna, 2 de la arteria comunicante anterior con orientación cefálica y 2 de la bifurcación de la arteria basilar. Así mismo, se logró la exéresis de 7 lesiones tumorales, de los cuales 4 fueron adenomas de hipófisis, 2 craneofaringioma y 1 glioma hipotalámico. Se estandarizaron los siguientes pasos para la realización del abordaje fronto-órbito-cigomático en dos piezas, compuesto por una craneotomía fronto-temporo-esfenoidal y una segunda pieza compuesta por techo orbitario y arcada cigomática. Conclusiones: dicho abordaje representa una importante vía de acceso para lesiones que comprometen región selar, paraselar y patología vascular del polígono de Willis con menor retracción cerebral y mayor exposición.
Purpose: To highlight the indications and surgical details in the two pieces fronto-orbito-zigomatic approach based on our surgical experience.Material and Methods: a descriptive retrospective study was done by the analysis of 18 medical histories of patients operated by a two pieces fronto-orbito-zigomatic approach, between June 2010-2013 in our sevice. Epidemiological facts and those related to the different pathology approaches were obtainedResults: 18 two pieces fronto-orbito-zigomatic craniotomies were performed during the analized period. 11 patients had cerebral aneurisms, 4 of them arised from the oftalmic segment of the internal carotid artery, 3 from a high bifurcation of the internal carotid artery, 2 from the basilar artery´s bifurcation. We also performed the resection of 7 tumors: 4 pituitary adenomas, 2 craniopharyngiomas and 1 hipotalamic glioma. The following steps were standarized to perform the two pieces fronto-orbito-zigomatic approach: first a fronto-temporo-esphenoidal craniotomy and then a second piece composed by the orbital roof and the zigomatic arc.Conclusions: this approach represents an important route of access to lesions that involve both sellar and parasellar regions and vascular pathology from the Circle of Willis with less brain retraction and more exposure.
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Humanos , Adenoma , Craneofaringioma , Aneurisma IntracranealRESUMEN
Objetivo: Relacionar las imágenes de los adenomas obtenidos mediante RM para su mejor caracterización mediante un protocolo de alta resolución. Material y Métodos: Se analizaron retrospectivamente 86 RM de la región selar realizadas en el Hospital Obrero N°1, y se las relacionó con el tipo de realce post contraste paramagnético y signos de compresión. Se estudiaron 72 mujeres y 14 hombres, con un promedio de 43 años. Las exploraciones se realizaron utilizando un Magneto Signa 1,5 T, con cortes sagitales y coronales ponderados en T1 sin y con contraste paramagnético. Resultados: El 67,5% (58 pacientes) presentó adenoma hipofisiario. De estos el 51.1% fueron tipo microadenoma y el 48.3% macroadenoma. El 53,3% de los microadenomas y el 14,3% de los macroadenomas presentaban hiperprolactinemia. El 100% de los macroadenomas presentaron realce heterogéneo tras la administración del medio de contraste. Los microadenomas presentaron realce homogéneo en el 76,6%. El 85,7% de los macroadenomas y el 16,7% de los microadenomas presentaron obliteración de la cisterna supraselar. El 85,7% de los macroadenomas y en el 10% de los microadenomas, produjeron desplazamiento del quiasma óptico. El 64,3% de los macroadenomas y en el 20,7% de los microadenomas, invadieron el seno cavernoso y desviaron el tallo hipofisiario. Conclusión: La RM es el método imagenológico de elección para el estudio de la hipófisis gracias a su caracterización anatómica y funcional, especialmente cuando se utiliza gadolinio.
Objective: To relate the images of adenomas obtained by MRI for further characterízation using a high-resolution protocol Material and Methods: We analyzed 86 MRI retrospectivamen the sellar region in the Hospital Obrero No. 1 and are related to the type of contrast enhancement after paramagnetic and signs of compression. We studied 72 women and 14 men with an average of 43 years gave. The scans were performed using Magneto do a 1.5 T Signa, with cut-weighted sagittal and coronal T1 with and without paramagnetic contrast. Results: 67.5% (58 patients) had pituitary adenoma. Of these the kind microadenomas were 51.1% and 48.3% macroadenoma. 53.3% of microadenomas and 14.3% of macroadenomas had hyperprolactinemia. 100% of the macroadenomas showed heterogeneous enhancement after administration of contrast medium. The microadenomas showed homogeneous enhancement in 76.6%. The 85.7% of macroadenomas and 16.7% of microadenomas showed obliteration of the tank supraselar. The 85.7% of macroadenomas and10% of microadenomas, produced displacement of optic chiasm. 64.3% of macroadenomas and 20.7% of microadenomas, invaded the cavernous sinus and diverted the pituitary stalk. Conclusión: MRI is the imaging method of choicefor the study of the pituitary due to its characterisation in anatomy and function especially when using gadolinium.
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Espectroscopía de Resonancia MagnéticaRESUMEN
La prevalencia de los tumores hipofisarios en la tercera edad se encuentra en aumento en relación a la mayor expectativa de vida de la población general. Nosotros evaluamos retrospectivamente, 36 pacientes de la tercera edad, con media de 71 años, (65 -85), 20 de ellos varones, con tumores hipofisarios. El adenoma hipofisario no funcionante (ANF) se observó en el 64% de los casos, seguido por la acromegalia, el prolactinoma, y por un caso de enfermedad de Cushing. Se suman 3 casos de hipofisitis linfocitiaria, craneofaringioma y quiste de la bolsa de Rathke respectivamente. El 89% de los tumores fueron macroadenomas y la forma de presentación más frecuente, los trastornos visuales, seguidos por el hallazgo incidental y la hipersecreción hormonal. En la evaluación bioquímica la mayoría presentaba diferentes grados de hipopituitarismo, y los pacientes con macroprolactinoma tenían PRL > 800 ng/ml. Se operaron 21 pacientes (58%), 9 recibieron radioterapia. En 3 pacientes que no recibieron ningún tipo de tratamiento hubo modificación del tamaño del adenoma (2 aumentaron y 1 disminuyó) durante el seguimiento de 3.6 años. Se indicó tratamiento médico en aquellos que persistieron con la enfermedad o como tratamiento primario si las comorbilidades imposibilitaban la cirugía. No hubo mejoría de la función hipofisaria luego de la cirugía y el 62% agregaron uno o más déficit, el campo visual mejoró en el 67%. La diabetes insípida definitiva como complicación sólo se observó en dos pacientes y hubo un caso de fístula y meningitis. En conclusión, nosotros reportamos una tasa menor a la bibliografía mundial en la prevalencia de adenomas hipofisarios no funcionantes y similar para el resto, con una mayor tasa de presentación incidental. La cirugía no tuvo más complicaciones por la edad de los pacientes, el seguimiento clínico y con imágenes de aquellos que no se operan y/o el tratamiento médico puede ser una opción en este grupo etario.
World population has been growing and aging; and pituitary tumors prevalence among the elderly has also increased, summing up to 10-15% of all pituitary tumors. Thirty six elderly patients with pituitary tumors, 20 of them male, were evaluated restrospectively (average age 71 years, range 65-85). A non-functioning adenoma (NFA) prevalence was reported in 64% of the cases, followed by acromegaly, prolactinoma and one each of Cushing´s disease, lymphocytic hypophysitis, craniopharyngioma, and Rathke's pouch cyst. Macroadenomas accounted for 89% of tumors and the most frequent presentation was through visual disorders symptoms followed by incidental finding and hormone hypersecretion. During biochemical evaluation, most patients presented different degrees of hypopituitarism, and patients with macroprolactinoma had PRL levels>800ng/ml. Twenty one patients (58%) underwent surgery, while 9 underwent radiotherapy. During the 3.6 years follow-up, changes in adenoma size were reported in 3 patients who received no treatment (in 2 cases size increased, whereas in another it decreased). Medical treatment was indicated for those whose pathology persisted or as primary treatment if comorbilities prevented from performing a surgery. No pituitary function improvement was detected after surgery and the appearance of one or more deficiency was noticed in 62% of patients, though visual field did improve in 67%. Definitive diabetes insipidus as a complication was only detected in two patients, and a case of fistula and meningitis was observed. As a conclusion, we reported a NFA prevalence rate smaller than that reported in the world literature, though similar for the other pituitary tumors, and a higher rate of incidental presentation. No complications attributable to patients' age group were detected during surgery. For elderly patients who do not undergo surgery, clinical and imaging follow-up and/or medical treatment could be an option of choice.
Asunto(s)
Humanos , Masculino , Femenino , Anciano , Anciano de 80 o más Años , Neoplasias Hipofisarias/epidemiología , Salud del Anciano , Neoplasias Hipofisarias/diagnóstico , Neoplasias Hipofisarias/terapiaRESUMEN
Paciente masculino de 57 años de edad, quien ingresó al Hospital Universitario de Los Andes, Mérida, Venezuela, por disminución de agudeza visual, astenia, cefalea y disminución de la libido. Al examen físico se encontraron signos de hipogonadismo, así como defectos de campo visual bilateral. Se realizó resonancia magnética contrastada con énfasis en área selar, donde se evidenció un macroadenoma de hipófisis, el cual resultó ser no productor por pruebas de función hipofisaria. El tratamiento quirúrgico constituyó la opción terapéutica en este caso, dado que la reducción de la masa tumoral brinda resolución de la cefalea, así como mejoría en los defectos de campo visual y en la mayoría de los casos, en la función hipofisaria...
A 57 years old male patient was admitted to the Hospital Universitario de Los Andes, Mérida, Venezuela because of decreased visual acuity, asthenia, headache and decreased libido. The physical examination revealed clinical findings of hypogonadism and visual dysfunction. A contrastenhanced MR of the sellar area showed a pituitary macroadenoma. The hormonal assays showed it was non-functioning. Surgery was the treatment in this case because the mass reduction brings resolution of headaches and short term improvement of visual field defects...
Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Adenoma/cirugía , Adenoma/diagnóstico , Hipogonadismo/diagnóstico , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/diagnóstico , Neoplasias Hipofisarias/terapia , Medicina Interna , Oncología MédicaRESUMEN
We report a 45 years old female that consulted for amenorrhea and galactorrhea after discontinuing oral contraceptives, that she used for several years. The patient had hyperprolactinemia, a hypogonadotrophic hypogonadism and a primary hypothyroidism. Sella turcica magnetic resonance showed a pituitary macroadenoma with a diameter of 23 mm and supraselar extension. The patient was subjected to a transsphenoidal excision of the tumor with good clinical and surgical results. Therefore radiotherapy was not used for the treatment of the tumor.
Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Adenoma/cirugía , Adenoma/radioterapia , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/radioterapia , Radioterapia Adyuvante , Toma de Decisiones , Selección de Paciente , Cuidados Posoperatorios , Procedimientos Neuroquirúrgicos/métodos , Recurrencia Local de Neoplasia/prevención & controlRESUMEN
Objetivo: Presentar el caso poco frecuente de una adolescente con un macroadenoma hipofisario no funcionante que le produjo un hipopituitarismo. Se hace una revisión de la literatura. Caso Clínico: Adolescente femenina de 16 años 2 meses de edad quien presenta poco progreso en talla y ausencia de caracteres sexuales secundarios; refiere concomitantemente cefaleas ocasionales. Al examen físico presenta talla y peso por debajo del pc 3, velocidad de crecimiento 2,8 cm/año, cubitus valgus, paladar ojival, cuarto metacarpiano corto, no tiromegalia. Genitales, mamas, vello axilar y púbico Tanner I. Se hace diagnóstico de talla baja patológica y retraso puberal y se indican exámenes de paraclínicos. Los resultados de laboratorio muestran un déficit de hormona de crecimiento (GH) y un hipocortisolismo, con función tiroidea conservada. Edad ósea de 11 años para una edad cronológica de 15 años. Cariotipo 46,XX. Rx lateral de cráneo muestra silla turca amplia y excavada. Us pélvico: útero en anteversion, central, con longitud de 20 mm; no se visualizan línea endometrial ni ovarios. La RMN muestra el piso de la región selar deformado, excavado, con imagen compatible con LOE de contornos más o menos definidos con señal intermedia baja y dishomogénea en T1, con realce hipertenso dishomogéneo tras la administración del medio de contraste, de aproximadamente 2,9 x 1,36 cm. de diámetro, con compromiso de los elementos supraselares, ejerciendo efecto compresivo sobre el infundíbulo el cual deforma el quiasma óptico. Se hace diagnóstico de Panhipopituitarismo secundario a Macroadenoma Hipofisario no Secretante, con Déficit de GH, Hipogonadismo Hipogonadotropo e Insuficiencia Suprarrenal Secundaria (Déficit de ACTH). Se indica tratamiento con hidrocortisona por vía oral. Se refiere para resolución quirúrgica por vía transesfenoidal ya que en nuestro centro no se realiza. Se desconoce el reporte histológico del tumor y la evolución de la paciente. Conclusiones: Los adenomas hipofisarios representan menos del 2-3% de todos los tumores intracraneales y sus manifestaciones clínicas dependen de la suma de un efecto masa, que causa alteraciones neurológicas, y la afectación de la secreción hormonal, ya sea por exceso o por defecto. Es importante realizar un diagnóstico temprano, así como un tratamiento efectivo y seguimiento a largo plazo.
Objective: To present the uncommon case of a teenager with a non-functioning pituitary macroadenoma that resulted in a hypopituitarism. A literature review is done. Case Report: Female adolescent of 16 years 2 months of age who presents little progress in stature and absence of secondary sexual characters; concomitantly refers occasional headaches. Physical examination: height and weight below the 3 pc, growth velocity 2.8 cm/year, cubitus valgus, ojival palate, short fourth metacarpal, no thyroid enlargement. Genitals, breasts, axillary and pubic hair, Tanner I. The diagnosis of pathological short stature and delayed puberty is done and paraclinical examinations are indicated. Laboratory results show a deficit of growth hormone (GH) and a hypocortisolism with preserved thyroid function. Bone age of 11 years for a chronological age of 15 years. Karyotype 46, XX. Rx of the skull shows a wide and excavated sella turcica. Pelvic Us: central uterus, in anteversion, 20 mm in length and the endometrium and ovaries were no visible. NMR shows the floor of the sellar region warped, carved, with a image compatible with tumour of defined contours, with low-intermediate and dishomogenea signal on T1, with hypertensive and heterogeneou enhancement after administration of contrast medium, approximately 2.9 x 1.36 cm. in diameter, with involvement of the supraselares elements exerting compressive effect on the infundibulum, which deforms the optic chiasm. The diagnosis of panhypopituitarism secondary to pituitary non functioning macroadenoma is done, with GH deficiency, hypogonadotropic hypogonadism and secondary adrenal insufficiency (ACTH deficiency). Treatment with oral hydrocortisone is indicated. The patient is referred for resolution by transsphenoidal surgery. The histological report of the tumor and the evolution of the patient are unknown. Conclusions: Pituitary adenomas account for less than 2-3% of all intracranial tumours, and their clinical manifestations depend on the sum of a mass effect, which causes neurological disorders, and the disruption of hormonal secretion, either up or down. It is important to make an early diagnosis, an effective treatment and long-term monitoring.