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2.
World Neurosurg ; 133: e241-e251, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31505289

RESUMO

BACKGROUND: Cystic sellar masses (CSMs) pose diagnostic and therapeutic challenges associated with subtotal cyst wall resection, cerebrospinal fluid (CSF) leak repair, and disease recurrence. Current magnetic resonance imaging (MRI) interpretation often cannot reliably differentiate CSMs, mandating adaptable intraoperative strategies. We reviewed our diagnostic and therapeutic experience after endoscopic endonasal approaches (EEAs) for CSMs. METHODS: A retrospective record review of patients with CSM managed via EEA at the University of Southern California from 2011 to 2018 was conducted. Patient demographics, preoperative characteristics, surgical details, pathologic findings, and postoperative outcomes were assessed. RESULTS: Analysis included 47 patients (mean age, 43.2 years); of these, 78.7% were women. Preoperative symptoms included headache (76.6%) and vision loss (42.6%). Histologically verified sellar pathology included 27 Rathke cleft cysts (RCCs) (57.4%), 17 cystic pituitary adenomas (CPAs) (36.2%), 2 arachnoid cysts (4.3%), and 1 xanthogranuloma (2.1%). Twelve patients (70.6%) with CPAs underwent complete resection and 5 (29.4%) underwent subtotal resection. All 27 patients with RCC and 2 patients with arachnoid cyst underwent complete fenestration and drainage. One xanthogranuloma was completely resected. There were 14 intraoperative (29.8%) and 4 postoperative CSF leaks (8.5%). Headaches, vision, and endocrinopathy improved in 69.2%, 80.0%, and 33.3% of patients with CPA and 73.9%, 71.4%, and 40.9% of patients with RCC, respectively. There were 2 RCC recurrences and 1 CPA recurrence over the follow-up period. CONCLUSIONS: Surgeons must prepare for versatile management strategies of CSMs based on pretest probability associated with MRI and intraoperative findings. Outcomes after EEA for CSMs show low complication profiles and excellent rates of headache and visual improvement, albeit lower rates of endocrine normalization.


Assuntos
Cistos do Sistema Nervoso Central/cirurgia , Cirurgia Endoscópica por Orifício Natural/métodos , Neuroimagem/métodos , Sela Túrcica , Adenoma/complicações , Adenoma/diagnóstico , Adenoma/diagnóstico por imagem , Adenoma/cirurgia , Adulto , Cistos do Sistema Nervoso Central/complicações , Cistos do Sistema Nervoso Central/diagnóstico , Cistos do Sistema Nervoso Central/diagnóstico por imagem , Vazamento de Líquido Cefalorraquidiano/etiologia , Drenagem , Fadiga/etiologia , Feminino , Cefaleia/etiologia , Humanos , Hipopituitarismo/etiologia , Complicações Intraoperatórias/etiologia , Masculino , Pessoa de Meia-Idade , Cavidade Nasal , Neoplasias Hipofisárias/complicações , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/diagnóstico por imagem , Neoplasias Hipofisárias/cirurgia , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Sela Túrcica/diagnóstico por imagem , Sela Túrcica/cirurgia , Transtornos da Visão/etiologia , Xantomatose/complicações , Xantomatose/diagnóstico , Xantomatose/diagnóstico por imagem , Xantomatose/cirurgia
3.
Zhonghua Yi Xue Za Zhi ; 99(48): 3803-3807, 2019 Dec 24.
Artigo em Chinês | MEDLINE | ID: mdl-31874518

RESUMO

Objective: To analyze the causes of complications after endoscopic transsphenoidal pituitary adenoma resection and summarize the corresponding prevention and treatment measures. Methods: The pathological data of 120 patients with pituitary adenoma in hospital were analyzed retrospectively. All patients were treated surgically, five kinds of common complications were collected, the risk factors of complications were statistically analyzed, the causes of postoperative complications were determined, and then the specific prevention and treatment strategies were summarized. Results: Long operation time (P<0.05), hypopituitarism before operation (P<0.01) and large tumor (P<0.01) were the main inducing factors of CSF rhinorrhea after operation; long operation time (OR=1.394, P<0.01), lumbar cistern puncture (OR=1.531, P<0.01) and CSF rhinorrhea after operation (OR=3.861, P<0.01) were the independent risk factors of infection; Age, large tumor and preoperative hypophysis are the main factors of postoperative hypophysis; Postoperative hypophysis and intraoperative cerebrospinal fluid rhinorrhea are the main factors of postoperative diabetes insipidus; Postoperative hypophysis and giant adenoma are the main factors of postoperative hyponatremia. Conclusion: Endoscopic transnasal transsphenoidal resection of pituitary adenoma is the most minimally invasive method at present. There are still risk factors for postoperative complications. Targeted prevention of risk factors for complications and corresponding interventions can effectively reduce the incidence of postoperative complications of pituitary adenoma.


Assuntos
Neoplasias Hipofisárias , Humanos , Neuroendoscópios , Hipófise , Neoplasias Hipofisárias/diagnóstico , Complicações Pós-Operatórias , Estudos Retrospectivos , Resultado do Tratamento
4.
Medicina (B Aires) ; 79(5): 419-423, 2019.
Artigo em Espanhol | MEDLINE | ID: mdl-31671397

RESUMO

We present the case of a patient who, during studies for fertility and subsequent pregnancy, showed an altered thyroid profile with elevated levels of free T4 and normal TSH. After ruling out a thyrotropic adenoma and in the absence of clinical symptoms of hyperthyroidism, the possibility of analytical interference in the immunoassays used to measure hormones was investigated. Interferences caused by heterophile antibodies, macro TSH, anti-thyroid antibodies, biotin, and to a lesser extent anti-streptavidin and anti-ruthenium antibodies have been described. The analysis of the patient was carried out in a self-analyzer whose platform uses the streptavidin-biotin system that is very susceptible to several interferents. A proposed algorithm includes a series of simple tests to perform and interpret that allow detecting or ruling out the presence of interferents. Accordingly, a comparison was made with a different analytical platform (which does not use the streptavidin-biotin system), serial dilutions, precipitation with polyethylene glycol 6000 and treatment with microparticles coated with streptavidin. Results obtained confirmed the presence of anti-streptavidin antibodies in the patient's serum. In the case of disagreements between clinical manifestations and laboratory results, the possibility of methodological interferences should be investigated in order to avoid the potential iatrogenic risk involved in an erroneous biochemical interpretation.


Assuntos
Adenoma/diagnóstico , Anticorpos Anti-Idiotípicos/imunologia , Hipertireoidismo/diagnóstico , Neoplasias Hipofisárias/diagnóstico , Estreptavidina/imunologia , Adenoma/imunologia , Adulto , Erros de Diagnóstico , Feminino , Humanos , Hipertireoidismo/imunologia , Neoplasias Hipofisárias/imunologia , Gravidez , Tireotropina/sangue , Tiroxina/sangue , Tri-Iodotironina/sangue
5.
Curr Urol Rep ; 20(11): 78, 2019 Nov 16.
Artigo em Inglês | MEDLINE | ID: mdl-31734886

RESUMO

PURPOSE OF REVIEW: Hypogonadism is a common endocrine dysfunction. This review focuses on the most up-to-date guideline for evaluation of pituitary function among men presenting with signs and symptoms of hypogonadism. RECENT FINDINGS: The clinician must differentiate between primary (testicular) and secondary (pituitary-hypothalamic or central) hypogonadisms and be aware of adult-onset hypogonadism. If gonadotropins are low or inappropriately normal, the clinician must consider potential reversible causes in the hypothalamus-pituitary axis. Also, it is critical to understand the pitfalls of testosterone testing. When clinically indicated, evaluation of other pituitary hormone functions as well as pituitary magnetic resonance imaging may be necessary. Furthermore, it is essential to recognize that pituitary incidentalomas are common. Patients with microprolactinoma are more likely to present with symptoms of sexual dysfunction while those with macroprolactinoma are more likely to present with symptoms of mass effect. Some functional pituitary tumors respond to drug therapy while other nonfunctional tumors require surgical intervention. It is important for the clinician to understand the proper work-up of the hypogonadal patient with pituitary dysfunction and when necessary to refer to an endocrinologist or a neurosurgeon.


Assuntos
Hipogonadismo/etiologia , Hipófise/fisiopatologia , Neoplasias Hipofisárias/complicações , Neoplasias Hipofisárias/terapia , Prolactinoma/complicações , Testosterona/uso terapêutico , Gonadotropinas/sangue , Humanos , Imagem por Ressonância Magnética , Masculino , Doenças da Hipófise/complicações , Doenças da Hipófise/diagnóstico , Doenças da Hipófise/fisiopatologia , Neoplasias Hipofisárias/diagnóstico , Guias de Prática Clínica como Assunto , Prolactinoma/diagnóstico , Prolactinoma/tratamento farmacológico , Disfunções Sexuais Fisiológicas/etiologia , Doenças Testiculares/sangue , Doenças Testiculares/complicações , Doenças Testiculares/diagnóstico , Testosterona/sangue
6.
Pan Afr Med J ; 33: 238, 2019.
Artigo em Francês | MEDLINE | ID: mdl-31692695

RESUMO

Multiple endocrine neoplasia type 1 (MEN1) is a rare disease, defined as a tumor developing in at least two endocrine glands including the anterior pituitary gland, the parathyroid glands and the duodenopancreatic endocrine tissue. This disorder, inherited in an autosomal dominant pattern, is caused by mutations in the MEN1 gene encoding the tumor suppressor menin and located on chromosome 11q13. However, sporadic cases account for 8-14%. The first endocrine lesion may be solitary in approximately 75% of cases. However, all major alterations can be inaugural. We here report a case of multiple endocrine neoplasia type 1 revealed by aggressive somatoprolactinic pituitary adenoma which didn't respond to conventional treatment. The detection of primary hyperparathyroidism as well as neuroendocrine tumor of the pancreas seven years later make this a very particular case. Therapeutic options are discussed within the multidisciplinary team specialized in endocrine diseases.


Assuntos
Neoplasia Endócrina Múltipla Tipo 1/diagnóstico , Tumores Neuroendócrinos/diagnóstico , Neoplasias Pancreáticas/diagnóstico , Neoplasias Hipofisárias/diagnóstico , Adenoma/diagnóstico , Adenoma/patologia , Adulto , Humanos , Hiperparatireoidismo Primário/diagnóstico , Masculino , Neoplasia Endócrina Múltipla Tipo 1/patologia , Tumores Neuroendócrinos/patologia , Neoplasias Pancreáticas/patologia , Neoplasias Hipofisárias/patologia , Prolactina/metabolismo
7.
Virchows Arch ; 475(6): 687-692, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31578606

RESUMO

The 2017 World Health Organization (WHO) classification proposes to type and subtype primary adenohypophyseal tumours according to their cell lineages with the aim to establish more uniform tumour groups. The definition of atypical adenoma was removed in favour of high-risk adenoma, and the assessment of proliferative activity and invasion was recommended to diagnose aggressive tumours. Recently, the International Pituitary Pathology Club proposed to replace adenoma with the term of pituitary neuroendocrine tumour (PitNET) to better reflect the similarities between adenohypophyseal and neuroendocrine tumours of other organs. The European Pituitary Pathology Group (EPPG) endorses this terminology and develops practical recommendations for standardised reports of PitNETs that are addressed to histo- and neuropathologists. This brief report presents the results of EPPG's consensus for the reporting of PitNETs and proposes a diagnostic algorithm.


Assuntos
Glucosiltransferases/metabolismo , Glicoproteínas/metabolismo , Tumores Neuroendócrinos/diagnóstico , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/patologia , Consenso , Humanos , Tumores Neuroendócrinos/patologia , Sistemas Neurossecretores/patologia , Organização Mundial da Saúde
8.
No Shinkei Geka ; 47(8): 901-907, 2019 Aug.
Artigo em Japonês | MEDLINE | ID: mdl-31477634

RESUMO

OBJECTIVES: Intracranial chordomas are thought to arise from remnants of the notochord and usually occur at the parasellar region. We present a case of a primary intrasellar chondroid chordoma in a patient who was initially diagnosed with a pituitary adenoma. CASE: A 77-year-old woman had a history of two surgeries for a tumor in the sella turcica(17 months after the 1st surgery). On initial MRI, the intrasellar mass showed low signal intensity on T1WI, very high signal intensity on T2WI, and inhomogeneous enhancement. On bone reconstructive CT, the sellar floor was thin, and no abnormalities were observed at the top of the clivus. Transsphenoidal surgery was performed. The pathological diagnosis was pituitary adenoma in both cases. Seventy-two months after the 1st(31 months after the 2nd)surgery, she developed right-sided oculomotor and abducent nerve palsies again. Since recurrence occurred during the relatively short period, the surgical specimens obtained from the 1st and 2nd surgeries were reexamined. Reexamination of the previously obtained specimen demonstrated areas of chondroid tissue that were embedded in a mucoid stroma and tumor cells that were composed of round or pleomorphic nuclei with vacuolated cytoplasm(physaliphorus cells)that were compatible with chondroid chordoma. The third surgery was performed. Postoperatively, her symptoms improved, and cyber knife therapy was administered for the residual part of the tumor. CONCLUSIONS: Although intrasellar chondroid chordomas are extremely rare, they should be considered in the differential diagnosis of tumors located in the sella turcica.


Assuntos
Adenoma , Cordoma , Neoplasias Hipofisárias , Adenoma/diagnóstico , Adenoma/cirurgia , Idoso , Cordoma/diagnóstico , Cordoma/cirurgia , Diagnóstico Diferencial , Feminino , Humanos , Recidiva Local de Neoplasia , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/cirurgia , Sela Túrcica/patologia
9.
Neurosurg Clin N Am ; 30(4): 457-463, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31471052

RESUMO

Pituitary apoplexy is a clinical condition with acute-onset headaches, vision loss, and/or pituitary dysfunction associated with a hemorrhagic or infarcted pituitary tumor or cyst. Treatment varies based on clinical presentation, although often urgent or emergent surgical resection is indicated. Conservative treatment strategies tend to be applied for more mild conditions of apoplexy. Overall outcomes may be similar in this less severe cohort. Acute-onset vision loss with apoplexy should be treated with urgent or emergent surgical evacuation of hematoma and resection of tumor when possible.


Assuntos
Apoplexia Hipofisária/diagnóstico , Apoplexia Hipofisária/cirurgia , Adenoma/complicações , Adenoma/diagnóstico , Adenoma/cirurgia , Humanos , Apoplexia Hipofisária/complicações , Neoplasias Hipofisárias/complicações , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/cirurgia
10.
Neurosurg Clin N Am ; 30(4): 465-471, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31471053

RESUMO

Pituitary adenomas are a rare but important central nervous system tumor in children. Because of differences in growth and development, the manifestations of pituitary adenomas in children may differ from those seen in adults. Unlike adult patients, the pediatric population more often presents with clinically secretory adenomas. Although medical management is first-line treatment of prolactinomas, transsphenoidal surgery is appropriate for most children with Cushing disease and gigantism. Although some pediatric patients present surgical challenges because of small anatomic dimensions or an incompletely developed sphenoid sinus, transsphenoidal surgery can be safely and effectively undertaken in most children, with low complication rates.


Assuntos
Adenoma/cirurgia , Neoplasias Hipofisárias/cirurgia , Adenoma/diagnóstico , Criança , Humanos , Hipersecreção Hipofisária de ACTH/complicações , Neoplasias Hipofisárias/diagnóstico , Prolactinoma/complicações , Osso Esfenoide/cirurgia , Resultado do Tratamento
11.
Pan Afr Med J ; 33: 92, 2019.
Artigo em Francês | MEDLINE | ID: mdl-31489070

RESUMO

Isolated involvement of the hypothalamic-pituitary axis in patients with sarcoidosis is rare. Only a few cases have been reported in the literature. We report the clinical case of a 50-years old female patient who had undergone total thyroidectomy followed by replacement therapy because of goitre 4 years before. She complained of chronic headaches persisting for 6 months and resistant to all appropriate therapies, with concomitant decrease in visual acuity. Clinical examination showed optochiasmatic syndrome with extensive visual field defect and common oculomotor nerve palsy in the right eye with ptosis. Brain MRI objectified pathological process to the sellar region with heterogeneous tissue signal intensity extending to the pituitary stalk with thickening in the latter. Preoperative hormonal assessment showed mild thyrotropic deficiency. The patient underwent wide transnasal endoscopic transsphenoidal resection of fibrous and little bleeding tumor. Postoperative outcome was marked by the occurrence of diabetes insipidus and CSF fistula two days after surgery. Patient's outcome was good under medical treatment with spinal drainage. Thoracoabdominal CT scan and cardiac ultrasound were performed which showed no other site of occurrence of sarcoidosis. Dose of converting enzyme was normal. The patient received corticosteroid therapy for the treatment of systemic disease. Neurosarcoidosis is a criterion of poor prognosis in a patient with sarcoidosis. Hypothalamopituitary involvement is rare resulting in complications which are more frequent than those of other neurological and systemic disorders. This requires multidisciplinary long term management.


Assuntos
Adenoma/diagnóstico , Doenças da Hipófise/diagnóstico , Neoplasias Hipofisárias/diagnóstico , Sarcoidose/diagnóstico , Doenças do Sistema Nervoso Central/diagnóstico , Doenças do Sistema Nervoso Central/patologia , Feminino , Humanos , Imagem por Ressonância Magnética , Doenças da Hipófise/patologia , Prognóstico , Sarcoidose/patologia , Tomografia Computadorizada por Raios X
13.
World Neurosurg ; 132: 1-3, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31442637

RESUMO

BACKGROUND: This report presents a rare presentation of a ganglioglioma in the sellar/suprasellar region. On the basis of the patient's presentation and imaging characteristics, the initial diagnosis was craniopharyngioma. While gangliogliomas are already rare brain tumors that are usually found in the frontal and temporal lobes of young patients, the presentation of this tumor in the sellar region is exceedingly rare. CASE DESCRIPTION: A 25-year-old male presented to the emergency department with headache, agitation, and combativeness. A head computed tomography scan showed a sellar/suprasellar mass with mixed solid and cystic components and peripheral calcifications. The mass compressed the third ventricle and cerebral aqueduct, resulting in obstructive hydrocephalus. The patient was intubated for decline in mental status and combativeness. A ventricular drain was placed emergently. A pituitary function panel did not show endocrine dysfunction. Magnetic resonance imaging showed a 3.6 cm × 4.2 cm solid mass in the sellar/suprasellar region with a cystic component. The mass displaced the adenohypophysis and extended into the prepontine and interpedicular cisterns. The clinical presentation and radiologic characteristics led to an initial diagnosis of craniopharyngioma. The patient underwent a right pterional craniotomy and transsylvian approach for resection of mass without complication, although a subtotal resection was achieved due to adherence of the tumor to optic nerves and carotid arteries. The resected specimen was diagnosed as ganglioglioma. CONCLUSIONS: This case is a reminder of how much the field of neurosurgery relies on imaging modalities but also emphasizes the importance of histopathology in the field of brain tumors.


Assuntos
Neoplasias Encefálicas/cirurgia , Ganglioglioma/cirurgia , Sela Túrcica/cirurgia , Adulto , Neoplasias Encefálicas/complicações , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/patologia , Craniofaringioma/diagnóstico , Craniotomia , Diagnóstico Diferencial , Ganglioglioma/complicações , Ganglioglioma/diagnóstico por imagem , Ganglioglioma/patologia , Humanos , Hidrocefalia/etiologia , Imagem por Ressonância Magnética , Masculino , Neoplasias Hipofisárias/diagnóstico , Sela Túrcica/diagnóstico por imagem , Sela Túrcica/patologia , Ventriculostomia
14.
Best Pract Res Clin Endocrinol Metab ; 33(2): 101299, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-31431397

RESUMO

In this paper, the currently used and well evaluated techniques for the surgery of pituitary tumors will be reviewed. Since the first surgical approaches to pituitary tumors more than 100 years have elapsed. Various surgical techniques have been developed, refined and standardized. Most of these tumors are to date treated via transsphenoidal approaches. Many pituitary adenomas, particularly, smaller, enclosed ones, can be completely excised and a selective adenomectomy is usually attempted. It leads to remission of hormonal oversecretion and also to recovery of pituitary function in many patients. The resection of pseudocapsule around the adenoma seems to improve the operative results further. Transcranial approaches, employing craniotomies, are still needed in some patients with pituitary adenomas and in many of those harbouring craniopharyngiomas. The operative techniques will be described and briefly commented. Moreover, the application and usefulness of several technical developments will be reviewed, such as the use of the endoscope, magnetic resonance imaging, fluorenscent dyes and neuronavigation. The use of the intraoperative Doppler probe, ultrasound and the value of intraoperative hormonal measurements will be briefly discussed. There is sufficient evidence that the best and optimal outcome in terms of tumor resection and correction of hormonal oversecretion as well as the lowest rate of complications are obtained in centers of excellence with sufficiently experienced, specialized surgeons and a high patient load.


Assuntos
Adenoma/cirurgia , Procedimentos Neurocirúrgicos/métodos , Neoplasias Hipofisárias/cirurgia , Adenoma/diagnóstico , Adenoma/epidemiologia , Humanos , Imagem por Ressonância Magnética , Microcirurgia/métodos , Procedimentos Neurocirúrgicos/normas , Hipófise/cirurgia , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/epidemiologia , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Resultado do Tratamento
15.
Best Pract Res Clin Endocrinol Metab ; 33(2): 101309, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-31405752

RESUMO

Pituitary diseases are rare conditions with severe chronic multiorgan and multisystemic morbidity requiring complex multidisciplinary treatment and usually life-long drug treatment. Most cases are caused by functioning or non-functioning pituitary adenoma. From the patient's perspective, the burden of disease is caused by the tumour itself and associated compression symptoms, interventions, hormone excess and deficiencies, systemic manifestations of these endocrine abnormalities and general psychosocial issues that can manifest in patients with a chronic condition. In this review, patient burden is classified according to classic endocrine syndromes, with burden at diagnosis and after long-term remission, and also within the framework of value-based health care and the conceptual model of wellbeing. The recently developed patient-reported outcome measurement tool that helps to evaluate burden of patients is also discussed.


Assuntos
Adenoma , Efeitos Psicossociais da Doença , Neoplasias Hipofisárias , Acromegalia/epidemiologia , Acromegalia/etiologia , Acromegalia/psicologia , Acromegalia/terapia , Adenoma/diagnóstico , Adenoma/epidemiologia , Adenoma/psicologia , Adenoma/terapia , Humanos , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/epidemiologia , Neoplasias Hipofisárias/psicologia , Neoplasias Hipofisárias/terapia , Qualidade de Vida
16.
Arch Endocrinol Metab ; 63(4): 385-393, 2019 Jul 29.
Artigo em Inglês | MEDLINE | ID: mdl-31365626

RESUMO

INTRODUCTION: Gigantism is a rare pediatric disease characterized by increased production of growth hormone (GH) before epiphyseal closure, that manifests clinically as tall stature, musculoskeletal abnormalities, and multiple comorbidities. MATERIALS AND METHODS: Case series of 6 male patients with gigantism evaluated at the Endocrinology Service of Hospital de San José (Bogotá, Colombia) between 2010 and 2016. RESULTS: All patients had macroadenomas and their mean final height was 2.01 m. The mean age at diagnosis was 16 years, and the most common symptoms were headache (66%) and hyperhidrosis (66%). All patients had acral changes, and one had visual impairment secondary to compression of the optic chiasm. All patients underwent surgery, and 5 (83%) required additional therapy for biochemical control, including radiotherapy (n = 4, 66%), somatostatin analogues (n = 5, 83%), cabergoline (n = 3, 50%), and pegvisomant (n = 2, 33%). Three patients (50%) achieved complete biochemical control, while 2 patients showed IGF-1 normalization with pegvisomant. Two patients were genetically related and presented a mutation in the aryl hydrocarbon receptor-interacting protein (AIP) gene (pathogenic variant, c.504G>A in exon 4, p.Trp168*), fulfilling the diagnostic criteria of familial isolated pituitary adenoma. CONCLUSIONS: This is the largest case series of patients with gigantism described to date in Colombia. Transsphenoidal surgery was the first-choice procedure, but additional pharmacological therapy was usually required. Mutations in the AIP gene should be considered in familial cases of GH-producing adenomas.


Assuntos
Adenoma/terapia , Gigantismo/terapia , Neoplasias Hipofisárias/terapia , Adenoma/diagnóstico , Adolescente , Colômbia , Seguimentos , Gigantismo/diagnóstico , Hormônio do Crescimento/sangue , Adenoma Hipofisário Secretor de Hormônio do Crescimento/genética , Humanos , Fator de Crescimento Insulin-Like I/análise , Peptídeos e Proteínas de Sinalização Intracelular/genética , Masculino , Mutação/genética , Linhagem , Neoplasias Hipofisárias/diagnóstico , Estudos Retrospectivos , Distribuição por Sexo , Resultado do Tratamento , Adulto Jovem
17.
World Neurosurg ; 132: e423-e429, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31470158

RESUMO

OBJECTIVE: To systematically review and analyze clinical, diagnostic, and management trends in sellar and parasellar brown tumors reported in existing literature. METHODS: In this systematic review, PubMed, Ovid MEDLINE, Scopus, and Google Scholar databases were searched for reported cases of sellar/parasellar brown tumors. Relevant titles and abstracts were screened in accordance to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses protocol. Articles meeting inclusion criteria were subjected to data extraction, summarization, and analysis. A rare case of parasellar brown tumor was also presented. RESULTS: Eight reports (including the current report) were eligible for inclusion. Mean patient age was 42.75 years. Reported symptoms included visual disturbances (n = 6), headache (n = 5), fatigue (n = 3), nausea/vomiting (n = 2), chest pain (n = 1), neck pain (n = 1), and dysphagia (n = 1). In cases where computed tomography findings were provided (n = 6), lesions were noted to be expansile and lytic. Lesions were hyperintense on T2-weighted magnetic resonance imaging (66.7%) and demonstrated contrast enhancement (83.3%). Histology unanimously showed multinucleated giant cells in a fibrovascular connective tissue stroma. Dramatic symptom resolution was noted in all patients who underwent resection of the sellar/parasellar brown tumor (n = 4; 50%). CONCLUSIONS: Sellar/parasellar brown tumors are a rare, tertiary manifestation of hyperparathyroidism and can be elusive to diagnose. Diagnosis requires a high index of clinical suspicion in addition to comprehensive biochemical testing, imaging, and histopathologic analysis. Surgical extirpation is favored in cases where the lesion is causing compressive symptoms, or if it is unresponsive to management of hyperparathyroidism.


Assuntos
Hiperparatireoidismo/complicações , Neoplasias Hipofisárias/patologia , Adolescente , Adulto , Feminino , Humanos , Hiperparatireoidismo/diagnóstico , Hiperparatireoidismo/cirurgia , Masculino , Pessoa de Meia-Idade , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/cirurgia , Adulto Jovem
18.
Ann Endocrinol (Paris) ; 80(4): 216-224, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31400861

RESUMO

TSH (thyroid-stimulating hormone)-secreting tumors are the rarest type of pituitary tumor. The objective of this study was to describe initial presentation and follow-up in patients presenting TSH-secreting tumors and to characterize the pathological features, based on a cohort of 20 patients treated in our referral center, between 1981 and 2014. Most of the patients (75%) were female, aged around 50 years (mean: 50±13 years). Initial symptoms were hyperthyroidism (8/20) and/or tumor mass-related symptoms. Median time to diagnosis was 18 months. Biochemical hyperthyroidism was found in 15 patients. Most of the tumors were macroadenomas (75%) and 30% were invasive. Seventeen patients underwent transsphenoidal surgery. All tumors expressed TSH, with>50% positive cells. Eleven were monohormonal and 6 plurihormonal, expressing ßTSH plus growth hormone (GH) and/or prolactin (PRL). Both subtypes showed high expression of Pit-1 and SSTR2A somatostatin receptors. SSTR5 was slightly expressed in the plurihormonal subtype. Ki-67 index was elevated (≥3%) in only one tumor. Signs of hyperthyroidism were more frequent in the plurihormonal than in the monohormonal subtype. At final follow-up (median: 34.79±66.7 months), 75% of the patients were in complete remission after surgery; persistent hyperthyroidism was controlled by somatostatin analogs, alone (n=3) or associated to radiotherapy (n=1). The multidisciplinary approach promoted early diagnosis and control of hyperthyroidism by neurosurgical treatment, associated to somatostatin analogs or not. Clinical/pathological correlations highlighted the variations in immune profiles and in clinical and biological symptoms.


Assuntos
Adenoma , Neoplasias Hipofisárias , Tireotropina/metabolismo , Adenoma/diagnóstico , Adenoma/metabolismo , Adenoma/patologia , Adenoma/terapia , Adulto , Idoso , Feminino , França , História do Século XX , História do Século XXI , Humanos , Hipertireoidismo/diagnóstico , Hipertireoidismo/patologia , Hipertireoidismo/terapia , Masculino , Pessoa de Meia-Idade , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/metabolismo , Neoplasias Hipofisárias/patologia , Neoplasias Hipofisárias/terapia , Estudos Retrospectivos , Somatostatina/análogos & derivados , Somatostatina/uso terapêutico
19.
Medicina (B Aires) ; 79(3): 191-196, 2019.
Artigo em Espanhol | MEDLINE | ID: mdl-31284253

RESUMO

Clinical presentation, treatment and its results were evaluated during long-term follow-up of 37 patients older than 65 years with pituitary adenoma. Causes of death were also evaluated. It was a retrospective and cross-sectional study. Prevalence of incidentalomas was 43% (16), macroadenomas 70.3% (26) and giant adenomas 16.2% (6). The most frequent tumor phenotype was the non-functioning adenoma (76%). The prevalence of visual field defects and neurological symptoms was 56% and 57% respectively. We found normal pituitary function in 54%, partial deficiency in 30% and panhypopituitarism in 16%. Thirty-two patients were treated, 5 were lost to follow-up without receiving treatment. Surgery was indicated in 18. Of those operated by trans-sphenoidal approach, 23% had postsurgical complications and 54% improved the visual field. By trans-craneal approach, 50% had post-surgical complications and 33% visual field improvement. During follow-up (55.1 ± 48.7 months) no tumor regrowth was observed, except in a giant adenoma. Four operated patients died, two due to causes related to tumor. Fourteen were not operated, 11 with non-functioning adenoma and normal visual field were periodically controlled and 3 with secreting adenomas received medical treatment. No tumor growth was observed during follow-up (43.7 ± 38.0 months). We did not observe tumor progression in elderly patients with non-functioning adenoma and normal visual field, so we suggest watchful approach and periodic control. When there are visual field defects, trans-sphenoidal surgery can be considered safe and effective. In secreting adenomas and depending on the associated comorbidities, medical treatment would be the appropriate approach.


Assuntos
Adenoma/terapia , Neoplasias Hipofisárias/terapia , Adenoma/diagnóstico , Adenoma/mortalidade , Adenoma/cirurgia , Idoso , Idoso de 80 Anos ou mais , Causas de Morte , Estudos Transversais , Feminino , Humanos , Masculino , Hormônios Hipofisários , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/mortalidade , Estudos Retrospectivos , Resultado do Tratamento
20.
Eur J Endocrinol ; 181(3): 351-361, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31319379

RESUMO

Objective: Silent corticotroph tumors are a pituitary neuroendocrine tumor subtype of corticotroph lineage that do not clinically express Cushing's disease. The silencing of this type of tumor is not fully understood. The aim of the present study was to delve into the lack of secretory activity, studying the post-transcriptional and post-translational regulation of POMC/ACTH in a series of molecularly identified functioning and silent corticotroph tumors. Design: We analyzed 24 silent corticotroph, 23 functioning corticotroph and 25 silent gonadotroph tumors. Methods: We used Sanger sequencing, quantitative real-time PCR and Western blot to analyze genetic alterations in POMC, gene expression of TBX19, NEUROD1, POMC, PCSK1, PCSK2, CPE and PAM and protein expression of POMC, PC1/3, PC2, CPE and PAM. Results: We found different polymorphisms in the POMC gene of corticotroph tumors, some of them related to deficiency of proopiomelanocortin. Silent corticotroph tumors showed lower PC1/3 gene and protein expression than functioning ones, especially compared to micro-functioning corticotroph tumors (all P < 0.05). Moreover, we found a positive correlation between PC2 and CPE gene and protein expression (rho ≥ 0.670, P < 0.009) in silent corticotroph tumors compared with functioning ones. Conclusions: By studying the post-transcriptional and post-translational processing of POMC and ACTH, respectively, in a large series of silent and functioning corticotroph tumors, we found that the lack of secretory activity of these tumors is related to an impaired processing of POMC and a high degradation of ACTH, with the macro-functioning corticotroph tumor behaving as an intermediate state between micro-functioning and silent corticotroph tumors.


Assuntos
Adenoma/genética , Hormônio Adrenocorticotrópico/genética , Corticotrofos , Hipersecreção Hipofisária de ACTH/genética , Neoplasias Hipofisárias/genética , Pró-Opiomelanocortina/genética , Adenoma/diagnóstico , Adenoma/metabolismo , Hormônio Adrenocorticotrópico/metabolismo , Adulto , Idoso , Corticotrofos/metabolismo , Corticotrofos/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Hipersecreção Hipofisária de ACTH/diagnóstico , Hipersecreção Hipofisária de ACTH/metabolismo , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/metabolismo , Pró-Opiomelanocortina/metabolismo , Interferência de RNA/fisiologia
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