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1.
Cureus ; 15(6): e39826, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-37397644

RESUMO

Objectives Meningiomas (MNGs) are the most common intracranial tumors found in the adult population. While most intracranial MNGs may be surgically removed, a subset of patients remains ineligible for conventional treatment. This is either because of a lack of surgical access or due to atypical, anaplastic or invasive characteristics of the tumors. These patients may benefit from targeted therapies that focus on cell receptor expression. The aim of this study was to assess dopamine receptor (DR) and Ki-67 expression in the MGNs of patients treated with surgery in the Instituto Nacional de Neurología y Neurocirugía, Mexico. Materials and methods This study analyzed 23 patients with confirmed MNG diagnoses (10 female and 13 male (mean age: 44.5 years)) who had undergone surgical resection between 2010 and 2014 at our institution. In the collected samples, we performed analyses for Ki-67, Dopamine 1 and Dopamine 2 receptors' expression. Results For the markers Ki-67, DR-D1 and DR-D2, the mean percentual expressions were 18.9%, 23.02% and 8.33%. No significant correlation was found between the expressions of these receptors and the studied MNG characteristics. The expression index of Ki-67 showed a significant relation with mean age (p = 0.03) and prolactin levels (p = 0.02). Conclusions Samples showed varied expressions of the studied receptors. Despite the difference in expressions between the markers, more studies are needed to confirm the findings. In contrast to previous studies, we could not find any relationship between D2-R and tumor characteristics.

2.
Int J Mol Sci ; 23(9)2022 Apr 27.
Artigo em Inglês | MEDLINE | ID: mdl-35563252

RESUMO

Corticotroph cells give rise to aggressive and rare pituitary neoplasms comprising ACTH-producing adenomas resulting in Cushing disease (CD), clinically silent ACTH adenomas (SCA), Crooke cell adenomas (CCA) and ACTH-producing carcinomas (CA). The molecular pathogenesis of these tumors is still poorly understood. To better understand the genomic landscape of all the lesions of the corticotroph lineage, we sequenced the whole exome of three SCA, one CCA, four ACTH-secreting PA causing CD, one corticotrophinoma occurring in a CD patient who developed Nelson syndrome after adrenalectomy and one patient with an ACTH-producing CA. The ACTH-producing CA was the lesion with the highest number of single nucleotide variants (SNV) in genes such as USP8, TP53, AURKA, EGFR, HSD3B1 and CDKN1A. The USP8 variant was found only in the ACTH-CA and in the corticotrophinoma occurring in a patient with Nelson syndrome. In CCA, SNV in TP53, EGFR, HSD3B1 and CDKN1A SNV were present. HSD3B1 and CDKN1A SNVs were present in all three SCA, whereas in two of these tumors SNV in TP53, AURKA and EGFR were found. None of the analyzed tumors showed SNV in USP48, BRAF, BRG1 or CABLES1. The amplification of 17q12 was found in all tumors, except for the ACTH-producing carcinoma. The four clinically functioning ACTH adenomas and the ACTH-CA shared the amplification of 10q11.22 and showed more copy-number variation (CNV) gains and single-nucleotide variations than the nonfunctioning tumors.


Assuntos
Adenoma Hipofisário Secretor de ACT , Adenoma , Carcinoma , Genômica , Síndrome de Nelson , Neoplasias Hipofisárias , Adenoma Hipofisário Secretor de ACT/genética , Adenoma/genética , Adenoma/patologia , Hormônio Adrenocorticotrópico , Aurora Quinase A , Carcinoma/genética , Corticotrofos/patologia , Receptores ErbB , Humanos , Melanocortinas , Complexos Multienzimáticos , Nucleotídeos , Neoplasias Hipofisárias/genética
3.
BMC Med Genomics ; 15(1): 52, 2022 03 08.
Artigo em Inglês | MEDLINE | ID: mdl-35260162

RESUMO

BACKGROUND: Pituitary adenomas (PA) are the second most common intracranial tumors and are classified according to hormone they produce, and the transcription factors they express. The majority of PA occur sporadically, and their molecular pathogenesis is incompletely understood. METHODS: Here we performed transcriptome and proteome analysis of tumors derived from POU1F1 (GH-, TSH-, and PRL-tumors, N = 16), NR5A1 (gonadotropes and null cells adenomas, n = 17) and TBX19 (ACTH-tumors, n = 6) lineages as well as from silent ACTH-tumors (n = 3) to determine expression of kinases, cyclins, CDKs and CDK inhibitors. RESULTS: The expression profiles of genes encoding kinases were distinctive for each of the three PA lineage: NR5A1-derived tumors showed upregulation of ETNK2 and PIK3C2G and alterations in MAPK, ErbB and RAS signaling, POU1F1-derived adenomas showed upregulation of PIP5K1B and NEK10 and alterations in phosphatidylinositol, insulin and phospholipase D signaling pathways and TBX19-derived adenomas showed upregulation of MERTK and STK17B and alterations in VEGFA-VEGFR, EGF-EGFR and Insulin signaling pathways. In contrast, the expression of the different genes encoding cyclins, CDK and CDK inhibitors among NR5A1-, POU1F1- and TBX19-adenomas showed only subtle differences. CDK9 and CDK18 were upregulated in NR5A1-adenomas, whereas CDK4 and CDK7 were upregulated in POUF1-adenomas. CONCLUSIONS: The kinome of PA clusters these lesions into three distinct groups according to the transcription factor that drives their terminal differentiation. And these complexes could be harnessed as molecular therapy targets.


Assuntos
Adenoma , Neoplasias Hipofisárias , Adenoma/metabolismo , Hormônio Adrenocorticotrópico/genética , Proteínas Reguladoras de Apoptose/genética , Quinases Ciclina-Dependentes/genética , Quinases Ciclina-Dependentes/metabolismo , Ciclinas/genética , Ciclinas/metabolismo , Humanos , Insulina , Neoplasias Hipofisárias/genética , Neoplasias Hipofisárias/metabolismo , Neoplasias Hipofisárias/patologia , Proteínas Serina-Treonina Quinases , Fatores de Transcrição/genética , Transcriptoma
4.
World Neurosurg ; 155: e761-e769, 2021 11.
Artigo em Inglês | MEDLINE | ID: mdl-34500097

RESUMO

BACKGROUND: Surgical resection remains the standard treatment for most giant pituitary adenomas (GPAs). The selected surgical approach for these complex lesions depends mainly on their extension. Single approaches may be limited in some cases presenting with invasion into multiple compartments, thereby limiting extent of resection. METHODS: We report a series of patients with GPA operated on through a combined approach involving an endoscopic endonasal transsphenoidal approach and a tubular retractor-assisted transventricular approach, describing the technique, its indications, limitations, and outcomes. Baseline and postoperative clinical, functional, and morphologic variables were documented up until each patient's last follow-up visit. RESULTS: Five patients harboring tumors extending into the third and lateral ventricles were included. Mean extent of resection was 94.6%. Mean follow-up was 39.4 months. One patient presented with a growth hormone-secreting GPA, who achieved remission after repeat resection during follow-up. There were no intraoperative complications, and 1 patient required reoperation for cerebrospinal fluid leak repair. One patient received adjuvant radiotherapy, and 3 patients remained stable requiring no additional treatment. All patients maintained an adequate postoperative functional status. CONCLUSIONS: The combined approach herein described may be a safe and effective option for some patients with GPAs extending into the third and lateral ventricles. An adequate patient selection is mandatory to exploit the benefits of each individual approach.


Assuntos
Adenoma/cirurgia , Ventrículos Cerebrais/cirurgia , Endoscopia/métodos , Microcirurgia/métodos , Neoplasias Hipofisárias/cirurgia , Osso Esfenoide/cirurgia , Adenoma/diagnóstico por imagem , Adulto , Ventrículos Cerebrais/diagnóstico por imagem , Terapia Combinada/métodos , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Cavidade Nasal/diagnóstico por imagem , Cavidade Nasal/cirurgia , Neoplasias Hipofisárias/diagnóstico por imagem , Osso Esfenoide/diagnóstico por imagem
5.
Endocrinol Diabetes Metab ; 4(2): e00229, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-33855226

RESUMO

Objective: To study the impact of secondary mental disorders in patients affected with acromegaly and correlate them with quality of life (QoL) and disease status. Design: An observational transversal descriptive and comparative study that evaluates QoL's impact due to secondary mental disorders in affected Mexicans with acromegaly using AcroQoL and SF-36 instruments. Correlation of the results with the disease's biochemical status was performed. According to Beck's scales, anxiety and depression analyses evaluate QoL's impact, and its gender variability is described. Results: Eighty-five patients with diagnoses of acromegaly were included. The mean age was 43.18 years, 47 being women (55.29%). The mean age at diagnosis was 37.95 years, with no difference between men and women. AcroQoL and SF-36 global and sub-domain scores differed significantly between men and women, the latter having lower global and individual sub-domain scores. The mean score of QoL, according to AcroQoL, is 59.40. In women, the mean values are less (55.13) than men (64.68), p = 0.021. The sub-domain analyses' scores in physical, appearance and social relationships were less in women (53.21; 47.34; 62.32) than men (62.68; 56.76; 73.87) p = 0.044, 0.069 and 0.013, respectively. Higher Beck's Depression Inventory (BDI) and Beck's Anxiety Inventory (BAI) scores correlated with lower QoL as assessed by global and individual sub-domain scores. Women presented significantly higher BDI and BAI mean scores when compared to men regardless of their biochemical status. Anxiety (p = 0.027) and depression (p < 0.001) severity were higher in women compared to men. Conclusion: Correlations between female gender, depression/anxiety scores and QoL require further validation. There is much to be routinely done to improve secondary psychopathology in patients affected by this disease. The need for mental status screening at diagnosis should be emphasized to identify secondary mental illnesses to improve QoL with its treatment.


Assuntos
Acromegalia/complicações , Transtornos Mentais/etiologia , Qualidade de Vida , Adulto , Ansiedade , Depressão , Feminino , Humanos , Masculino , Transtornos Mentais/diagnóstico , Transtornos Mentais/psicologia , Fatores Sexuais
6.
J Clin Endocrinol Metab ; 105(12)2020 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-32944780

RESUMO

CONTEXT: Acromegaly registries constitute a valuable source of therapeutic outcome information in real-life. OBJECTIVE: The objective of this work is to analyze surgical and pharmacological outcomes in the Mexican Acromegaly Registry (MAR). DESIGN AND METHODS: Data were extracted from the MAR informatic platform. Surgical remission was defined by a postoperative postglucose (GH) of less than 1 ng/mL and an insulin-like growth factor 1 (IGF-1) of less than 1.2 × upper limit of normal (ULN). Pharmacological remission was defined by a basal GH of less than 1 ng/mL and an IGF-1 of less than 1.2 × ULN. RESULTS: A total of 650 surgical outcomes were analyzed (94.6% transsphenoidal). Surgical remission was achieved in 40.15%, whereas 44.15% remained biochemically active. Persistently active disease after surgery was significantly associated with harboring an invasive macroadenoma, a basal GH of greater than 10 ng/mL, and/or an IGF-1 of greater than 2 × ULN at diagnosis on bivariate and multivariate analysis. The outcome of monotherapy with first-generation somatostatin analogs (SSAs) was evaluated in 267 patients (adjunctive in 65%), of whom 28.4% achieved remission. Persistently active disease was significantly associated with harboring an invasive macroadenoma as well as with pretreatment basal GH and IGF-1 levels of greater than 10 ng/mL and greater than 2 × ULN, respectively, on bivariate and multivariate analysis. Combined therapy with SSA and cabergoline was analyzed in 100 patients, of whom 19% achieved remission and 44% remained active; in this subset of patients, only a pretreatment IGF-1 of greater than 2 × ULN was significantly associated with persistent disease activity. CONCLUSION: Surgical and pharmacological outcomes in acromegaly are highly dependent on tumor size/invasiveness as well as on the degree of hypersomatotropinemia.


Assuntos
Acromegalia/tratamento farmacológico , Acromegalia/cirurgia , Acromegalia/diagnóstico , Acromegalia/epidemiologia , Adenoma/diagnóstico , Adenoma/tratamento farmacológico , Adenoma/epidemiologia , Adenoma/cirurgia , Adulto , Cabergolina/uso terapêutico , Terapia Combinada , Feminino , Adenoma Hipofisário Secretor de Hormônio do Crescimento/diagnóstico , Adenoma Hipofisário Secretor de Hormônio do Crescimento/tratamento farmacológico , Adenoma Hipofisário Secretor de Hormônio do Crescimento/epidemiologia , Adenoma Hipofisário Secretor de Hormônio do Crescimento/cirurgia , Hormônio do Crescimento Humano/sangue , Humanos , Fator de Crescimento Insulin-Like I/metabolismo , Masculino , México/epidemiologia , Pessoa de Meia-Idade , Procedimentos Neurocirúrgicos , Período Pós-Operatório , Prognóstico , Sistema de Registros , Estudos Retrospectivos , Somatostatina/análogos & derivados , Resultado do Tratamento
7.
Rev Invest Clin ; 72(1): 8-18, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32132734

RESUMO

The pituitary gland is responsible for the synthesis and secretion of various hormones that play a key role in regulating endocrine function and homeostasis. Pituitary adenomas (PA) are benign epithelial tumors arising from the endocrine cells of the anterior pituitary gland. Clinically relevant PA are relatively common and they occur in 0.1% of the general population. They are mostly benign monoclonal neoplasms that arise from any of the five hormone-secreting cell types of the anterior pituitary gland. PA are categorized as either functioning or non-functioning, depending on whether or not they produce a hormonal hypersecretion syndrome. Both functioning and non-functioning adenomas can produce symptoms or signs resulting from compression of the optic chiasm or invasion of cavernous sinuses. Only 5% of PA occur within the context of hereditary syndromes with reasonably well-defined oncogenic mechanisms. The vast majority of PA are sporadic, and their etiopathogenesis remains largely unknown. Pituitary tumor oncogenesis involves several mechanisms that eventually lead to abnormal cell proliferation and dysregulated hormone production. Among these factors, we found inactivating mutations of tumor suppressor genes, activating mutation of oncogenes and the participation of hormonal signals coming from the hypothalamus, all resulting in cell-cycle regulation abnormalities. In this review, we summarize the clinical and pathophysiological aspects of the different hereditary pituitary tumor syndromes.


Assuntos
Adenoma/patologia , Hipófise/patologia , Neoplasias Hipofisárias/patologia , Adenoma/epidemiologia , Adenoma/genética , Animais , Humanos , Mutação , Neoplasias Hipofisárias/epidemiologia , Neoplasias Hipofisárias/genética , Síndrome
8.
Case Rep Endocrinol ; 2020: 6827109, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32082649

RESUMO

Introduction. Pheochromocytomas (Pheo) and paragangliomas (PGL) are rare neuroendocrine tumors arising from chromaffin cells of the adrenal medulla and from the extra-adrenal autonomic paraganglia, respectively. Only 1-3% of head and neck PGL (HNPGL) show elevated catecholamines, and at least 30% of Pheo and PGL (PCPG) are associated with genetic syndromes caused by germline mutations in tumor suppressor genes and proto-oncogenes. Clinical Case. A 33-year-old man with a past medical history of resection of an abdominal PGL at the age of eleven underwent a CT scan after a mild traumatic brain injury revealing an incidental brain tumor. The diagnosis of a functioning PGL was made, and further testing was undertaken with a PET-CT with 68Ga-DOTATATE, SPECT-CT 131-MIBG, and genetic testing. Discussion and Conclusion. The usual clinical presentation of functioning PCPG includes paroxistic hypertension, headache, and diaphoresis, sometimes with a suggestive family history in 30-40% of cases. Only 20% of PGL are located in head and neck, of which only 1-3% will show elevated catecholamines. Metastatic disease is present in up to 50% of cases, usually associated with a hereditary germline mutation. However, different phenotypes can be observed depending on such germline mutations. Genetic testing is important in patients with PCPG since 31% will present a germline mutation. In this particular patient, an SDHB gene mutation was revealed, which can drastically influence the follow-up plan and the genetic counsel offered. A multidisciplinary approach is mandatory for every patient presenting with PCPG.SDHB gene mutation was revealed, which can drastically influence the follow-up plan and the genetic counsel offered. A multidisciplinary approach is mandatory for every patient presenting with PCPG.

9.
Rev. invest. clín ; Rev. invest. clín;72(1): 8-18, Jan.-Feb. 2020. tab, graf
Artigo em Inglês | LILACS | ID: biblio-1251829

RESUMO

ABSTRACT The pituitary gland is responsible for the synthesis and secretion of various hormones that play a key role in regulating endocrine function and homeostasis. Pituitary adenomas (PA) are benign epithelial tumors arising from the endocrine cells of the anterior pituitary gland. Clinically relevant PA are relatively common and they occur in 0.1% of the general population. They are mostly benign monoclonal neoplasms that arise from any of the five hormone-secreting cell types of the anterior pituitary gland. PA are categorized as either functioning or non-functioning, depending on whether or not they produce a hormonal hypersecretion syndrome. Both functioning and non-functioning adenomas can produce symptoms or signs resulting from compression of the optic chiasm or invasion of cavernous sinuses. Only 5% of PA occur within the context of hereditary syndromes with reasonably well-defined oncogenic mechanisms. The vast majority of PA are sporadic, and their etiopathogenesis remains largely unknown. Pituitary tumor oncogenesis involves several mechanisms that eventually lead to abnormal cell proliferation and dysregulated hormone production. Among these factors, we found inactivating mutations of tumor suppressor genes, activating mutation of oncogenes and the participation of hormonal signals coming from the hypothalamus, all resulting in cell-cycle regulation abnormalities. In this review, we summarize the clinical and pathophysiological aspects of the different hereditary pituitary tumor syndromes.


Assuntos
Humanos , Animais , Hipófise/patologia , Neoplasias Hipofisárias/patologia , Adenoma/patologia , Neoplasias Hipofisárias/genética , Neoplasias Hipofisárias/epidemiologia , Síndrome , Adenoma/genética , Adenoma/epidemiologia , Mutação
10.
Case Rep Endocrinol ; 2018: 5027859, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29854488

RESUMO

OBJECTIVE: Pituitary adenomas can be classified as clinically functional or silent. Depending on the reviewed literature, these are the first or second place in frequency of the total pituitary adenomas. Even rarer is the presence of a functional gonadotropinoma since only very few case reports exist to date. The conversion of a clinically silent to functional pituitary adenoma is extraordinarily rare; the mechanisms that explain these phenomena are unknown or not fully understood. METHODS: We report the case of a woman who initially had a nonfunctional gonadotropinoma and in the course of her medical condition showed biochemical changes in her hormonal pituitary profile compatible with a functional gonadotropinoma. RESULTS: We considered that the patient had a functional gonadotropinoma due to the hyperestrogenemia in the context of secondary amenorrhea, resolving the hyperestrogenemia after almost complete resection of the tumor. CONCLUSION: It is necessary to point out from a clinical and/or biochemical point of view the change in functionality that a nonfunctional pituitary adenoma may have. In the case of our patient, the suspicion of this change in functionality became evident when we found an increase in the FSH/LH ratio and a progressive increase in serum estradiol concentrations when the patient had amenorrhea.

11.
J Clin Endocrinol Metab ; 101(11): 3997-4004, 2016 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27428551

RESUMO

CONTEXT: Acromegaly is a systemic disorder caused by a GH-secreting pituitary adenoma. As with other rare diseases, acromegaly registries developed in various European countries have provided us with important information. OBJECTIVE: The objective of the study was to analyze the epidemiological, clinical, biochemical, and therapeutic data from the Mexican Acromegaly Registry (MAR). SETTING: The setting of the study was a nationwide patient registry. DESIGN AND METHODOLOGY: The MAR was created in 2009. It gathers data from 24 participating centers belonging to three different institutions using a specifically designed on-line platform. Only patients diagnosed after 1990 were included in the program. RESULTS: A total of 2057 patients (51% female, mean age at diagnosis 41.1 ± 24.5 y) have been registered for an estimated prevalence of 18 cases per 1 million inhabitants. Hypertension, glucose intolerance, diabetes, and dyslipidemia were present in 27%, 18.4%, 30%, and 24% of the patients, respectively. The IGF-1 level at diagnosis and the concomitant presence of hypertension were significantly associated with the development of diabetes. Transsphenoidal surgery was the primary treatment in 72% of the patients. Pharmacological treatment, mostly with somatostatin analogs, was administered primarily and adjunctively in 26% and 54% of the patients, respectively. Treatment choice varied among the three participating institutions, with the predominance of pharmacological therapy in two of them and of radiation therapy in the third. Therapeutic outcomes were similar to those reported in the European registries. CONCLUSIONS: The MAR is the largest and first non-European registry of the disease. Our findings highlight important within-country differences in treatment choice due to variations in the availability of resources.


Assuntos
Acromegalia/etiologia , Adenoma/diagnóstico , Adenoma Hipofisário Secretor de Hormônio do Crescimento/diagnóstico , Hipófise/fisiopatologia , Acromegalia/prevenção & controle , Adenoma/epidemiologia , Adenoma/fisiopatologia , Adenoma/terapia , Adulto , Estudos de Coortes , Terapia Combinada , Feminino , Seguimentos , Adenoma Hipofisário Secretor de Hormônio do Crescimento/epidemiologia , Adenoma Hipofisário Secretor de Hormônio do Crescimento/fisiopatologia , Adenoma Hipofisário Secretor de Hormônio do Crescimento/terapia , Terapia de Reposição Hormonal/efeitos adversos , Hospitais Públicos , Humanos , Hipofisectomia/efeitos adversos , Masculino , México/epidemiologia , Hipófise/efeitos dos fármacos , Hipófise/efeitos da radiação , Hipófise/cirurgia , Padrões de Prática Médica , Prevalência , Radioterapia/efeitos adversos , Sistema de Registros , Estudos Retrospectivos , Somatostatina/análogos & derivados , Somatostatina/uso terapêutico , Resultado do Tratamento
12.
Endocrine ; 53(2): 402-11, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27033541

RESUMO

Although aryl hydrocarbon receptor-interacting protein (AIP) mutations are rare in sporadic acromegaly, their prevalence among young patients is nonnegligible. The objectives of this study were to evaluate the frequency of AIP mutations in a cohort of Mexican patients with acromegaly with disease onset before the age of 30 and to search for molecular abnormalities in the AIP gene in teeth obtained from the "Tampico Giant". Peripheral blood DNA from 71 patients with acromegaly (51 females) with disease onset <30 years was analysed (median age of disease onset of 23 years) and correlated with clinical, biochemical and imaging characteristics. Sequencing was also carried out in DNA extracted from teeth of the Tampico Giant. Five patients (7 %) harboured heterozygous, germline mutations of the AIP gene. In two of them (a 9-year-old girl with gigantism and a young man with symptoms of GH excess since age 14) the c.910C>T (p.Arg304Ter), well-known truncating mutation was identified; in one of these two cases and her identical twin sister, the mutation proved to be a de novo event, since neither of their parents were found to be carriers. In the remaining three patients, new mutations were identified: a frameshift mutation (c.976_977insC, p.Gly326AfsTer), an in-frame deletion (c.872_877del, p.Val291_Leu292del) and a nonsense mutation (c.868A > T, p.Lys290Ter), which are predicted to be pathogenic based on in silico analysis. Patients with AIP mutations tended to have an earlier onset of acromegaly and harboured larger and more invasive tumours. A previously described genetic variant of unknown significance (c.869C > T, p.Ala299Val) was identified in DNA from the Tampico Giant. The prevalence of AIP mutations in young Mexican patients with acromegaly is similar to that of European cohorts. Our results support the need for genetic evaluation of patients with early onset acromegaly.


Assuntos
Acromegalia/genética , Gigantismo/genética , Peptídeos e Proteínas de Sinalização Intracelular/genética , Adenoma/genética , Adolescente , Adulto , Feminino , Frequência do Gene , Adenoma Hipofisário Secretor de Hormônio do Crescimento/genética , Humanos , Masculino , México , Mutação , Adulto Jovem
13.
Int J Surg Case Rep ; 9: 57-60, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-25725331

RESUMO

INTRODUCTION: Primary ectopic craniopharyngiomas have only rarely been reported. Craniopharyngiomas involve usually the sellar and suprasellar region, but can be originated from cell remnants of the obliterated craniopharyngeal duct or metaplastic change of andenohypophyseal cells. We present the first case of a primary ectopic frontotemporal craniopharyngioma. PRESENTATION OF CASE: A 35-year old woman presented with a one-year history of headache and diplopia. MRI showed a large frontotemporal cystic lesion. Tumor resection was performed with a keyhole endoscopic frontal lateral approach. The pathological features showed an adamantinomatous craniopharyngioma with a cholesterol granuloma reaction. DISCUSSION: There have been reported different localizations for primary ectopic craniopharyngioma. Our case presented a lobulated frontotemporal cystic mass formed by a dense eosinophilic proteinaceous material dystrophic calcifications and cholesterol crystals, with epithelial remnants. No tumor regrowth was observed in the magnetic resonance image 27 months postoperatively. CONCLUSION: Primary ectopic craniopharyngioma is a rare entity with a pathogenesis that remains uncertain. This is an unusual anatomic location associated with unique clinical findings.

15.
Neurosurg Focus ; 33(2): E1, 2012 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-22853827

RESUMO

Human sacrifice became a common cultural trait during the advanced phases of Mesoamerican civilizations. This phenomenon, influenced by complex religious beliefs, included several practices such as decapitation, cranial deformation, and the use of human cranial bones for skull mask manufacturing. Archaeological evidence suggests that all of these practices required specialized knowledge of skull base and upper cervical anatomy. The authors conducted a systematic search for information on skull base anatomical and surgical knowledge among Mesoamerican civilizations. A detailed exposition of these results is presented, along with some interesting information extracted from historical documents and pictorial codices to provide a better understanding of skull base surgical practices among these cultures. Paleoforensic evidence from the Great Temple of Tenochtitlan indicates that Aztec priests used a specialized decapitation technique, based on a deep anatomical knowledge. Trophy skulls were submitted through a stepwise technique for skull mask fabrication, based on skull base anatomical landmarks. Understanding pre-Columbian Mesoamerican religions can only be realized by considering them in their own time and according to their own perspective. Several contributions to medical practice might have arisen from anatomical knowledge emerging from human sacrifice and decapitation techniques.


Assuntos
Comportamento Ritualístico , Indígenas Centro-Americanos/história , Indígenas Norte-Americanos/história , Religião/história , Base do Crânio/anatomia & histologia , Base do Crânio/cirurgia , América Central , Cultura , História Antiga , Humanos , México
16.
Arch Med Res ; 43(5): 347-55, 2012 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-22824214

RESUMO

BACKGROUND AND AIMS: Craniopharyngioma is a rare and mostly benign epithelial tumor of the central nervous system, mostly affecting children. Considering that most of the published series of craniopharyngioma are based on pediatric populations, studies in adults gain importance based mainly on the reduced number of cases and the possible differences emerging from a mostly different histological type. We undertook this study to establish the pattern of presentation, morphological features and specific characteristics of craniopharyngioma in an adult Mexican population, as well as discussing the long-term outcome and how it may be influenced by surgical, anatomic and clinical factors. METHODS: A total of 153 adult patients (16 years or older) underwent transcranial and transsphenoidal surgery between January 1985 and December 2009, all with histological confirmation of craniopharyngioma. Hypothalamic involvement, surgical complications, rate of tumor resection and endocrinological outcome were evaluated. RESULTS: Seventy nine males (51.6%) and 74 females (48.4%) were included. Mean age at diagnosis was 32.4 years (range: 16-77 years). Mean initial tumor volume was 28.44 mL (range: 0.18-100.44 mL). Partial or complete hypothalamic involvement (Samii Grades III, IV and V) was found in 90.2%. The overall rate of new endocrinopathies was 37.25% after surgery (95% CI = 33.9-41.2). CONCLUSIONS: Gross total removal of craniopharyngiomas with large hypothalamic involvement was related to poor neuroendocrine outcome in adults. Partial removal should be indicated, associated with adjuvant therapy, in order to improve postoperative neuroendocrine status.


Assuntos
Craniofaringioma/cirurgia , Neoplasias Hipofisárias/cirurgia , Adolescente , Adulto , Idoso , Craniofaringioma/sangue , Craniofaringioma/mortalidade , Craniofaringioma/patologia , Feminino , Humanos , Sistema Hipotálamo-Hipofisário/fisiopatologia , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Neoplasias Hipofisárias/sangue , Neoplasias Hipofisárias/mortalidade , Neoplasias Hipofisárias/patologia , Sistema Hipófise-Suprarrenal/fisiopatologia , Estudos Retrospectivos , Estatísticas não Paramétricas , Tireotropina/sangue , Tiroxina/sangue , Tri-Iodotironina/sangue , Carga Tumoral , Adulto Jovem
17.
Pituitary ; 13(3): 230-5, 2010 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-20182808

RESUMO

UNLABELLED: ACTH-secreting tumors represent 10% of functioning pituitary adenomas, and most of them are microadenomas. It is generally accepted that only half of these tumors are correctly identified with current magnetic resonance imaging (MRI) techniques. The objective of the paper is to report a method for detecting suspected ACTH-secreting pituitary tumors undetectable by conventional dynamic MRI using dynamic 3-Tesla MRI (3T MRI) and half-dose gadopentetate dimeglumine (0.05 mmol/Kg). Eight patients were included (5 men and 3 women) with a mean age of 29.12 years. Each of them had a confirmed diagnosis of Cushing disease and a negative dynamic MRI for microadenoma using full-dose gadopentetate dimeglumine. A second MRI was then performed using only half the usual dose of contrast material. Images from the second MRI where compared with the first study. Microadenomas were detected in 100% of the patients using a half dose of the contrast. All were recognized on the basis of the presence of a hypointense nodular lesion surrounded by normal contrast-enhanced tissue. Six patients were submitted to surgery, and the results were confirmed by immunohistochemistry in all of them. The remaining subject had a sinus sample catheterization coincident with the MRI results. CONCLUSION: A half dose of dynamic resonance imaging contrast material increases the sensitivity of MRI detection of ACTH-secreting pituitary tumors.


Assuntos
Hormônio Adrenocorticotrópico/metabolismo , Gadolínio , Imageamento por Ressonância Magnética/métodos , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/metabolismo , Adolescente , Adulto , Criança , Feminino , Humanos , Masculino , Adulto Jovem
18.
J Neurooncol ; 95(2): 281-284, 2009 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-19449147

RESUMO

Follicular thyroid cancer rarely manifests itself as a distant metastatic lesion. We report a case of a 61-year-old woman presented with a solid mass located in the left temporo-occipital region. The 3D computed tomography showed a large solid mass with high vascularity, skull erosion and supra-infratentorial epidural mass effect. After magnetic resonance imaging (MRI) a suspect diagnosis of meningioma was made. The patient underwent surgery where a soft mass with transverse sinus invasion was encountered, the tumor was successfully resected employing microsurgical techniques. Histological examination revealed a thyroid follicular neoplasm with positive staining for follicular carcinoma in immunohistochemical analysis. Postoperatively levels of thyroid hormones were normal. Treatment was planned for the thyroid gland, but the patient did not consent. The present case emphasizes that although they are uncommon, dural metastasis can be mistaken for meningiomas. The definitive diagnosis of a meningioma should be established only after the histopathological analysis. Thyroid follicular carcinoma should be included in the differential diagnosis in cases of extrinsic tumoral lesions.


Assuntos
Adenocarcinoma Folicular/diagnóstico , Dura-Máter/patologia , Neoplasias Meníngeas/diagnóstico , Meningioma/diagnóstico , Neoplasias Cranianas/diagnóstico , Neoplasias da Glândula Tireoide/diagnóstico , Adenocarcinoma Folicular/cirurgia , Diagnóstico Diferencial , Dura-Máter/cirurgia , Feminino , Humanos , Técnicas Imunoenzimáticas , Neoplasias Meníngeas/cirurgia , Meningioma/cirurgia , Pessoa de Meia-Idade , Neoplasias Cranianas/secundário , Neoplasias Cranianas/cirurgia , Neoplasias da Glândula Tireoide/cirurgia
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