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[PMID]:28885086
[Au] Autor:Kamei T; Nonaka M; Uemura Y; Yamanouchi Y; Komori Y; Iwata R; Takeda J; Hashiba T; Yoshimura K; Asai A
[Ad] Endereço:Departments of 1 Neurosurgery and.
[Ti] Título:Enlarging pediatric ectopic Rathke's cleft cyst in the prepontine cistern: case report.
[So] Source:J Neurosurg Pediatr;20(5):480-484, 2017 Nov.
[Is] ISSN:1933-0715
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Rathke's cleft cyst is a cystic disease that occurs in the sella turcica or, occasionally, in the suprasellar area. An ectopic Rathke's cleft cyst is extremely rare, and its nature is less well understood. The authors report the case of a 14-year-old girl who presented with a growing cystic lesion in the prepontine cistern, immediately behind the dorsum sellae. Preoperative imaging and intraoperative investigation showed part of the cyst wall continuing into the dorsum sellae, to the pituitary gland. The cisternal portion of the cyst wall was totally resected via a right subtemporal approach. Histopathological examination of the cyst wall showed a monolayer of ciliated cells, identical to those of Rathke's cleft cyst. To the best of the authors' knowledge, this represents the first pediatric case of Rathke's cleft cyst occurring in the prepontine cistern.
[Mh] Termos MeSH primário: Cistos do Sistema Nervoso Central/diagnóstico por imagem
Cistos do Sistema Nervoso Central/cirurgia
Hipófise/diagnóstico por imagem
Hipófise/cirurgia
Espaço Subaracnóideo
[Mh] Termos MeSH secundário: Adolescente
Cistos do Sistema Nervoso Central/patologia
Feminino
Seres Humanos
Imagem por Ressonância Magnética
Hipófise/patologia
Ponte
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171102
[Lr] Data última revisão:
171102
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170909
[St] Status:MEDLINE
[do] DOI:10.3171/2017.6.PEDS1727


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[PMID]:28532906
[Au] Autor:Pojskic M; Zbytek B; Beckford NS; Boop FA; Arnautovic KI
[Ad] Endereço:Department of Neurosurgery, University of Marburg, Marburg, Germany.
[Ti] Título:First Report of Coexistence of Two Ectopic Pituitary Tumors: Rathke Cleft Cyst and Silent Adrenocorticotropic Hormone Adenoma.
[So] Source:World Neurosurg;104:1048.e1-1048.e7, 2017 Aug.
[Is] ISSN:1878-8769
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Rathke cleft cysts (RCCs) and pituitary adenomas (PAs) are thought to have a common embryonic ancestry; however, PAs with a concomitant RCC inside the sella turcica are rarely observed. Ectopic pituitary tumors are also rare. CASE DESCRIPTION: We present the case of a 65-year-old woman with an ectopic RCC in the sphenoid sinus and outside the sella turcica concomitant with an adrenocorticotropic hormone (ACTH)-staining, clinically silent PA. The patient had headache but no endocrine or visual disturbances. Preoperative magnetic resonance imaging revealed infrasellar cystic lesion in the sphenoid sinus with erosion of the clivus and intact sellar floor. The patient underwent gross total microsurgical resection through the transnasal route with an uneventful postoperative course. CONCLUSIONS: To our knowledge, this is the first reported ectopic RCC located outside the sella turcica with a concomitant ACTH-staining PA. This also appears to be the first ACTH-staining adenoma concomitant with RCC reported in the literature, regardless of location, not presenting with Cushing disease. This case shows that we can now include pituitary adenoma with or without a concomitant RCC in the differential diagnosis of processes in the sphenoid sinus. As both PAs and RCCs are benign sellar lesions, surgical management of a concomitant occurrence of these tumors mainly depends on the size of the lesions and their clinical manifestations. For patients with PA and concomitant RCC, surgical resection should be considered, as there is an approximatrely 20% recurrence rate of the cyst after resection and the possibility of future clival erosion, if left untreated.
[Mh] Termos MeSH primário: Adenoma Hipofisário Secretor de ACT/complicações
Adenoma/complicações
Cistos do Sistema Nervoso Central/complicações
Coristoma/complicações
Doenças dos Seios Paranasais/complicações
Neoplasias dos Seios Paranasais/complicações
Hipófise
[Mh] Termos MeSH secundário: Adenoma Hipofisário Secretor de ACT/diagnóstico por imagem
Adenoma Hipofisário Secretor de ACT/patologia
Adenoma Hipofisário Secretor de ACT/cirurgia
Adenoma/diagnóstico por imagem
Adenoma/patologia
Adenoma/cirurgia
Idoso
Cistos do Sistema Nervoso Central/diagnóstico por imagem
Cistos do Sistema Nervoso Central/patologia
Cistos do Sistema Nervoso Central/cirurgia
Coristoma/diagnóstico por imagem
Coristoma/patologia
Coristoma/cirurgia
Feminino
Seres Humanos
Imagem por Ressonância Magnética
Neoplasias Primárias Múltiplas/diagnóstico por imagem
Neoplasias Primárias Múltiplas/patologia
Neoplasias Primárias Múltiplas/cirurgia
Doenças dos Seios Paranasais/diagnóstico por imagem
Doenças dos Seios Paranasais/patologia
Doenças dos Seios Paranasais/cirurgia
Neoplasias dos Seios Paranasais/diagnóstico por imagem
Neoplasias dos Seios Paranasais/patologia
Neoplasias dos Seios Paranasais/cirurgia
Seio Esfenoidal/diagnóstico por imagem
Seio Esfenoidal/patologia
Seio Esfenoidal/cirurgia
Tomografia Computadorizada por Raios X
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170925
[Lr] Data última revisão:
170925
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170524
[St] Status:MEDLINE


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[PMID]:28512049
[Au] Autor:Beez T; Burgula S; Kamp M; Rapp M; Steiger HJ; Sabel M
[Ad] Endereço:Department of Neurosurgery, Medical Faculty, Heinrich Heine University, Düsseldorf, Germany. Electronic address: thomas.beez@med.uni-duesseldorf.de.
[Ti] Título:Space-Occupying Tumor Bed Cysts as a Complication of Modern Treatment for High-Grade Glioma.
[So] Source:World Neurosurg;104:509-515, 2017 Aug.
[Is] ISSN:1878-8769
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: The management of high-grade glioma (HGG) has been affected by recent landmark trials and is now more proactive. More aggressive treatment leads to hospitalization due to side effects, however. Space-occupying tumor bed cysts have been described, but not systematically assessed. We sought to analyze this complication in a contemporary HGG cohort. METHODS: We performed a retrospective review of patients with HGG treated between 2007 and 2013, identified patients with space-occupying tumor bed cysts, and reviewed their hospital notes for relevant variables. Statistical analyses were performed, and odds ratios (ORs) with 95% confidence intervals (CIs) were calculated. RESULTS: Tumor bed cysts were found in 12 of 282 patients (4%). The main symptoms were increased intracranial pressure (n = 11), new focal deficits (n = 6), and pseudomeningocele (n = 3), presenting at a median of 19 days since the last resection. Cysts were treated with cystoperitoneal (n = 7) and ventriculoperitoneal (n = 5) shunts, resulting in clinical benefit in 75% of those treated. Intraoperative opening of ventricles is a risk factor, with an OR of 39.339. We propose a classification system comprising 3 cyst types: isolated cyst, cyst with local cerebrospinal fluid (CSF) disturbance, and cyst with global CSF disturbance. CONCLUSIONS: In modern neuro-oncology, the rate of tumor bed cysts complicating HGG management appears stable compared with historical data. Shunt implantation is feasible and effective. We propose a classification system as a common data element for comparison across future studies.
[Mh] Termos MeSH primário: Cistos do Sistema Nervoso Central/patologia
Cistos do Sistema Nervoso Central/cirurgia
Glioma/patologia
Glioma/cirurgia
Neoplasias Supratentoriais/patologia
Neoplasias Supratentoriais/cirurgia
[Mh] Termos MeSH secundário: Adolescente
Adulto
Idoso
Idoso de 80 Anos ou mais
Edema Encefálico/classificação
Edema Encefálico/diagnóstico
Edema Encefálico/patologia
Edema Encefálico/cirurgia
Carmustina/administração & dosagem
Cistos do Sistema Nervoso Central/classificação
Cistos do Sistema Nervoso Central/diagnóstico
Ventrículos Cerebrais/patologia
Ventrículos Cerebrais/cirurgia
Quimiorradioterapia Adjuvante
Terapia Combinada
Irradiação Craniana
Craniotomia
Feminino
Glioblastoma/classificação
Glioblastoma/diagnóstico
Glioblastoma/patologia
Glioblastoma/cirurgia
Glioma/classificação
Glioma/diagnóstico
Seres Humanos
Imagem por Ressonância Magnética
Masculino
Meia-Idade
Gradação de Tumores/classificação
Estudos Retrospectivos
Neoplasias Supratentoriais/classificação
Neoplasias Supratentoriais/diagnóstico
Tomografia Computadorizada por Raios X
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
U68WG3173Y (Carmustine)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170928
[Lr] Data última revisão:
170928
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170518
[St] Status:MEDLINE


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[PMID]:28427982
[Au] Autor:Tosaka M; Higuchi T; Horiguchi K; Osawa T; Arisaka Y; Fujita H; Tsushima Y; Yoshimoto Y
[Ad] Endereço:Department of Neurosurgery, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan. Electronic address: nstosaka@gunma-u.ac.jp.
[Ti] Título:Preoperative Evaluation of Sellar and Parasellar Macrolesions by [ F]Fluorodeoxyglucose Positron Emission Tomography.
[So] Source:World Neurosurg;103:591-599, 2017 Jul.
[Is] ISSN:1878-8769
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:OBJECTIVE: Various diseases can occur in the sellar and suprasellar regions. The potential of [ F]fluorodeoxyglucose (FDG) positron emission tomography (PET) for the preoperative evaluation of sellar and parasellar lesions was investigated. METHODS: A total of 49 patients aged 8-82 years with sellar and parasellar macroscopic lesions (≥10 mm) underwent FDG PET. Twenty-two patients had pituitary adenomas, including 14 nonfunctioning and 8 growth hormone-secreting adenomas. Eleven patients had craniopharyngiomas, including 5 adamantinomatous and 6 squamous-papillary types. Eight patients had chordoma, 4 had meningioma, and 4 had a Rathke cleft cyst. The maximum standardized uptake value (SUV ), and the ratio of the SUV in the tumor to the mean standardized uptake value in the normal cortex (T/N ratio) or in the normal white matter (T/W ratio) were calculated. The relationships between SUV , T/N ratio, and T/W ratio, and lesion disease were evaluated. RESULTS: Uptakes of FDG, including SUV , T/N ratio, and T/W ratio, were lower in chordoma and Rathke cleft cyst compared with pituitary adenoma. SUV , T/N ratio, and T/W ratio of nonfunctioning adenoma were significantly higher than those of growth hormone-secreting adenoma. SUV , T/N ratio, and T/W ratio of squamous-papillary type were significantly higher than those of the adamantinomatous type of craniopharyngioma. CONCLUSIONS: FDG PET is useful for the preoperative diagnosis of sellar and parasellar macrolesions. High uptake in nonfunctioning pituitary adenoma, and low uptake in chordoma are significant. The difference in FDG uptake dependent on the histologic subtype may be related to the specific genetics of the craniopharyngioma subtype.
[Mh] Termos MeSH primário: Adenoma/diagnóstico por imagem
Cistos do Sistema Nervoso Central/diagnóstico por imagem
Cordoma/diagnóstico por imagem
Craniofaringioma/diagnóstico por imagem
Adenoma Hipofisário Secretor de Hormônio do Crescimento/diagnóstico por imagem
Neoplasias Meníngeas/diagnóstico por imagem
Meningioma/diagnóstico por imagem
Neoplasias Hipofisárias/diagnóstico por imagem
Sela Túrcica/diagnóstico por imagem
[Mh] Termos MeSH secundário: Adenoma/patologia
Adenoma/cirurgia
Adolescente
Adulto
Idoso
Idoso de 80 Anos ou mais
Cistos do Sistema Nervoso Central/patologia
Cistos do Sistema Nervoso Central/cirurgia
Criança
Cordoma/patologia
Cordoma/cirurgia
Craniofaringioma/patologia
Craniofaringioma/cirurgia
Feminino
Fluordesoxiglucose F18
Adenoma Hipofisário Secretor de Hormônio do Crescimento/patologia
Adenoma Hipofisário Secretor de Hormônio do Crescimento/cirurgia
Seres Humanos
Masculino
Neoplasias Meníngeas/patologia
Neoplasias Meníngeas/cirurgia
Meningioma/patologia
Meningioma/cirurgia
Meia-Idade
Neoplasias Hipofisárias/patologia
Neoplasias Hipofisárias/cirurgia
Tomografia por Emissão de Pósitrons
Cuidados Pré-Operatórios
Compostos Radiofarmacêuticos
Estudos Retrospectivos
Sela Túrcica/patologia
Sela Túrcica/cirurgia
Carga Tumoral
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Radiopharmaceuticals); 0Z5B2CJX4D (Fluorodeoxyglucose F18)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170925
[Lr] Data última revisão:
170925
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170422
[St] Status:MEDLINE


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[PMID]:28272259
[Au] Autor:Yang C; Bao X; Liu X; Deng K; Feng M; Yao Y; Wang R
[Ad] Endereço:Department of Neurosurgery, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing, China.
[Ti] Título:Rathke cleft cyst masquerading as pituitary abscess: A case report.
[So] Source:Medicine (Baltimore);96(10):e6303, 2017 Mar.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Rathke cleft cyst (RCC) is a rare cystic sellar entity, which is usually small in size and asymptomatic in most patients. RCC presenting panhypopituitarism and a cystic lesion with rim enhancement on magnetic resonance imaging is extremely rare. Therefore, it is easy to be misdiagnosed as pituitary abscess because of the similar clinical manifestations and neuroimaging changes. CASE SUMMARY: We report a rare case of RCC masquerading as pituitary abscess clinically and radiologically with no evidence of central nervous system infection. The patient was initially suspected to be diagnosed with pituitary abscess, which was denied by the histopathological findings of RCC with no intraoperative drainage of abscess.We present an uncommon case of RCC masquerading as pituitary abscess in a 62-year-old Chinese male patient. The patient was admitted to Peking Union Medical College Hospital complaining of severe frontal pulsatile headache, visual acuity deficit, polyuria, polydipsia, and slight disturbance of consciousness. The biochemical and endocrinological examinations revealed severe hyponatremia and panhypopituitarism. Magnetic resonance imaging showed a sellar lesion with the apparent cystic change and rim enhancement. Accordingly, pituitary abscess was misdiagnosed at the beginning.The patient received hormone replacement therapy and underwent a trans-sphenoidal surgery. The surgical findings were uneventful. The histopathological examinations showed no infiltration of inflammatory cells or pus, and proved the lesion to be RCC. CONCLUSION: Through this rare case, we aim to emphasize that the differential diagnosis of sellar lesions requires constant vigilance and that RCC may lead to clinical and radiological changes similar with pituitary abscess.
[Mh] Termos MeSH primário: Abscesso Encefálico/diagnóstico
Cistos do Sistema Nervoso Central/diagnóstico
Doenças da Hipófise/diagnóstico
[Mh] Termos MeSH secundário: Diagnóstico Diferencial
Seres Humanos
Imagem por Ressonância Magnética
Masculino
Meia-Idade
Neuroimagem
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1703
[Cu] Atualização por classe:170324
[Lr] Data última revisão:
170324
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170309
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000006303


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[PMID]:28213198
[Au] Autor:Li T; Wu X; Zhang Y
[Ad] Endereço:Department of Neurosurgery, West China Hospital of Sichuan University, Chengdu, Sichuan Province, P. R. China.
[Ti] Título:A Rare Presentation of an Enterogenous Cyst as an Intra-Axial Pontine Lesion.
[So] Source:World Neurosurg;100:707.e1-707.e3, 2017 Apr.
[Is] ISSN:1878-8769
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:A 56-year-old man was admitted to our hospital with intermittent right facial numbness for 1 year and instability on walking for 6 months. Magnetic resonance imaging revealed an ovoid and well-demarcated intra-axial pontine lesion. Cyst fenestration and biopsy were carried out, and the diagnosis of enterogenous cyst was made by magnetic resonance imaging and pathologic findings. Preoperative symptoms resolved completely 3 months after surgery and no evidence of recurrence was found during 3 years of follow-up. Although total resection of enterogenous cysts is recommended, cyst fenestration may be useful if the cyst is adherent to vital neurovascular structures.
[Mh] Termos MeSH primário: Neoplasias do Tronco Encefálico/diagnóstico por imagem
Neoplasias do Tronco Encefálico/cirurgia
Cistos do Sistema Nervoso Central/diagnóstico por imagem
Cistos do Sistema Nervoso Central/cirurgia
Ponte/diagnóstico por imagem
Ponte/cirurgia
[Mh] Termos MeSH secundário: Neoplasias do Tronco Encefálico/patologia
Cistos do Sistema Nervoso Central/patologia
Diagnóstico Diferencial
Quarto Ventrículo/diagnóstico por imagem
Quarto Ventrículo/cirurgia
Seres Humanos
Masculino
Meia-Idade
Ponte/patologia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170918
[Lr] Data última revisão:
170918
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170219
[St] Status:MEDLINE


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[PMID]:28131229
[Au] Autor:Tamura R; Ohira T; Emoto K; Fujiwara H; Horikoshi T; Yoshida K
[Ad] Endereço:Department of Neurosurgery, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan. Electronic address: moltobello-r-610@hotmail.co.jp.
[Ti] Título:Leukoencephalopathy, cerebral calcifications, and cysts: A clinical case involving a long-term follow-up and literature review.
[So] Source:J Neurol Sci;373:60-65, 2017 Feb 15.
[Is] ISSN:1878-5883
[Cp] País de publicação:Netherlands
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Leukoencephalopathy, cerebral calcifications, and cysts (LCC) is a rare disease that was first reported by Labrune in 1996. A case of adult-onset LCC was successfully followed up for a long period. CASE PRESENTATION: A 30-year-old female presented with visual field disturbance and seizure on several occasions. Radiographic images revealed multiple supratentorial cysts and calcifications in the bilateral nucleus basalis and cerebella. Aspiration, Ommaya reservoir placement, and nodule removal were performed for the responsible cysts, and the patient had a good postoperative course. DISCUSSION: A tiny, strongly enhanced nodule was identified before cyst formation on her radiographic images. Thus, cyst growth may be related to nodule microbleeding. According to our review, if the responsible cyst is located on the noneloquent area, surgical removal of the cyst should be considered. However, if the responsible cyst is located on the eloquent area, the nodule should be first removed because nodules can bleed and enlarge cysts. CONCLUSION: Careful follow-up is needed, especially for cysts with a strongly enhanced nodule.
[Mh] Termos MeSH primário: Calcinose/diagnóstico
Calcinose/terapia
Cistos do Sistema Nervoso Central/diagnóstico
Cistos do Sistema Nervoso Central/terapia
Leucoencefalopatias/diagnóstico
Leucoencefalopatias/terapia
[Mh] Termos MeSH secundário: Adulto
Encéfalo/diagnóstico por imagem
Encéfalo/patologia
Encéfalo/cirurgia
Calcinose/diagnóstico por imagem
Calcinose/patologia
Cistos do Sistema Nervoso Central/diagnóstico por imagem
Cistos do Sistema Nervoso Central/patologia
Diagnóstico Diferencial
Feminino
Seguimentos
Seres Humanos
Leucoencefalopatias/diagnóstico por imagem
Leucoencefalopatias/patologia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (Leukoencephalopathy Brain Calcifications and Cysts)
[Em] Mês de entrada:1705
[Cu] Atualização por classe:170817
[Lr] Data última revisão:
170817
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170130
[St] Status:MEDLINE


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[PMID]:28120170
[Au] Autor:Duan K; Asa SL; Winer D; Gelareh Z; Gentili F; Mete O
[Ad] Endereço:Department of Pathology, University Health Network, 200 Elizabeth Street, 11th floor, Toronto, ON, M5G 2C4, Canada.
[Ti] Título:Xanthomatous Hypophysitis Is Associated with Ruptured Rathke's Cleft Cyst.
[So] Source:Endocr Pathol;28(1):83-90, 2017 Mar.
[Is] ISSN:1559-0097
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Xanthomatous hypophysitis is a rare inflammatory disease of the pituitary gland that can mimic a neoplastic lesion clinically and radiologically. Its pathogenesis remains largely unknown, although recent evidence suggests that pituitary inflammation may occur as a secondary reaction to mucous content released from a ruptured cyst. In a series of 1221 pituitary specimens, we identified seven cases of xanthomatous hypophysitis. Six patients had complete radiological and biochemical workup preoperatively: a cystic-appearing pituitary mass was identified in all six patients (100%) with a mean size of 2.0 cm (range 1.4-2.5 cm) on imaging, and pituitary endocrine dysfunction was noted in five patients (83.3%). In all cases, the pituitary mass was resected through an endoscopic transsphenoidal approach. Pathological examination revealed the presence of foamy macrophages admixed with variable amounts of giant cells and chronic inflammatory cells, confirming the diagnosis of xanthomatous hypophysitis. Additionally, all cases presented with concurrent findings of ruptured Rathke's cleft cyst, with the exception of one patient who had previous surgery for a Rathke's cleft cyst, followed by recurrence and diagnosis of xanthomatous hypophysitis. While accurate distinction of hypophysitis from a pituitary neoplasm can be problematic in the preoperative setting, the identification of a cystic lesion in the sella turcica should raise the possibility of such an entity in the clinical and radiological differential diagnosis. The current series provides further evidence that xanthomatous hypophysitis predominantly occurs as a secondary reaction to a ruptured Rathke's cleft cyst; thus, it is best classified as a secondary (reactive) hypophysitis.
[Mh] Termos MeSH primário: Cistos do Sistema Nervoso Central/complicações
Cistos do Sistema Nervoso Central/patologia
Hipofisite/etiologia
Neoplasias Hipofisárias/complicações
Neoplasias Hipofisárias/patologia
[Mh] Termos MeSH secundário: Adulto
Feminino
Seres Humanos
Masculino
Meia-Idade
Estudos Retrospectivos
Ruptura Espontânea
Xantomatose/complicações
Xantomatose/patologia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1703
[Cu] Atualização por classe:171107
[Lr] Data última revisão:
171107
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170126
[St] Status:MEDLINE
[do] DOI:10.1007/s12022-017-9471-x


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[PMID]:28072696
[Au] Autor:Xu W; Zhao J; Zhu Y; Zhang W
[Ad] Endereço:aDepartment of Radiology bDepartment of Rheumatology cDepartment of Neurology, Peking Union Medical College Hospital (PUMCH), Peking Union Medical College and Chinese Academy of Medical Sciences (PUMC&CAMS), Beijing, People's Republic of China.
[Ti] Título:Cerebroretinal microangiopathy with calcifications and cysts: A case report.
[So] Source:Medicine (Baltimore);96(1):e5545, 2017 Jan.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONAL: Cerebroretinal microangiopathy with calcifications and cysts (CRMCC) is believed to be an autosomal recessive genetic disease, with disorders in multisystem organs. Its characteristic neurological disorders manifested on neuroimaging are a triad of leukoencephalopathy, intracranial calcifications, and parenchymal cysts. In this paper, we report a CRMCC patient with multisystem involvement, focusing on the neuroimaging features, to get a better understanding of the rare disease and improve our diagnostic ability. PATIENT CONCERNS: The 23-year-old female patient firstly presented with an adolescence onset of ophthalmological manifestations. Four years later, hematological and neurological disorders occurred, the latter of which demonstrated a relatively slow progression in the following 7 years preceding her presentation to our hospital. INTERVENTIONS: During hospitalization, disorders involving digestive, cardiovascular and respiratory systems were also detected. In addition, a more comprehensive depiction of neurological disorders on neuroimaging was also obtained. DIAGNOSES: On the basis of multiple system disorders and the detection of mutations in conserved telomere maintenance component 1(CTC1) gene, a diagnosis of CRMCC was made. OUTCOMES: After supportive therapy during her 4-week hospitalization, the patient's general condition improved and was released from the hospital. LESSONS: CRMCC could be primarily diagnosed with the aid of its multiple system disorders and remarkable neuroimaging features. Cerebral micro hemorrhages determined by the combination of CT and T2-weighted magnetic resonance images in our case could provide some additional information for diagnosis. Furthermore, several other associated disorders were depicted for the first time in our case, expanding the clinical spectrum of CRMCC.
[Mh] Termos MeSH primário: Assistência ao Convalescente/métodos
Ataxia
Neoplasias Encefálicas
Encéfalo
Calcinose
Cistos do Sistema Nervoso Central
Leucoencefalopatias
Espasticidade Muscular
Neuroimagem/métodos
Doenças Retinianas
Convulsões
Proteínas de Ligação a Telômeros/genética
[Mh] Termos MeSH secundário: Adulto
Ataxia/diagnóstico
Ataxia/genética
Ataxia/fisiopatologia
Ataxia/terapia
Encéfalo/diagnóstico por imagem
Encéfalo/patologia
Neoplasias Encefálicas/diagnóstico
Neoplasias Encefálicas/genética
Neoplasias Encefálicas/fisiopatologia
Neoplasias Encefálicas/terapia
Calcinose/diagnóstico
Calcinose/genética
Calcinose/fisiopatologia
Calcinose/terapia
Cistos do Sistema Nervoso Central/diagnóstico
Cistos do Sistema Nervoso Central/genética
Cistos do Sistema Nervoso Central/fisiopatologia
Cistos do Sistema Nervoso Central/terapia
Diagnóstico Diferencial
Gerenciamento Clínico
Progressão da Doença
Feminino
Seres Humanos
Leucoencefalopatias/diagnóstico
Leucoencefalopatias/genética
Leucoencefalopatias/fisiopatologia
Leucoencefalopatias/terapia
Espasticidade Muscular/diagnóstico
Espasticidade Muscular/genética
Espasticidade Muscular/fisiopatologia
Espasticidade Muscular/terapia
Mutação
Espectroscopia de Prótons por Ressonância Magnética/métodos
Doenças Retinianas/diagnóstico
Doenças Retinianas/genética
Doenças Retinianas/fisiopatologia
Doenças Retinianas/terapia
Convulsões/diagnóstico
Convulsões/genética
Convulsões/fisiopatologia
Convulsões/terapia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Ctc1 protein, human); 0 (Telomere-Binding Proteins)
[Em] Mês de entrada:1702
[Cu] Atualização por classe:170224
[Lr] Data última revisão:
170224
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170111
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000005545


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[PMID]:28057594
[Au] Autor:Fukui I; Hayashi Y; Kita D; Sasagawa Y; Oishi M; Tachibana O; Nakada M
[Ad] Endereço:Department of Neurosurgery, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan.
[Ti] Título:Significant Improvement in Chronic Persistent Headaches Caused by Small Rathke Cleft Cysts After Transsphenoidal Surgery.
[So] Source:World Neurosurg;99:362-368, 2017 Mar.
[Is] ISSN:1878-8769
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:PURPOSE: Rathke cleft cysts (RCC) usually are asymptomatic and can be observed via the use of conservative methods. Some patients with RCCs, however, have severe headaches even if they are small enough to be confined to the sella, and these small RCCs seldom have been discussed. This study presents an investigation into clinical characteristics of small RCCs associated with severe headaches, demonstrating efficacy and safety of endoscopic transsphenoidal surgery (ETSS) to relieve headaches. METHODS: In this study, 13 patients with small RCCs (maximum diameter <10 mm) who presented with headaches and were treated by ETSS at our institute from 2009 to 2014 were recruited. These RCCs were treated Headache Impact Test-6 (HIT-6) score was calculated both pre- and postoperatively to evaluate headache severity. RESULTS: All patients complained of severe headaches, which disturbed their daily life. Most headaches were nonpulsating and localized in the frontal area. Characteristically, 6 patients (46%) experienced severe headaches with sudden onset that continued chronically. HIT-6 score was 64 on average, meaning headaches affected daily life severely. After surgical decompression of the cyst, headache in all of the patients improved dramatically and HIT-6 score decreased significantly to 37, suggesting that headaches were diminished. No newly developed deficiencies of the anterior pituitary lobe function were detected. Postoperative occurrence of diabetes insipidus was found in 2 patients, both of which were transient. No recurring cysts were found. CONCLUSIONS: Severe headaches can develop from small RCCs. In the present study, ETSS was performed on such patients effectively and safely to relieve their headaches.
[Mh] Termos MeSH primário: Cistos do Sistema Nervoso Central/cirurgia
Descompressão Cirúrgica/métodos
Transtornos da Cefaleia/cirurgia
Neuroendoscopia/métodos
[Mh] Termos MeSH secundário: Adulto
Idoso
Cistos do Sistema Nervoso Central/complicações
Diabetes Insípido/epidemiologia
Feminino
Transtornos da Cefaleia/etiologia
Seres Humanos
Masculino
Meia-Idade
Complicações Pós-Operatórias/epidemiologia
Resultado do Tratamento
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170918
[Lr] Data última revisão:
170918
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170107
[St] Status:MEDLINE



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