RESUMO
BACKGROUND: The origin of syrinx fluid is controversial. OBJECTIVE: To elucidate the mechanisms of syringomyelia associated with cerebrospinal fluid pathway obstruction and with intramedullary tumors, contrast transport from the spinal subarachnoid space (SAS) to syrinx was evaluated in syringomyelia patients. METHODS: We prospectively studied patients with syringomyelia: 22 with Chiari I malformation and 16 with SAS obstruction-related syringomyelia before and 1 wk after surgery, and 9 with tumor-related syringomyelia before surgery only. Computed tomography-myelography quantified dye transport into the syrinx before and 0.5, 2, 4, 6, 8, 10, and 22 h after contrast injection by measuring contrast density in Hounsfield units (HU). RESULTS: Before surgery, more contrast passed into the syrinx in Chiari I malformation-related syringomyelia and spinal obstruction-related syringomyelia than in tumor-related syringomyelia, as measured by (1) maximum syrinx HU, (2) area under the syrinx concentration-time curve (HU AUC), (3) ratio of syrinx HU to subarachnoid cerebrospinal fluid (CSF; SAS) HU, and (4) AUC syrinx/AUC SAS. More contrast (AUC) accumulated in the syrinx and subarachnoid space before than after surgery. CONCLUSION: Transparenchymal bulk flow of CSF from the subarachnoid space to syrinx occurs in Chiari I malformation-related syringomyelia and spinal obstruction-related syringomyelia. Before surgery, more subarachnoid contrast entered syringes associated with CSF pathway obstruction than with tumor, consistent with syrinx fluid originating from the subarachnoid space in Chiari I malformation and spinal obstruction-related syringomyelia and not from the subarachnoid space in tumor-related syringomyelia. Decompressive surgery opened subarachnoid CSF pathways and reduced contrast entry into syringes associated with CSF pathway obstruction.
Assuntos
Líquido Cefalorraquidiano/diagnóstico por imagem , Siringomielia/líquido cefalorraquidiano , Siringomielia/diagnóstico por imagem , Siringomielia/patologia , Adulto , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Espaço Subaracnóideo/diagnóstico por imagem , Espaço Subaracnóideo/patologiaRESUMO
Von Hippel-Lindau (VHL) disease is a multisystem genetic disease, the cardinal manifestations of which include central nervous system hemangioblastomas (CNS HB), renal cell carcinomas (RCC), and pheochromocytoma. Tumorigenesis in VHL of both RCC and CNS HB occurs secondary to downstream effects of a mutated or absent VHL protein. Treatment of RCCs with tyrosine kinase inhibitors (TKIs) such as Pazopanib is now first line therapy, but their effect on VHL-associated CNS HBs remains unknown. We report the use of Pazopanib in a patient with VHL disease for treatment of RCC who also harbored multiple CNS HBs. Following initiation of treatment, a large cervical and a lumbar spinal HB regressed in size while the remaining CNS HBs exhibited stable or progressive disease. These findings highlight the multiplicity of factors contributing to hemangioblastoma development, even among tumors with a common germline mutation, and the potential limitations of TKIs, but additionally this report supports the conservative management of asymptomatic VHL patients with spinal HBs whereby tumor response to TKI treatment may alleviate or postpone the need for surgery.
Assuntos
Inibidores da Angiogênese/uso terapêutico , Neoplasias do Sistema Nervoso Central/tratamento farmacológico , Hemangioblastoma/tratamento farmacológico , Pirimidinas/uso terapêutico , Sulfonamidas/uso terapêutico , Doença de von Hippel-Lindau/complicações , Adulto , Carcinoma de Células Renais/tratamento farmacológico , Carcinoma de Células Renais/genética , Neoplasias do Sistema Nervoso Central/genética , Hemangioblastoma/genética , Humanos , Indazóis , Neoplasias Renais/tratamento farmacológico , Neoplasias Renais/genética , MasculinoRESUMO
Immunomodulation and tumor-induced tolerance is one of the central mechanisms in the oncogenesis of malignant and benign neoplasms. While numerous pathways have been described, signaling through the programmed death receptor 1 (PD-1) on T lymphocytes, via activation through its ligand, programmed death ligand 1 (PD-L1) expressed on tumor cells is one of the central pathways involved in tumor-induced tolerance. While the neoplastic component of germinomas of the CNS is the germ cell, these tumors also exhibit an abundance of quiescent tumor-infiltrating lymphocytes. We therefore investigated whether PD-L1 expression may be responsible for germinoma-induced T cell anergy, and if these tumors may be susceptible to immunotherapy. Pathologic specimens obtained from 21 cases of CNS germinomas between 2000 and 2016 were analyzed for the presence of PD-L1 and PD-1 expression by immunohistochemistry. Nineteen of 21 germinomas (90%) harbored germ cell components that stained positively for PD-L1. Positive lymphocyte staining for PD-L1 was evident in 16 cases. PD-1 expression was largely confined to lymphocytes; PD-L1 therefore may contribute to lymphocyte quiescence observed in these tumors. These results raise the possibility that immune checkpoint inhibitors such as nivolumab may have a therapeutic role in future treatment of germinomas.
Assuntos
Neoplasias do Sistema Nervoso Central/metabolismo , Regulação Neoplásica da Expressão Gênica/fisiologia , Germinoma/metabolismo , Receptor de Morte Celular Programada 1/metabolismo , Linfócitos T/metabolismo , Adolescente , Adulto , Antígeno B7-H1/metabolismo , Neoplasias do Sistema Nervoso Central/patologia , Criança , Feminino , Células Germinativas/metabolismo , Germinoma/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto JovemRESUMO
OBJECTIVE Many centers use conventional and dynamic contrast-enhanced MRI (DMRI) sequences in patients with Cushing's disease. The authors assessed the utility of the 3D volumetric interpolated breath-hold examination, a spoiled-gradient echo 3D T1 sequence (SGE) characterized by superior soft tissue contrast and improved resolution, compared with DMRI and conventional MRI (CMRI) for detecting microadenomas in patients with Cushing's disease. METHODS This study was a blinded assessment of pituitary MRI in patients with proven Cushing's disease. Fifty-seven patients who had undergone surgery for Cushing's disease (10 male, 47 female; age range 13-69 years), whose surgical findings were considered to represent a microadenoma, and who had been examined with all 3 imaging techniques were included. Thus, selection emphasized patients with prior negative or equivocal MRI on referral. The MRI annotations were anonymized and 4 separate imaging sets were independently read by 3 blinded, experienced clinicians: a neuroradiologist and 2 pituitary surgeons. RESULTS Forty-eight surgical specimens contained an adenoma (46 ACTH-staining adenomas, 1 prolactinoma, and 1 nonfunctioning microadenoma). DMRI detected 5 adenomas that were not evident on CMRI, SGE detected 8 adenomas not evident on CMRI, including 3 that were not evident on DMRI. One adenoma was detected on DMRI that was not detected on SGE. McNemar's test for efficacy between the different MRI sets for tumor detection showed that the addition of SGE to CMRI increased the number of tumors detected from 18 to 26 (p = 0.02) based on agreement of at least 2 of 3 readers. CONCLUSIONS SGE shows higher sensitivity than DMRI for detecting and localizing pituitary microadenomas, although rarely an adenoma is detected exclusively by DMRI. SGE should be part of the standard MRI protocol for patients with Cushing's disease.
Assuntos
Adenoma/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Hipersecreção Hipofisária de ACTH/diagnóstico por imagem , Neoplasias Hipofisárias/diagnóstico por imagem , Adenoma/cirurgia , Adolescente , Hormônio Adrenocorticotrópico/metabolismo , Adulto , Idoso , Imagem de Difusão por Ressonância Magnética/métodos , Feminino , Humanos , Processamento de Imagem Assistida por Computador , Masculino , Pessoa de Meia-Idade , Hipersecreção Hipofisária de ACTH/cirurgia , Hipófise/diagnóstico por imagem , Hipófise/cirurgia , Neoplasias Hipofisárias/cirurgia , Adulto JovemRESUMO
BACKGROUND: Complete or partial agenesis of the falx cerebri may occur in pediatric patients with developmental anomalies. However, isolated agenesis of the falx in a developmentally normal adult is exceptionally rare. We describe the first reported case of a patient with a third ventricular mass associated with partial agenesis of the anterior falx cerebri, a circumstance that influenced surgical access to a third ventricular epidermoid cyst. CASE DESCRIPTION: A 60-year-old developmentally normal woman presented with progressively worsening aphasia and altered mental status. Brain magnetic resonance imaging showed obstructive hydrocephalus from a third ventricular mass. An anterior interhemispheric transcallosal approach was planned to remove the tumor. However, upon dural opening there was no evidence of a falx cerebri, an anomaly visible but not reported on the prior imaging studies. An interhemispheric fissure was present, but the medial frontal lobes were densely adherent, with multiple traversing veins within the superficial arachnoid of the fissure. Therefore, a left frontal transcortical approach was performed for microsurgical resection of the tumor. Histopathologic analysis identified the lesion to be an epidermoid cyst. CONCLUSIONS: Partial agenesis of the falx cerebri is exceedingly rare in a developmentally normal adult, particularly in the presence of an anatomically normal superior sagittal sinus. If present, however, it is important to note this association preoperatively because partial agenesis of the falx cerebri precludes an interhemispheric transcallosal approach to the lateral and third ventricles.
Assuntos
Neoplasias do Ventrículo Cerebral/cirurgia , Procedimentos Neurocirúrgicos/métodos , Medula Espinal/anormalidades , Terceiro Ventrículo/cirurgia , Neoplasias do Ventrículo Cerebral/diagnóstico por imagem , Feminino , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Medula Espinal/diagnóstico por imagem , Terceiro Ventrículo/diagnóstico por imagemRESUMO
OBJECTIVE Extracapsular resection of pituitary microadenomas improves remission rates, but the application of pseudocapsular techniques for macroadenomas has not been well described. In larger tumors, the extremely thin, compressed normal gland or its complete absence along the tumor's anterior surface limits the application of the traditional pseudocapsular technique that can be used for microadenomas. However, in the authors' experience, the interface between the pseudocapsule at the posterior margin of the adenoma and the compressed normal gland behind it is universally present, providing a surgical dissection plane. In mid-2010, the authors began using a new surgical technique to identify and use this interface for the resection of larger macroadenomas, a technique that can be used with the microscope or the endoscope. METHODS The authors performed a cohort study using prospectively collected preoperative imaging reports and operative details and retrospectively reviewed postoperative images and clinical follow-up of patients with a pituitary macroadenoma 20-40 mm in maximum diameter undergoing microscopic transsphenoidal resection. Since dissection of the tumor capsule only pertains to encapsulated tumor within the sella and not to tumor invading the cavernous sinus, assessment of tumor removal of noninvasive tumors emphasized the entire tumor, while that of invasive tumors emphasized the intrasellar component only. The incidence of residual tumor on postoperative imaging, new-onset endocrinopathy, and recovery of preoperative pituitary deficits was compared between patients who underwent surgery before (Group A) and after (Group B) implementation of the new technique. RESULTS There were 34 consecutive patients in Group A and 74 consecutive patients in Group B. Tumors in 18 (53%) Group A and 40 (54%) Group B patients had no evidence of cavernous sinus invasion on MRI. Use of the posterior pseudocapsule technique reduced the incidence of intrasellar residual tumor on postoperative MRI for tumors without cavernous sinus invasion (39% [Group A] vs 10%, p < 0.05) and in all tumors regardless of invasion (50% vs 18%, p < 0.005). The incidence of new endocrinopathy was less likely (25% vs 12%, p = 0.098) and the recovery of prior deficits more likely (13% vs 27%, p = 0.199) among patients treated using the pseudocapsule approach, although the differences are not statistically significant. CONCLUSIONS Use of the posterior pseudocapsule dissection plane can enhance the resection of pituitary macroadenomas.
Assuntos
Adenoma/cirurgia , Procedimentos Neurocirúrgicos/métodos , Neoplasias Hipofisárias/cirurgia , Adenoma/diagnóstico por imagem , Estudos de Coortes , Humanos , Incidência , Imageamento por Ressonância Magnética , Margens de Excisão , Microcirurgia , Neoplasia Residual/epidemiologia , Neoplasia Residual/cirurgia , Doenças da Hipófise/epidemiologia , Doenças da Hipófise/etiologia , Neoplasias Hipofisárias/diagnóstico por imagem , Complicações Pós-Operatórias/epidemiologia , Estudos Prospectivos , Resultado do TratamentoRESUMO
BACKGROUND: Cauda equina hemangioblastomas in von Hippel-Lindau (VHL) disease can cause significant neurological signs and symptoms. Despite their associated morbidity, the management of these tumors remains incompletely defined. OBJECTIVE: To determine optimal management, we analyzed the functional outcomes after resection of these tumors. METHODS: VHL patients who underwent surgical resection of cauda equina hemangioblastomas at the National Institutes of Health and the University of Virginia were included. Clinical and radiological follow-up was performed at 6- to 12-month intervals after surgery. RESULTS: Fifteen patients underwent 18 operations for 21 cauda equina hemangioblastomas (median follow-up 5.9 years). Patients often presented with multiple symptoms, including pain (67%), numbness (50%), urinary complaints (33%), and weakness (11%). Median preoperative tumor volume was 1.2 cm 3 . Four tumors at 3 operations were not resected due to a motor nerve root origin. Gross total resection was achieved in 14 surgeries (93% of operations when resection was attempted). New mild (non-function limiting) neurological symptoms were noted after 11 operations (61%), which most often (64%) resolved within 2 weeks of surgery. At 6-month follow-up, 15 patients (83%) were stable, 2 (11%) were improved, and 1 (6%) was worse. Histological analysis revealed that all tumors originated from within the involved nerve fascicle. CONCLUSIONS: VHL-associated cauda equina hemangioblastomas have an intrafascicular origin and require interruption of the rootlet of origin for complete resection. Motor nerve root involvement may preclude complete resection but strategies including bony decompression and/or interruption of vascular supply may provide a therapeutic option. Nevertheless, most VHL patients with symptom-producing lesions improve with resection.
Assuntos
Cauda Equina/patologia , Neoplasias Cerebelares/cirurgia , Hemangioblastoma/cirurgia , Laminectomia/métodos , Resultado do Tratamento , Doença de von Hippel-Lindau/cirurgia , Adulto , Cauda Equina/diagnóstico por imagem , Neoplasias Cerebelares/complicações , Estudos de Coortes , Feminino , Hemangioblastoma/complicações , Hemangioblastoma/terapia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Exame Neurológico , Tomógrafos Computadorizados , Adulto Jovem , Doença de von Hippel-Lindau/complicaçõesRESUMO
Context: Perioperative increases in adrenocorticotropic hormone (ACTH) and cortisol mimic results of corticotropin-releasing hormone (CRH) stimulation testing. This phenomenon may help identify patients with residual adenoma after transsphenoidal surgery (TSS) for Cushing disease (CD). Objective: To predict nonremission after TSS for CD. Design: Retrospective case-control study of patients treated at a single center from December 2003 until July 2016. Early and medium-term remission were assessed at 10 days and 11 months. Patients and Setting: Two hundred and ninety-one consecutive TSS cases from 257 patients with biochemical evidence of CD seen at a clinical center. Interventions: Normalized early postoperative values (NEPVs) for cortisol and ACTH were calculated as immediate postoperative cortisol or ACTH levels minus preoperative post-CRH-stimulation test levels. Main Outcome Measures: Prediction of early nonremission was evaluated using logistic regression. Prediction of medium-term remission was assessed using Cox regression. Predictive ability was quantified by area under the receiver operating characteristic curve (AUROC). Results: NEPVs for cortisol and ACTH predicted early nonremission [adjusted odds ratio (OR): 1.1; 95% confidence interval (CI): 1.0, 1.1; P = 0.016 and adjusted OR: 1.0; 95% CI: 1.0, 1.0; P = 0.048, respectively]. AUROC for NEPV of cortisol was 0.78 (95% CI: 0.61, 0.95); for NEPV of ACTH, it was 0.80 (95% CI: 0.61, 0.98). NEPVs for cortisol and ACTH predicted medium-term nonremission [hazard ratio (HR): 1.1; 95% CI: 1.0, 1.1; P = 0.023 and HR: 1.0; 95% CI: 1.0, 1.0; P = 0.025, respectively]. Conclusions: NEPVs for cortisol and ACTH predicted nonremission after TSS for CD.
Assuntos
Hormônio Adrenocorticotrópico/sangue , Hidrocortisona/sangue , Hipersecreção Hipofisária de ACTH/cirurgia , Adulto , Fatores Etários , Biomarcadores/sangue , Feminino , Humanos , Masculino , Hipersecreção Hipofisária de ACTH/sangue , Período Pós-Operatório , Valor Preditivo dos Testes , Prognóstico , Valores de Referência , Indução de Remissão , Estudos Retrospectivos , Fatores SexuaisAssuntos
Hipófise/fisiologia , Neoplasias Hipofisárias/patologia , Neoplasias Hipofisárias/fisiopatologia , Prolactinoma/patologia , Prolactinoma/fisiopatologia , Carga Tumoral , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Carcinoma/diagnóstico , Carcinoma/fisiopatologia , Carcinoma/secundário , Cisplatino/administração & dosagem , Fluoruracila/administração & dosagem , Humanos , Masculino , Carcinoma Nasofaríngeo , Neoplasias Nasofaríngeas/diagnóstico , Neoplasias Nasofaríngeas/fisiopatologia , Neoplasias Nasofaríngeas/secundário , Tamanho do Órgão , Testes de Função Hipofisária , Hipófise/patologia , Hipófise/fisiopatologia , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/tratamento farmacológico , Prolactinoma/diagnóstico , Prolactinoma/tratamento farmacológico , Indução de Remissão , Carga Tumoral/efeitos dos fármacosRESUMO
Cushing's disease (CD) is the result of excess secretion of adrenocorticotropic hormone (ACTH) by a benign monoclonal pituitary adenoma. The excessive secretion of ACTH stimulates secretion of cortisol by the adrenal glands, resulting in supraphysiological levels of circulating cortisol. The pathophysiological levels of cortisol are associated with hypertension, diabetes, obesity, and early death. Successful resection of the CD-associated ACTH-secreting pituitary adenoma is the treatment of choice and results in immediate biochemical remission with preservation of pituitary function. Accurate and early identification of CD is critical for effective surgical management and optimal prognosis. The authors review the current pathophysiological principles, diagnostic methods, and management of CD.
Assuntos
Hipersecreção Hipofisária de ACTH , Humanos , Hipersecreção Hipofisária de ACTH/diagnóstico , Hipersecreção Hipofisária de ACTH/fisiopatologia , Hipersecreção Hipofisária de ACTH/terapiaRESUMO
BACKGROUND: In the immediate postoperative period following resection of growth hormone (GH)-secreting pituitary tumors, serum concentrations of GH have limited ability to predict remission of acromegaly. Since many actions of GH actions are mediated by insulin-like growth factor-1 (IGF-I), we aimed to determine the rates of fall of IGF-I during 72 h after surgical resection of pituitary tumors. METHODS: We studied patients who were undergoing pituitary surgery for acromegaly. IGF-I was measured by LC-MS and GH by immunoassay. Remission was defined by the combination of serum GH <0.4 ng/mL during oral glucose tolerance testing performed 8 weeks after the surgical procedure and normal IGF-I at ≥8 weeks. RESULTS: During the first 72 h after surgery, the mean (SD) rate of decline of IGF-I was 185 (61) ng/mL per 24 h in those who achieved remission (n = 23), with a mean (SD) apparent half-life of 55 (19) h. IGF-I had decreased to <65% of the preoperative IGF-I on postoperative day 2 in 20 of 23 remission patients (87%) vs none of 5 patients who did not achieve remission. GH was <2.7 ng/mL on day 2 in 21 of 23 remission patients (91%), but in none of the nonremission patients. The combination of IGF-I and GH on day 2 separated the remission and nonremission groups of patients. CONCLUSIONS: Rapid decline of serum IGF-I during the immediate postoperative period warrants further study as an analytically independent adjunct to GH measurement for early prediction of biochemical remission of acromegaly.
Assuntos
Acromegalia/cirurgia , Fator de Crescimento Insulin-Like I/análise , Neoplasias Hipofisárias/cirurgia , Acromegalia/sangue , Acromegalia/metabolismo , Feminino , Humanos , Fator de Crescimento Insulin-Like I/metabolismo , Masculino , Neoplasias Hipofisárias/sangue , Neoplasias Hipofisárias/metabolismoRESUMO
OBJECTIVE The relationship between growth hormone (GH) and insulin-like growth factor-1 (IGF-1) in patients with acromegaly as serial levels drop over time after treatment has not been examined previously. Knowledge of this relationship is important to correlate pretreatment levels that best predict response to treatment. To examine the correlation between GH and IGF-1 and IGF-1 z-scores over a wide range of GH levels, the authors examined serial GH and IGF-1 levels at intervals before and after surgery and radiosurgery for acromegaly. METHODS This retrospective analysis correlates 414 pairs of GH and IGF-1 values in 93 patients with acromegaly. RESULTS Absolute IGF-1 levels increase linearly with GH levels only up to a GH of 4 ng/ml, and with IGF-1 z-scores only to a GH level of 1 ng/ml. Between GH levels of 1 and 10 ng/ml, increases in IGF-1 z-scores relative to changes in GH diminish and then plateau at GH concentrations of about 10 ng/ml. From patient to patient there is a wide range of threshold GH levels beyond which IGF-1 increases are no longer linear, GH levels at which the IGF-1 response plateaus, IGF-1 levels at similar GH values after the IGF-1 response plateaus, and of IGF-1 levels at similar GH levels. CONCLUSIONS In acromegaly, although IGF-1 levels represent a combination of the integrated effects of GH secretion and GH action, the tumor produces GH, not IGF-1. Nonlinearity between GH and IGF-1 occurs at GH levels far below those previously recognized. To monitor tumor activity and tumor viability requires measurement of GH levels.
Assuntos
Acromegalia/sangue , Hormônio do Crescimento Humano/sangue , Fator de Crescimento Insulin-Like I/metabolismo , Acromegalia/tratamento farmacológico , Acromegalia/radioterapia , Acromegalia/cirurgia , Adolescente , Adulto , Idoso , Feminino , Hormônio do Crescimento Humano/análogos & derivados , Hormônio do Crescimento Humano/uso terapêutico , Humanos , Masculino , Pessoa de Meia-Idade , Radiocirurgia , Estudos Retrospectivos , Resultado do Tratamento , Adulto JovemRESUMO
OBJECTIVE A subset of patients with Chiari I malformation demonstrate patent subarachnoid spaces around the cerebellum, indicating that reduced posterior fossa volume alone does not account for tonsillar descent. The authors distinguish two subsets of Chiari I malformation patients based on the degree of "posterior fossa crowdedness" on MRI. METHODS Two of the coauthors independently reviewed the preoperative MR images of 49 patients with Chiari I malformation and categorized the posterior fossa as "spacious" or "crowded." Volumetric analysis of posterior fossa structures was then performed using open-source DICOM software. The preoperative clinical and imaging features of the two groups were compared. RESULTS The posterior fossae of 25 patients were classified as spacious and 20 as crowded by both readers; 4 were incongruent. The volumes of the posterior fossa compartment, posterior fossa tissue, and hindbrain (posterior fossa tissue including herniated tonsils) were statistically similar between the patients with spacious and crowed subtypes (p = 0.33, p = 0.17, p = 0.20, respectively). However, patients in the spacious and crowded subtypes demonstrated significant differences in the ratios of posterior fossa tissue to compartment volumes as well as hindbrain to compartment volumes (p = 0.001 and p = 0.0004, respectively). The average age at surgery was 29.2 ± 19.3 years (mean ± SD) and 21.9 ± 14.9 years for spacious and crowded subtypes, respectively (p = 0.08). Syringomyelia was more prevalent in the crowded subtype (50% vs 28%, p = 0.11). CONCLUSIONS The authors' study identifies two subtypes of Chiari I malformation, crowded and spacious, that can be distinguished by MRI appearance without volumetric analysis. Earlier age at surgery and presence of syringomyelia are more common in the crowded subtype. The presence of the spacious subtype suggests that crowdedness alone cannot explain the pathogenesis of Chiari I malformation in many patients, supporting the need for further investigation.
Assuntos
Malformação de Arnold-Chiari/diagnóstico por imagem , Fossa Craniana Posterior/diagnóstico por imagem , Imageamento por Ressonância Magnética , Adolescente , Adulto , Malformação de Arnold-Chiari/cirurgia , Criança , Fossa Craniana Posterior/cirurgia , Descompressão Cirúrgica/métodos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto JovemRESUMO
BACKGROUND: Carney complex (CNC) is a familial neoplasia syndrome that is associated with pituitary-associated hypersecretion of growth hormone (GH) (acromegaly). The underlying cause of pituitary GH hypersecretion and its management have been incompletely defined. OBJECTIVE: To provide biological insight into CNC-associated pituitary pathology and improve management, we analyzed findings in CNC patients who underwent transsphenoidal surgery. METHODS: Consecutive CNC patients at the National Institutes of Health with acromegaly and imaging evidence of a pituitary adenoma(s) who underwent transsphenoidal resection of tumor(s) were included. Prospectively acquired magnetic resonance imaging and biochemical, surgical, and histological data were analyzed. RESULTS: Seven acromegalic CNC patients (2 male, 5 female) were included. The mean age at surgery was 29.7 years (range, 18-44 years). The mean follow-up was 4.7 years (range, 0.2-129 months). Magnetic resonance imaging revealed a single pituitary adenoma in 4 patients and multiple pituitary adenomas in 3 patients. Whereas patients with single discrete pituitary adenomas underwent selective adenomectomy, patients with multiple adenomas underwent selective adenomectomy of multiple tumors, as well as partial or total hypophysectomy. All adenomas were either GH and prolactin positive or exclusively prolactin positive. Pituitary tissue surrounding the adenomas in patients with multiple adenomas revealed hyperplastic GH- and prolactin-positive tissue. CONCLUSION: CNC-associated acromegaly results from variable pituitary pathology, including a single GH-secreting adenoma or multiple GH-secreting adenomas and/or GH hypersecretion of the pituitary gland surrounding multiple adenomas. Although selective adenomectomy is the preferred treatment for cases of GH-secreting adenomas, multiple adenomas with associated pituitary gland GH hypersecretion may require partial or complete hypophysectomy to achieve biochemical remission.
Assuntos
Acromegalia/cirurgia , Adenoma/cirurgia , Complexo de Carney/cirurgia , Hormônio do Crescimento Humano/metabolismo , Doenças da Hipófise/cirurgia , Neoplasias Hipofisárias/cirurgia , Acromegalia/diagnóstico por imagem , Adenoma/diagnóstico por imagem , Adolescente , Adulto , Complexo de Carney/diagnóstico por imagem , Gerenciamento Clínico , Feminino , Humanos , Imageamento por Ressonância Magnética/efeitos adversos , Masculino , Doenças da Hipófise/diagnóstico por imagem , Hipófise/diagnóstico por imagem , Hipófise/metabolismo , Hipófise/cirurgia , Neoplasias Hipofisárias/diagnóstico por imagem , Adulto JovemRESUMO
BACKGROUND: The coincidence of parasellar aneurysms (IAs) and anterior skull base (ASB) lesions, while rare, presents a management challenge. IAs embedded within, or adjacent to, ASB lesions are at risk in the perioperative period and may have unique presentations, natural histories, and outcomes. The objective of this study was to outline management options and nuances in patients with coexisting IAs and ASB lesions. METHODS: We retrospectively evaluated all patients who presented for management of IAs and ASB lesions from January 2006 to January 2014. Medical charts and imaging were reviewed for patient, tumor and IA characteristics, pathology, operative findings, complications, and outcomes. RESULTS: Of 13 patients included in the study, 11 had histologically proven or presumed pituitary macroadenomas. The majority of cases presented with visual and endocrine deficits, and the median maximal tumor diameter was 3.1 cm. There were 17 IAs, all located in the parasellar area. Endovascular treatment of the IA was performed before tumor resection in 2 cases. Transsphenoidal resection was performed before IA occlusion in 1 case, and intraoperative vascular injury occurred in 2 cases. The median follow-up was 36 months. CONCLUSIONS: Management decisions for patients with coincident IA and ASB lesions require careful, individualized treatment plans. Coil embolization is well tolerated and does not delay surgery, except in cases requiring stent placement. Inadvertent intraoperative rupture of an adjacent IA during anterior skull base surgery may be treated with emergent coil embolization, flow diversion, or carotid sacrifice, but adequate preoperative planning can reduce this risk.
Assuntos
Adenoma/cirurgia , Procedimentos Endovasculares/métodos , Aneurisma Intracraniano/cirurgia , Neuroendoscopia/métodos , Neoplasias Hipofisárias/cirurgia , Adenoma/complicações , Adulto , Idoso , Idoso de 80 Anos ou mais , Angiografia Cerebral , Angiografia por Tomografia Computadorizada , Feminino , Humanos , Aneurisma Intracraniano/complicações , Masculino , Pessoa de Meia-Idade , Procedimentos Neurocirúrgicos/métodos , Neoplasias Hipofisárias/complicações , Estudos Retrospectivos , Base do CrânioRESUMO
von Hippel-Lindau (VHL) patients develop multiple central nervous system hemangioblastomas (HB). Some HBs become symptomatic with exponential growth or cyst formation following long periods of quiescence. Understanding the factors underlying growth in hemangioblastoma may lead to better strategies to arrest or prevent tumor growth. In 5 VHL patients, we resected quiescent hemangioblastomas (Q-HB) that were en-route during surgical access to symptomatic hemangioblastomas (S-HB), for matched tumor analysis. Quantitative reverse transcriptase analysis demonstrated a 2-fold increase in EPO expression in all S-HB, while 4/5 showed either Hypoxia Inducible Factor-1α or 2α upregulation. Additionally, all S-HB had increased phosphorylated erythropoietin (EPO) receptor and phosphorylated STAT-5 relative to matched Q-HB, with increased phosphorylated JAK-2 largely confined to the stromal cells in clusters within the tumors. These findings suggest that Q-HB to S-HB conversion may be associated with an erythropoietin-signaling loop. Furthermore, we found that EPO is detectable in cyst fluid from S-HB (n = 14), while absent in CSF (n = 1). Additionally, S-HB presentation or S-HB resection does not result in discernible change in serum EPO or hemoglobin (n = 60). These observations suggest that the altered erythropoietin signaling is focal and suggests that studying modulation of erythropoietin receptor pathway may lead to strategies in preventing HB growth.
Assuntos
Eritropoetina/metabolismo , Hemangioblastoma/etiologia , Hemangioblastoma/metabolismo , Transdução de Sinais , Doença de von Hippel-Lindau/etiologia , Doença de von Hippel-Lindau/metabolismo , Adulto , Biomarcadores , Feminino , Hemangioblastoma/diagnóstico , Humanos , Masculino , Pessoa de Meia-Idade , Radiografia , Índice de Gravidade de Doença , Doença de von Hippel-Lindau/diagnósticoRESUMO
This report describes the circumstances of a patient with a cauda equina syndrome due to the development of a lumbar subdural CSF collection with ventral displacement of the cauda equina shortly following posterior fossa decompression for Chiari malformation Type I (CM-I). This unusual, but clinically significant, complication was successfully treated with percutaneous drainage of the extraarachnoid CSF collection. Although there are a few cases of intracranial subdural hygroma developing after surgery for CM-I, often attributed to a pinhole opening in the arachnoid, as far as the authors can determine, a spinal subdural hygroma associated with surgery for CM-I has not been recognized.
Assuntos
Malformação de Arnold-Chiari/cirurgia , Descompressão Cirúrgica/efeitos adversos , Polirradiculopatia/etiologia , Complicações Pós-Operatórias , Derrame Subdural/etiologia , Adulto , Malformação de Arnold-Chiari/complicações , Malformação de Arnold-Chiari/diagnóstico por imagem , Descompressão Cirúrgica/métodos , Drenagem/métodos , Feminino , Humanos , Vértebras Lombares/diagnóstico por imagem , Polirradiculopatia/diagnóstico por imagem , Polirradiculopatia/terapia , Complicações Pós-Operatórias/diagnóstico por imagem , Complicações Pós-Operatórias/terapia , Derrame Subdural/diagnóstico por imagem , Derrame Subdural/terapia , Vértebras Torácicas/diagnóstico por imagemRESUMO
OBJECTIVE Pituitary MR imaging fails to detect over 50% of microadenomas in Cushing's disease and nearly 80% of cases of dural microinvasion. Surface coils can generate exceptionally high-resolution images of the immediately adjacent tissues. To improve imaging of the pituitary gland, a receive-only surface coil that can be placed within the sphenoid sinus (the endosphenoidal coil [ESC]) during transsphenoidal surgery (TSS) was developed and assessed. METHODS Five cadaver heads were used for preclinical testing of the ESC. The ESC (a double-turn, 12-mm-diameter surface coil made from 1-mm-diameter copper wire) was developed to obtain images in a 1.5-T MR scanner. The ESC was placed (via a standard sublabial TSS approach) on the anterior sella face. Clinical MR scans were obtained using the 8-channel head coil and ESC as the receiver coils. Using the ESC, ultra-high-resolution, 3D, balanced fast field echo (BFFE) and T1-weighted imaging were performed at resolutions of 0.25 × 0.25 × 0.50 mm3 and 0.15 × 0.15 × 0.30 mm3, respectively. RESULTS Region-of-interest analysis indicated a 10-fold increase in the signal-to-noise ratio (SNR) of the pituitary when using the ESC compared with the 8-channel head coil. ESC-related improvements (p < 0.01) in the SNR were inversely proportional to the distance from the ESC tip to the anterior pituitary gland surface. High-resolution BFFE MR imaging obtained using ESC revealed a number of anatomical features critical to pituitary surgery that were not visible on 8-channel MR imaging, including the pituitary capsule, the intercavernous sinus, and microcalcifications in the pars intermedia. These ESC imaging findings were confirmed by the pathological correlation with whole-mount pituitary sections. CONCLUSIONS ESC can significantly improve SNR in the sellar region intraoperatively using current 1.5-T MR imaging platforms. Improvement in SNR can provide images of the sella and surrounding structures with unprecedented resolution. Clinical use of this ESC may allow for MR imaging detection of previously occult pituitary adenomas and identify microscopic invasion of the dura or cavernous sinus.
Assuntos
Imageamento por Ressonância Magnética/instrumentação , Monitorização Intraoperatória/métodos , Hipófise/diagnóstico por imagem , Hipófise/cirurgia , Cadáver , Humanos , Procedimentos Neurocirúrgicos/métodos , Seio EsfenoidalRESUMO
OBJECTIVE: Peritumoral cysts are frequently associated with CNS hemangioblastomas and often underlie neurological morbidity and mortality. To determine their natural history and clinical impact, the authors prospectively analyzed hemangioblastoma-associated peritumoral cysts in patients with von Hippel-Lindau (VHL) disease. METHODS: Patients with VHL disease who had 2 or more years of follow-up and who were enrolled in a prospective study at the National Institutes of Health were included. Serial prospectively acquired laboratory, genetic, imaging, and clinical data were analyzed. RESULTS: One hundred thirty-two patients (of 225 in the VHL study with at least 2 years of follow-up) had peritumoral cysts that were followed for more than 2 years (total of 292 CNS peritumoral cysts). The mean age at study entrance was 37.4 ± 13.1 years ([mean ± SD], median 37.9, range 12.3-65.1 years). The mean follow-up was 7.0 ± 1.7 years (median 7.3, range 2.1-9.0 years). Over the study period, 121 of the 292 peritumoral cysts (41.4%) became symptomatic. Development of new cysts was associated with a larger number cysts at study enrollment (p = 0.002) and younger age (p < 0.0001). Cyst growth rate was associated with anatomical location (cerebellum cysts grew faster than spine and brainstem cysts; p = 0.0002 and p = 0.0008), younger age (< 35 years of age; p = 0.0006), and development of new neurological symptoms (p < 0.0001). Cyst size at symptom production depended on anatomical location (p < 0.0001; largest to smallest were found, successively, in the cerebellum, spinal cord, and brainstem). The most common location for peritumoral cysts was the cerebellum (184 cysts [63%]; p < 0.0001). CONCLUSIONS: Peritumoral cysts frequently underlie symptom formation that requires surgical intervention in patients with VHL disease. Development of new cysts was associated with a larger number of cysts at study enrollment and younger age. Total peritumoral cyst burden was associated with germline partial deletion of the VHL gene.
Assuntos
Cistos/patologia , Hemangioblastoma/complicações , Hemangioblastoma/cirurgia , Doença de von Hippel-Lindau/patologia , Adolescente , Adulto , Idoso , Envelhecimento/patologia , Tronco Encefálico/patologia , Cerebelo/patologia , Criança , Efeitos Psicossociais da Doença , Cistos/genética , Cistos/cirurgia , Feminino , Seguimentos , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Doenças do Sistema Nervoso/etiologia , Doenças do Sistema Nervoso/fisiopatologia , Procedimentos Neurocirúrgicos/métodos , Estudos Prospectivos , Coluna Vertebral/patologia , Adulto Jovem , Doença de von Hippel-Lindau/genéticaRESUMO
OBJECT Both endoscopic and microscopic transsphenoidal approaches are accepted techniques for the resection of pituitary adenomas. Although studies have explored patient outcomes for each technique individually, none have prospectively compared sinonasal and quality of life outcomes in a concurrent series of patients at the same institution, as has been done in the present study. METHODS Patients with nonfunctioning adenomas undergoing transsphenoidal surgery were assessed for sinonasal function, quality of life, and pain using the Sino-Nasal Outcome Test-20 (SNOT-20), the short form of the Nasal Obstruction Symptom Evaluation (NOSE) instrument, the SF-36, and a headache scale. Eighty-two patients undergoing either endoscopic (47 patients) or microscopic (35 patients) surgery were surveyed preoperatively and at 24-48 hours, 2 weeks, 4 weeks, 8 weeks, and 1 year after surgery. RESULTS Patients who underwent endoscopic and microscopic transsphenoidal surgery experienced a similar recovery pattern, showing an initial increase in symptoms during the first 2 weeks, followed by a return to baseline by 4 weeks and improvement beyond baseline functioning by 8 weeks. Patients who underwent endoscopic surgery experienced better sinonasal outcomes at 24-48 hours (SNOT total p = 0.015, SNOT rhinologic subscale [ssRhino] p < 0.001), 2 weeks (NOSE p = 0.013), and 8 weeks (SNOT total p = 0.032 and SNOT ssRhino p = 0.035). By 1 year after surgery, no significant differences in sinonasal outcomes were observed between the 2 groups. Headache scales at 1 year improved in all dimensions except duration for both groups (total result 73%, p = 0.004; severity 46%, p < 0.001; frequency 53%, p < 0.001), with 80% of either microscopic or endoscopic patients experiencing improvement or resolution of headache symptoms. Endoscopic and microscopic patients experienced reduced vitality preoperatively compared with US population norms and remained low postoperatively. By 8 weeks after surgery, both groups experienced significant improvements in mental health (13%, p = 0.005) and vitality (15%, p = 0.037). By 1 year after surgery, patients improved significantly in mental health (14%, p = 0.03), role physical (14%, p = 0.036), social functioning (16%, p = 0.009), vitality (22%, p = 0.002), and SF-36 total (10%, p = 0.024) as compared with preoperative measures. There were no significant differences at any time point between the 2 groups for the total SF-36 or for any of the 8 subscales. CONCLUSIONS Patients who underwent either an endoscopic or a microscopic approach experienced the greatest nasal symptoms at 2 weeks postoperatively and exhibited similar time courses of recovery in nasal, headache, and quality of life assessments. Although patients who underwent endoscopic surgery experienced significantly fewer nasal symptoms during the first 8 weeks, by 1 year after surgery, there were no significant differences between the 2 groups.