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PURPOSE: To highlight the role of in vivo magnetic resonance spectroscopy (MRS) as a non-invasive tool that can clarify the etiology of sellar tumors by presenting the case of a boy with central precocious puberty (CPP) and to review the current literature. METHODS: A 4-year-old boy was admitted to our hospital due to repeated episodes of focal and gelastic seizures in the previous year. Clinical examination (testicular volume 4-5 ml bilaterally, penile length of 7.5 cm, and absence of axillary or pubic hair) and laboratory tests (FSH, LH, and testosterone) were indicative of CPP. The combination of gelastic seizures with CPP in a 4-year-old boy raised the suspicion of hypothalamic hamartoma (HH). Brain MRI revealed a lobular mass in the suprasellar-hypothalamic region. The differential diagnosis included glioma, HH, and craniopharyngioma. To further investigate the CNS mass, an in vivo brain MRS was performed. RESULTS: Οn conventional MRI, the mass demonstrated isointensity to gray matter on T1 weighted images but slight hyperintensity on T2-weighted images. It did not show restricted diffusion or contrast enhancement. On MRS, it showed reduced N-acetyl aspartate (NAA) and slightly elevated myoinositol (MI) compared with values in normal deep gray matter. The MRS spectrum, in combination with the conventional MRI findings, were consistent with the diagnosis of a HH. CONCLUSION: MRS is a state-of-the-art, non-invasive imaging technique that compares the chemical composition of normal tissue to that of abnormal regions by juxtaposing the frequency of measured metabolites. MRS, in combination with clinical evaluation and classic MRI, can provide identification of CNS masses, thus eliminating the need for an invasive biopsy.
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Hamartoma , Doenças Hipotalâmicas , Puberdade Precoce , Pré-Escolar , Humanos , Masculino , Diagnóstico Diferencial , Hamartoma/complicações , Hamartoma/diagnóstico , Doenças Hipotalâmicas/diagnóstico , Imageamento por Ressonância Magnética , Espectroscopia de Ressonância Magnética , Puberdade Precoce/diagnóstico , Puberdade Precoce/etiologia , Convulsões/complicações , Convulsões/diagnósticoRESUMO
Introduction: Radiotherapy of central nervous system (CNS) is treatment against many paediatric cancers, even if it is a well-recognized risk factor for meningioma formation. An increased risk of developing secondary brain tumors like radiation-induced meningiomas (RIM) is related to irradiated patients. Research question: This retrospective study aims to present RIM cases treated in a single tertiary-hospital in Greece and compare the results with international literature and cases of sporadic meningiomas. Materials and methods: A single-centre retrospective study of all patients diagnosed between January 2012 and September 2022 with RIM after having been irradiated in CNS for paediatric cancer was undertaken through hospital's electronic record and clinical notes, identifying baseline demographics and latency period. Results: Thirteen patients were identified with RIM diagnosis after receiving irradiation for Acute Lymphoblastic Leukaemia (69.2%), Premature Neuro-Ectodermal Tumour (23.1%), and Astrocytoma (7.7%). Median age at irradiation was 5 years old and 32 years old at RIM's presentation. The latent period from irradiation to meningioma diagnosis was 26.23 â± â5.96 years. After surgical excision, histopathologic results showed grade I meningiomas in 12 out of thirteen cases, while only one atypical meningioma was diagnosed. Conclusion: Patients who underwent CNS-radiotherapy in childhood for any condition have an increased risk of developing secondary brain tumors such as radiation-induced meningiomas. RIMs resemble sporadic meningiomas in symptomatology, location, treatment, and histologic grade. However, long-term follow-up and regular check-ups are recommended in irradiated patients due to short latency period from irradiation to RIM development, which means younger age patients than those with sporadic meningiomas cases.
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Primary intraosseous cavernous hemangioma (PICH) is a rare, benign tumor of vascular origin, typically arising in the vertebral body. Its presence in the skull is exceedingly rare, with only a few cases being reported worldwide. We carried out the first systematic review of the literature, covering the epidemiology, clinical and imaging features, management, and prognosis of cranial PICH. The literature search revealed 51 studies with 77 patients; the mean age of the patients was 32.7 years with a female predominance of 1.4:1. The majority of cranial PICHs were located in the calvarium, primarily in the frontal and parietal regions, with only a few located in the skull base. The most common initial clinical manifestation was local growth or swelling, followed by a headache. Radiographically, PICHs represented osteolytic, intradiploic masses, which in many cases displayed trabeculations, leading to the so-called "honeycomb" or "starburst" pattern. After contrast administration, PICHs typically enhance. Tumor removal, with craniectomy or en bloc resection and subsequent skull reconstruction, was selected for calvarial PICHs, whereas a transsphenoidal approach, with only partial resection, was applied for clival/sella PICHs. Preoperative embolization, aiming to minimize intraoperative blood loss, was performed in the case of large tumors. At a mean follow-up of 39 months, no patient experienced tumor recurrence, even after subtotal resection. Owing to the benign nature of the tumor, maximal safe resection is recommended as the treatment of choice for patients with cranial PICH.
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Hemangioma Cavernoso , Neoplasias Cranianas , Neoplasias Vasculares , Adulto , Feminino , Hemangioma Cavernoso/diagnóstico por imagem , Hemangioma Cavernoso/patologia , Hemangioma Cavernoso/cirurgia , Humanos , Masculino , Recidiva Local de Neoplasia , Crânio/anormalidades , Crânio/patologia , Crânio/cirurgia , Neoplasias Cranianas/diagnóstico por imagem , Neoplasias Cranianas/patologia , Neoplasias Cranianas/cirurgia , Coluna Vertebral/anormalidades , Malformações VascularesRESUMO
BACKGROUND: The SIOP-Renal Tumor Study Group (RTSG) does not advocate invasive procedures to determine histology before the start of therapy. This may induce misdiagnosis-based treatment initiation, but only for a relatively small percentage of approximately 10% of non-Wilms tumors (non-WTs). MRI could be useful for reducing misdiagnosis, but there is no global consensus on differentiating characteristics. PURPOSE: To identify MRI characteristics that may be used for discrimination of newly diagnosed pediatric renal tumors. STUDY TYPE: Consensus process using a Delphi method. POPULATION: Not applicable. FIELD STRENGTH/SEQUENCE: Abdominal MRI including T1- and T2-weighted imaging, contrast-enhanced MRI, and diffusion-weighted imaging at 1.5 or 3 T. ASSESSMENT: Twenty-three radiologists from the SIOP-RTSG radiology panel with ≥5 years of experience in MRI of pediatric renal tumors and/or who had assessed ≥50 MRI scans of pediatric renal tumors in the past 5 years identified potentially discriminatory characteristics in the first questionnaire. These characteristics were scored in the subsequent second round, consisting of 5-point Likert scales, ranking- and multiple choice questions. STATISTICAL TESTS: The cut-off value for consensus and agreement among the majority was ≥75% and ≥60%, respectively, with a median of ≥4 on the Likert scale. RESULTS: Consensus on specific characteristics mainly concerned the discrimination between WTs and non-WTs, and WTs and nephrogenic rest(s) (NR)/nephroblastomatosis. The presence of bilateral lesions (75.0%) and NR/nephroblastomatosis (65.0%) were MRI characteristics indicated as specific for the diagnosis of a WT, and 91.3% of the participants agreed that MRI is useful to distinguish NR/nephroblastomatosis from WT. Furthermore, all participants agreed that age influenced their prediction in the discrimination of pediatric renal tumors. DATA CONCLUSION: Although the discrimination of pediatric renal tumors based on MRI remains challenging, this study identified some specific characteristics for tumor subtypes, based on the shared opinion of experts. These results may guide future validation studies and innovative efforts. LEVEL OF EVIDENCE: 3 Technical Efficacy Stage: 3.
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Neoplasias Renais , Radiologia , Tumor de Wilms , Técnica Delphi , Imagem de Difusão por Ressonância Magnética , Humanos , Neoplasias Renais/diagnóstico por imagemAssuntos
Ascite/cirurgia , Proteínas do Líquido Cefalorraquidiano/líquido cefalorraquidiano , Átrios do Coração/cirurgia , Hidrocefalia/cirurgia , Complicações Pós-Operatórias/cirurgia , Derivação Ventriculoperitoneal , Albuminas/líquido cefalorraquidiano , alfa-Globulinas/líquido cefalorraquidiano , Ascite/líquido cefalorraquidiano , Derivações do Líquido Cefalorraquidiano , Feminino , Humanos , Neoplasias Meníngeas/cirurgia , Meningioma/cirurgia , Pessoa de Meia-Idade , Neoplasias Primárias Múltiplas/cirurgia , Paracentese , Complicações Pós-Operatórias/líquido cefalorraquidiano , Pré-Albumina/líquido cefalorraquidiano , ReoperaçãoRESUMO
This study is designed based on the retrospective analysis of patients treated in the Neurosurgical Department of two major hospitals and review of the literature. The aim of this study is to evaluate the efficacy of surgery and address controversial issues in the treatment of symptomatic lumbar intraspinal synovial cysts. Spinal juxtafacet cysts (synovial and ganglion cysts) are a rare cause of low back and radicular leg pain. Although the relevant reports in the international literature are increasing, the controversy about conservative versus surgical treatment and the need for concomitant fusion still exists. Data from seven patients (age range 58-69 years, mean age 61 years) with low back and radicular leg pain due to a lumbar facet joint cyst were retrospectively analyzed. Demographic data, cyst level, presence of concomitant local pathology, treatment and results of treatment were recorded. A follow-up of at least 6 months (range 6-48 months) was conducted and results were noted. All patients had back pain, while five also experienced unilateral radicular leg pain and one had bilateral leg pain. One patient had neurogenic claudication. MRI identified the cyst and highlighted underlying pathology in all cases. All patients underwent surgical cyst excision. No fusion was performed. Post-operatively, all patients showed a total resolution of symptoms with sustained benefit at final evaluation. Review of the literature revealed a trend towards surgery, as this is correlated to a more favorable outcome compared with conservative treatment. Fusion should be performed on a case-by-case basis only. Surgery is a safe and effective treatment choice in this increasingly appearing ailment. A prospective, randomized trial should clarify issues under debate.