RESUMO
Erdheim-Chester disease (ECD) is a rare, multisystemic, inflammatory, non-Langerhans cell histiocytic neoplasm. The discovery of recurrent and somatic mutations in the mitogen-activated protein kinase signaling pathway, most commonly BRAFV600E, has led to a reclassification of ECD from an inflammatory disorder to a neoplastic process. It is now included in the revised 2016 World Health Organization classification of hematopoietic tumors and in the Langerhans group in the revised 2016 Histiocytosis Classification of the Histiocyte Society. When symptomatic, ECD most commonly manifests with bone pain and fatigue. Also, neurologic manifestations, central diabetes insipidus, exophthalmos, and periorbital xanthelasma-like lesions are frequently encountered. Pathologic findings may vary depending on the site of biopsy and may display a spectrum of features. Thus, due to the diverse clinical presentation and variable histologic findings, imaging can often show the first sign of the disease. Radiologic findings are, however, interpreted in conjunction with clinical and histologic findings to establish the diagnosis of ECD. From providing classic findings that facilitate diagnosis to helping radiologists determine the extent of disease and predicting a prognosis, the role of radiology in ECD has evolved with the understanding of the disease itself. Insights into the molecular pathogenesis and the development of targeted therapeutic agents along with approval of vemurafenib and cobimetinib have necessitated revision of the guidelines for the management of ECD. The authors discuss various radiologic findings of ECD and differential diagnoses by using an organ system-based approach and briefly describe the revised consensus recommendations for evaluation, diagnosis, and treatment based on the International Medical Symposia on ECD from a radiologist's perspective. ©RSNA, 2024 Supplemental material is available for this article. The full digital presentation is available online.
Assuntos
Doença de Erdheim-Chester , Doença de Erdheim-Chester/diagnóstico por imagem , Humanos , Diagnóstico DiferencialRESUMO
Meningiomas are the most common primary intracranial tumors and can be associated with significant morbidity and mortality. Radiologists, neurosurgeons, neuro-oncologists, and radiation oncologists rely on brain MRI for diagnosis, treatment planning, and longitudinal treatment monitoring. However, automated, objective, and quantitative tools for non-invasive assessment of meningiomas on multi-sequence MR images are not available. Here we present the BraTS Pre-operative Meningioma Dataset, as the largest multi-institutional expert annotated multilabel meningioma multi-sequence MR image dataset to date. This dataset includes 1,141 multi-sequence MR images from six sites, each with four structural MRI sequences (T2-, T2/FLAIR-, pre-contrast T1-, and post-contrast T1-weighted) accompanied by expert manually refined segmentations of three distinct meningioma sub-compartments: enhancing tumor, non-enhancing tumor, and surrounding non-enhancing T2/FLAIR hyperintensity. Basic demographic data are provided including age at time of initial imaging, sex, and CNS WHO grade. The goal of releasing this dataset is to facilitate the development of automated computational methods for meningioma segmentation and expedite their incorporation into clinical practice, ultimately targeting improvement in the care of meningioma patients.
Assuntos
Imageamento por Ressonância Magnética , Neoplasias Meníngeas , Meningioma , Meningioma/diagnóstico por imagem , Humanos , Neoplasias Meníngeas/diagnóstico por imagem , Masculino , Feminino , Processamento de Imagem Assistida por Computador/métodos , Pessoa de Meia-Idade , IdosoRESUMO
The "puffed cheek" technique is routinely performed during CT neck studies in patients with suspected oral cavity cancers. The insufflation of air within the oral vestibule helps in the detection of small buccal mucosal lesions, with better delineation of lesion origin, depth, and extent of spread. The pitfalls associated with this technique are often underrecognized and poorly understood. They can mimic actual lesions, forfeiting the technique's primary purpose. This review provides an overview of the puffed cheek technique and its associated pitfalls. These pitfalls include pneumoparotid, soft palate elevation that resembles a nasopharyngeal mass, various tongue displacements or distortions that obscure tongue lesions or mimic them, sublingual gland herniation, an apparent exacerbation of the airway edema, vocal cord adduction that hinders glottic evaluation, and false indications of osteochondronecrosis in laryngeal cartilage. Most stem from a common underlying mechanism of unintentional Valsalva maneuver engaged in by the patient while trying to perform a puffed cheek, creating a closed air column under positive pressure with resultant surrounding soft-tissue displacement. These pitfalls can thus be avoided by instructing the patient to maintain continuous nasal breathing while puffing out their cheek during image acquisition, preventing the formation of the closed air column. Keywords: CT, Head/Neck © RSNA, 2024.
Assuntos
Bochecha , Tomografia Computadorizada por Raios X , Humanos , Bochecha/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Neoplasias Bucais/diagnóstico por imagem , Insuflação/métodosRESUMO
OBJECTIVE: To analyze clinical and radiographic features that may impact the rate of focal hyperostosis (FH) on computed tomography (CT) for primary and recurrent sinonasal inverted papillomas (IPs) as well as highlight factors that may affect concordance between FH and IP true attachment point (TAP). METHODS: All IPs resected between 2006 and 2022 were retrospectively reviewed. CTs were read by a neuroradiologist blinded to operative details. IP with malignancy was excluded. Operative reports and long-term follow-up data were evaluated. RESULTS: Of 92 IPs, 60.1% had FH, 25% had no CT bony changes, and 20.7% were revision cases. The recurrence rate for rhinologists was 10.5% overall and 7.3% for primary IPs. Primary and revision IPs had a similar rate of FH (63% vs. 52.6%; p = 0.646) and FH-TAP agreement (71.7% vs. 90%; p = 0.664). Nasal cavity IPs, especially with septal attachment, were more likely to lack bony changes on CT (57.1%) compared to other subsites (p = 0.018). Recurrent tumors were 16 mm larger on average (55 mm vs. 39 mm; p = 0.008). FH (75.0% vs. 60.9%; p = 0.295), FH-TAP concordance (91.7% vs. 74.4%; p = 0.094), and secondary IP (18.8% vs. 20.3%; p = 0.889) rates were similar between recurrent and nonrecurrent tumors. CONCLUSION: Primary and revision IPs have a similar rate of FH and FH-TAP agreement. Nasal cavity IPs are less likely to exhibit bony CT changes. Lower recurrence was associated with smaller size and fellowship training but not multiple TAPs, revision, FH absence, or FH-TAP discordance. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:1591-1596, 2024.
Assuntos
Neoplasias Nasais , Papiloma Invertido , Neoplasias dos Seios Paranasais , Humanos , Papiloma Invertido/diagnóstico por imagem , Papiloma Invertido/cirurgia , Papiloma Invertido/patologia , Neoplasias Nasais/diagnóstico por imagem , Neoplasias Nasais/cirurgia , Neoplasias Nasais/patologia , Estudos Retrospectivos , Imageamento por Ressonância Magnética , Recidiva Local de Neoplasia/diagnóstico por imagem , Recidiva Local de Neoplasia/cirurgia , Neoplasias dos Seios Paranasais/diagnóstico por imagem , Neoplasias dos Seios Paranasais/cirurgia , Neoplasias dos Seios Paranasais/patologia , Tomografia Computadorizada por Raios XRESUMO
Imaging is essential for evaluating patients with glioblastoma. Traditionally a multimodality undertaking, CT, including CT cerebral blood profusion, PET/CT with traditional fluorine-18 fluorodeoxyglucose (18F-FDG), and MRI have been the mainstays for diagnosis and post-therapeutic assessment. However, recent advances in these modalities, in league with the emerging fields of radiomics and theranostics, may prove helpful in improving diagnostic accuracy and treating the disease.
RESUMO
The pericardial cyst is a benign, uncommon congenital cystic lesion of pericardial origin located in the anterior and middle mediastinum. Most commonly, pericardial cysts are located in the right anterior cardiophrenic angle. Computed tomography (CT) and magnetic resonance imaging (MRI) of the chest are the non-invasive imaging modalities for the diagnosis of the pericardial cyst. Here we present a case of a 55-year-old male who presented with coughing and chest pain for two weeks. A chest X-ray revealed a soft-tissue opacity mass in the left lower zone. A CT of the chest showed a fluid-density cystic lesion in close proximity to the pericardium, located along the left posterior cardiophrenic angle, an uncommon location for a pericardial cyst.
RESUMO
In this study, we developed an automated workflow using a deep learning model (DL) to measure the lateral ventricle linearly in fetal brain MRI, which are subsequently classified into normal or ventriculomegaly, defined as a diameter wider than 10 mm at the level of the thalamus and choroid plexus. To accomplish this, we first trained a UNet-based deep learning model to segment the brain of a fetus into seven different tissue categories using a public dataset (FeTA 2022) consisting of fetal T2-weighted images. Then, an automatic workflow was developed to perform lateral ventricle measurement at the level of the thalamus and choroid plexus. The test dataset included 22 cases of normal and abnormal T2-weighted fetal brain MRIs. Measurements performed by our AI model were compared with manual measurements performed by a general radiologist and a neuroradiologist. The AI model correctly classified 95% of fetal brain MRI cases into normal or ventriculomegaly. It could measure the lateral ventricle diameter in 95% of cases with less than a 1.7 mm error. The average difference between measurements was 0.90 mm in AI vs. general radiologists and 0.82 mm in AI vs. neuroradiologists, which are comparable to the difference between the two radiologists, 0.51 mm. In addition, the AI model also enabled the researchers to create 3D-reconstructed images, which better represent real anatomy than 2D images. When a manual measurement is performed, it could also provide both the right and left ventricles in just one cut, instead of two. The measurement difference between the general radiologist and the algorithm (p = 0.9827), and between the neuroradiologist and the algorithm (p = 0.2378), was not statistically significant. In contrast, the difference between general radiologists vs. neuroradiologists was statistically significant (p = 0.0043). To the best of our knowledge, this is the first study that performs 2D linear measurement of ventriculomegaly with a 3D model based on an artificial intelligence approach. The paper presents a step-by-step approach for designing an AI model based on several radiological criteria. Overall, this study showed that AI can automatically calculate the lateral ventricle in fetal brain MRIs and accurately classify them as abnormal or normal.
RESUMO
Craniopharyngiomas emanate from squamous cell remnants in the hypophyseal/pharyngeal duct region. This report details the unprecedented case of a 29-year-old male with adamantinomatous craniopharyngioma, who, following a motor vehicle collision (MVC), presented with post-traumatic intratumoral hemorrhage leading to acute basal ganglia infarct. The patient, previously subjected to subtotal resection, exhibited focal neurological deficits attributed to compression of lenticulostriate arteries due to the sudden increase in tumor volume. The patient, ineligible for thrombolysis or thrombectomy, was conservatively managed post-MVC. Subtotal resection occurred four months later. After one year, persistent right-sided weakness (2/5 motor power) remained, and the recommended stereotactic radiotherapy was declined by the patient. Notably, this instance represents the first documented case of post-traumatic intratumoral hemorrhage in adamantinomatous craniopharyngioma. This report distinguishes between adamantinomatous and papillary subtypes, noting their prevalence in different age groups. While these tumors commonly present with gradual vision changes, fatigue, and endocrine dysfunction, complications such as intra-tumoral hemorrhage remain rare. This report serves as an educational tool, shedding light on potential complications and urging increased vigilance in managing craniopharyngiomas.
RESUMO
Recurrent painful ophthalmoplegic neuropathy (RPON), previously known as ophthalmoplegic migraine, is a rare disease that predominantly affects children. Recurrent episodes of ocular cranial nerve paresis with ipsilateral headache characterize this disorder. Diagnosis is mainly clinical with imaging being used as an adjunct. The pathophysiology of the disease is unknown. We present here a case of RPON in a 50-year-old female presenting with multiple episodes of headache and diplopia with associated transient thickening and enhancement of the ipsilateral oculomotor nerve on magnetic resonance imaging (MRI).
RESUMO
A 52-year-old female presented with epigastric pain, yellowing of the sclera, and vomiting for three weeks. Laboratory investigations revealed markedly elevated serum bilirubin and alkaline phosphatase levels, accompanied by a modest rise in transaminases. A clinical diagnosis of obstructive jaundice was established. Ultrasound of the abdomen depicted a mass in the region of the head of the pancreas. Contrast-enhanced computed tomography (CECT) of the abdomen revealed an infiltrative gastric mass spreading across the gastroduodenal junction and involving the ampulla of Vater. Owing to comorbid conditions and widespread lymphadenopathy, a palliative gastrojejunostomy with excision biopsy was performed. Histopathology confirmed an undifferentiated gastric adenocarcinoma.
RESUMO
Pancreatic fluid collections (PFC) are notorious for their extension beyond the normal confines of the pancreatic bed. This distribution is explained by dissection along the fascial planes in retroperitoneum due to the digestive enzymes within the PFC. In genitourinary track, PFCs have been described to involve the kidneys and the ureters. We report a case of severe acute necrotizing pancreatitis in a 28-year-old male, chronic alcoholic, who on readmission developed features of cystitis. The urine was turbid but did not show significant bacteriuria. Close location of the PFC near the urinary bladder (UB) prompted evaluation of urinary lipase and amylase. Elevated urinary enzyme levels suggested a Pancreatico-vesical fistula, conclusive demonstration of which was established by CT cystography. Percutaneous drainage of the necrosum and stenting of pancreatic duct led to spontaneous healing of the pancreatico-vesical fistula. Our case reiterates the remarkable property of pancreatic enzymes to dissect the fascial planes which is demonstrated by decompression of PFC via UB causing spontaneous Pancreatico-vesical fistula. Further, presence of main pancreatic duct fistulization should prompt endoscopic-guided stenting to obliterate the communication with the fistula and accelerate healing.