Multimodality Imaging Review Research Articles

Malignant Rhabdoid Tumor and Related Pediatric Tumors: Multimodality Imaging Review with Pathologic Correlation.

Malignant rhabdoid tumors (MRTs) are rare but lethal solid neoplasms that overwhelmingly affect infants and young children. While the central nervous system is the most common site of occurrence, tumors can develop at other sites, including the kidneys and soft tissues throughout the body. The anatomic site of involvement dictates tumor nomenclature and nosology. While the clinical and imaging manifestations of MRTs and other more common entities may overlap, there are some site-specific distinctive imaging characteristics. Irrespective of the site of occurrence, somatic and germline mutations in SMARCB1, and rarely in SMARCA4, underlie the entire spectrum of rhabdoid tumors. MRTs have a simple and remarkably stable genome but can demonstrate considerable molecular and biologic heterogeneity. Related neoplasms encompass an expanding category of phenotypically dissimilar (nonrhabdoid tumors driven by SMARC-related alterations) entities. US, CT, MRI, and fluorodeoxyglucose PET/CT or PET/MRI facilitate diagnosis, initial staging, and follow-up, thus informing therapeutic decision making. Multifocal synchronous or metachronous rhabdoid tumors occur predominantly in the context of underlying rhabdoid tumor predisposition syndromes (RTPSs). These autosomal dominant disorders are driven in most cases by pathogenic variants in SMARCB1 (RTPS type 1) and rarely by pathogenic variants in SMARCA4 (RTPS type 2). Genetic testing and counseling are imperative in RTPS. Guidelines for imaging surveillance in cases of RTPS are based on age at diagnosis. ©RSNA, 2024 Supplemental material is available for this article.

Radiation-associated Angiosarcoma of the Breast: A Clinicopathological and Multimodality Imaging Review

Radiation-associated Angiosarcoma of the Breast: A Clinicopathological and Multimodality Imaging Review

Normal and Abnormal Appearances of the Ovaries during Assisted Reproduction: Multimodality Imaging Review.

Infertility is a common diagnosis that prompts many couples and individuals to seek assisted reproductive technology (ART) for assistance with conception. These technologies have become increasingly used in the United States in the past several decades, with 326 468 ART cycles performed in 2020, resulting in 75 023 live births. This ubiquity of ART also increases the likelihood that radiologists will encounter both normal and abnormal imaging findings associated with these treatments. Thus, radiologists of all subspecialties should be familiar with the multimodality appearance of the ovaries and pelvis in patients undergoing ART treatments. Furthermore, it is imperative that radiologists understand the appearance expected during different stages of the ART process. During stimulated ovulatory cycles, it is normal and expected for the ovaries to appear enlarged and to contain numerous cystic follicles, often with a small to moderate volume of pelvic free fluid. After oocyte retrieval, hemorrhagic ovarian follicles and a small to moderate volume of blood products in the cul-de-sac can be expected to be seen. Multiple nonemergency and emergency complications are related to ART, many of which can be seen at imaging. The most encountered emergency complications of ART include ovarian hyperstimulation syndrome, ectopic pregnancy, heterotopic pregnancy, multiple gestations, ovarian torsion, and procedural complications related to oocyte retrieval. These complications have important clinical implications, thus necessitating accurate and timely detection by the radiologist and the clinical team. ©RSNA, 2023 Supplemental material is available for this article. Quiz questions for this article are available through the Online Learning Center.

Spectrum of Mucin-containing Lesions of the Breast: Multimodality Imaging Review with Pathologic Correlation.

Mucin-containing lesions of the breast encompass a wide range of benign and malignant processes. The spectrum of histologic findings includes incidental mucus-filled benign cysts or ducts, mucocele-like lesions (MLLs), mucin-producing ductal carcinoma in situ, and invasive mucinous carcinoma. MLL is characterized by the presence of mucin-containing cysts that are typically associated with extravasated stromal mucin. MLL is often benign but can be associated with epithelial atypia or malignancy. Mucinous carcinoma represents the malignant end of the spectrum of mucinous lesions of the breast. Evidence-based literature supports a conservative approach for benign MLLs without associated atypia or malignancy, reserving excision for those lesions exhibiting such pathologic features. The most common imaging finding for MLL is microcalcifications at mammography. No specific imaging feature is predictive of malignant outcome at surgical excision. Invasive mucinous carcinoma is a heterogeneous breast tumor subtype, as defined according to the World Health Organization criteria. Mucinous carcinomas are categorized into pure (>90% mucinous component) or mixed (10%-90% mucinous component) subtypes. Pure mucinous carcinomas are generally associated with excellent prognosis and survival, with a few exceptions. Mixed mucinous carcinomas do not have the same favorable prognosis and instead behave similarly to invasive breast carcinomas of no special type. Characteristic diagnostic imaging features can be identified for mucinous carcinoma based on its mucinous and nonmucinous contents. ©RSNA, 2023 Online supplemental material is available for this article. Quiz questions for this article are available through the Online Learning Center.

Severe macular complications in glaucoma: high-resolution multimodal imaging characteristics and review of the literature

PurposeTo describe imaging characteristics of severe macular complications occurring in glaucoma and discuss available treatments.MethodsRetrospective case series of glaucomatous patients with macular retinoschisis (MR) and/or serous retinal detachment (SRD). Patients underwent a complete ophthalmological examination and multimodal imaging including retinography, SD-OCT, fluorescein and indocyanine green angiography (FA & ICGA) and adaptive optics (AO).ResultsTen eyes (8 patients) were included. Initial BCVA was 1.04 ± 1.12 logMAR and IOP was 24.0 ± 9.3mmHg. All eyes presented with MR while SRD was present in 5 eyes (5 patients), with a central macular thickness of 573 ± 152 μm. FA and ICGA allowed to exclude leakage in all cases. A focal lamina cribrosa defect (LCD) was found in four eyes (4 patients) using OCT, with AO providing en-face visualization of the defect in one eye. Outer retinal hole was present in 3 eyes (3 patients). No visual improvement or resolution of the macular retinoschisis was observed in eyes with medical or surgical IOP control (N = 9). Vitrectomy with internal membrane limiting peeling and gas tamponade was performed in one eye with good visual results.ConclusionsMultimodal high-resolution imaging is essential to diagnose severe macular complications associated with advanced glaucoma.

Multimodality imaging review of ulnar nerve pathologies.

The ulnar nerve is the second most commonly entrapped nerve after the median nerve. Although clinical evaluation and electrodiagnostic studies remain widely used for the evaluation of ulnar neuropathy, advancements in imaging have led to increased utilization of these newer / better imaging techniques in the overall management of ulnar neuropathy. Specifically, high-resolution ultrasonography of peripheral nerves as well as MRI has become quite useful in evaluating the ulnar nerve in order to better guide treatment. The caliber and fascicular pattern identified in the normal ulnar nerves are important distinguishing features from ulnar nerve pathology. The cubital tunnel within the elbow and Guyon's canal within the wrist are important sites to evaluate with respect to ulnar nerve compression. Both acute and chronic conditions resulting in deformity, trauma as well as inflammatory conditions may predispose certain patients to ulnar neuropathy. Granulomatous diseases as well as both neurogenic and non-neurogenic tumors can also potentially result in ulnar neuropathy. Tumors around the ulnar nerve can also lead to mass effect on the nerve, particularly in tight spaces like the aforementioned canals. Although high-resolution ultrasonography is a useful modality initially, particularly as it can be helpful for dynamic evaluation, MRI remains most reliable due to its higher resolution. Newer imaging techniques like sonoelastography and microneurography, as well as nerve-specific contrast agents, are currently being investigated for their usefulness and are not routinely being used currently.

Multimodality Imaging Review of HER2-positive Breast Cancer and Response to Neoadjuvant Chemotherapy

Human epidermal growth factor receptor 2 (HER2/neu or ErbB2)-positive breast cancers comprise 15%-20% of all breast cancers. The most common manifestation of HER2-positive breast cancer at mammography or US is an irregular mass with spiculated margins that often contains calcifications; at MRI, HER2-positive breast cancer may appear as a mass or as nonmass enhancement. HER2-positive breast cancers are often of intermediate to high nuclear grade at histopathologic analysis, with increased risk of local recurrence and metastases and poorer overall prognosis. However, treatment with targeted monoclonal antibody therapies such as trastuzumab and pertuzumab provides better local-regional control and leads to improved survival outcome. With neoadjuvant treatments, including monoclonal antibodies, taxanes, and anthracyclines, women are now potentially able to undergo breast conservation therapy and sentinel lymph node biopsy versus mastectomy and axillary lymph node dissection. Thus, the radiologist's role in assessing the extent of local-regional disease and response to neoadjuvant treatment at imaging is important to inform surgical planning and adjuvant treatment. However, assessment of treatment response remains difficult, with the potential for different imaging modalities to result in underestimation or overestimation of disease to varying degrees when compared with surgical pathologic analysis. In particular, the presence of calcifications at mammography is especially difficult to correlate with the results of pathologic analysis after chemotherapy. Breast MRI findings remain the best predictor of pathologic response. The authors review the initial manifestations of HER2-positive tumors, the varied responses to neoadjuvant chemotherapy, and the challenges in assessing residual cancer burden through a multimodality imaging review with pathologic correlation. © RSNA, 2023 Quiz questions for this article are available through the Online Learning Center.

Adenoid Cystic Carcinoma of the Breast: Multimodality Imaging Findings and Review of the Literature

Adenoid cystic carcinoma (ACC) of the breast is a rare type of breast cancer with favorable prognosis. There is limited data on the radiological findings of this rare tumor in literature. The aim of this study is to determine the most common imaging features and review the literature. Pathological databases of seven institutions from 2009 to 2021 were retrospectively reviewed, and patients with a diagnosis of ACC of the breast were determined. Thirteen patients whose imaging studies could be recalled from the picture archiving systems (PACS) were included in the study. Clinical and pathological findings as well as follow-up data were recorded. Radiological findings were analyzed and categorized based on BI-RADS 5th edition. There were 16 mass lesions in 13 patients (two multifocal cases, one case with recurrence). Mammography demonstrated 14 masses, while ultrasound (US) demonstrated all. MRI was available in only seven cases, with eight masses. The most common findings were round or oval shape on all modalities (78.57%-93.75%). Other frequent findings were parallel orientation (81.25%), isoechoic or hyperechoic echogenicity (62.5%), high T2 signal (87.5%), restricted diffusion (71.43%), and homogeneous enhancement (62.5%). Mammography, US and MRI showed circumscribed margins resembling a benign lesion in 35.71%, 37.5% and 50% of the lesions respectively. Three patients had a cyst-like echogenicty on US. Half of the lesions were avascular on Doppler US (6/12) and half were soft (2/4) on strain elastography. Although there were benign features on all imaging modalities seperately, all lesions could be categorized as BI-RADS 4 or 5 when the findings were combined. However 9/16 masses were BI-RADS 4A, emphasizing the subtlety of the malignant features. ACC of the breast can present with findings resembling a benign lesion on different imaging modalities. Although combination of all imaging findings correctly indicated the suspicious nature of the lesions in all cases, final classification was BI-RADS 4A in most of them. Radiologists should be aware of the more frequent findings of ACC of the breast for early diagnosis. US findings of isoechoic or hyperechoic appearance, and cyst-like echogenicity have not been reported previously in literature.

Extraskeletal Ewing Sarcoma from Head to Toe: Multimodality Imaging Review.

Extraskeletal Ewing sarcoma (EES) is a rare subtype in the Ewing sarcoma family of tumors (ESFT), which also includes Ewing sarcoma of bone (ESB) and, more recently, primitive neuroectodermal tumors. Although these tumors often have different manifestations, they are grouped on the basis of common genetic translocation and diagnosis from specific molecular and immunohistochemical features. While the large majority of ESFT cases occur in children and in bones, approximately 25% originate outside the skeleton as EES. Importantly, in the adult population these extraskeletal tumors are more common than ESB. Imaging findings of EES tumors are generally nonspecific, with some variation based on location and the tissues involved. A large tumor with central necrosis that does not cross the midline is typical. Despite often nonspecific findings, imaging plays an important role in the evaluation and management of ESFT, with MRI frequently the preferred imaging modality for primary tumor assessment and local staging. Chest CT and fluorine 18 fluorodeoxyglucose PET/CT are most sensitive for detecting lung and other distant or nodal metastases. Management often involves chemotherapy with local surgical excision, when possible. A multidisciplinary treatment approach should be used given the propensity for large tumor size and local invasion, which can make resection difficult. Despite limited data, outcomes are similar to those of other ESFT cases, with 5-year survival exceeding 80%. However, with metastatic disease, the long-term prognosis is poor. ©RSNA, 2022.

Nonvestibular Schwannomas of the Head and Neck

Schwannomas are benign peripheral nerve sheath tumors, of which almost half occur in the head and neck. Schwannomas may arise from any cranial, autonomic, or peripheral nerve. It is crucial to recognize the imaging features suggestive of schwannoma because the management decisions are unique due to the generally benign nature of this disease. While most nonvestibular schwannomas will be asymptomatic until they are large, tumors within or adjacent to bony structures are often associated with neurologic symptoms. This article provides a multimodality imaging review of the varied appearance of nonvestibular schwannomas in the head and neck as well as correlative discussion of the clinical presentation and management.Learning Objective: To describe the imaging appearance, critical clinical features, and management implications of nonvestibular schwannomas of the head and neck

A narrative review of multimodal imaging of white matter lesions in type-2 diabetes mellitus.

To discuss the relevant studies about the structural and functional changes of the brain in patients with type-2 DM (T2DM) and white matter lesion (WML) in recent years, and to summarize them. T2DM is a common metabolic disease with increasing prevalence worldwide. This disease is closely related to central nervous system and vascular disease, and is considered a risk factor for white matter lesions in the brain. Compared to healthy individuals, WML patients with T2DM exhibit changes in brain perfusion, functional networks, nerve fiber structure, and brain tissue metabolism. We analyzed recent studies related to structural and functional changes in the brain of patients suffering from T2DM and WML and summarized them. Multimodal magnetic resonance imaging (MRI) utilizes noninvasive and sensitive imaging techniques to provide multiparametric information in patients with T2DM to help in clinical practice. It features non-invasively and with high sensitivity assess the histomorphological and functional abnormalities of white matter in patients with T2DM using various parameters. We can use multimodal MRI methods to reflect the microscopic damage of neuromyelin structures and pathological changes of neuronal metabolic functions in WML in T2DM patients, and thus speculate the disease progression. This approach can be helpful for the early diagnosis and treatment of patients with such a disease who do not exhibit neurological deficit, to effectively improve their prognosis.

Role of Imaging in Diagnosis and Management of COVID-19: A Multiorgan Multimodality Imaging Review.

In this pandemic of Coronavirus disease 2019 (COVID-19), a vast proportion of healthcare resources, including imaging tools, have been dedicated to the management of affected patients; yet, the frequent reports of unknown presentations and complications of disease over time have been changing the usual standard of care and resource allocation in health centers. As of now, we have witnessed multisystemic symptoms requiring the collaboration of different clinical teams in COVID-19 patients' care. Compared to previous viral pandemics, imaging modalities are now playing an essential role in the diagnosis and management of patients. This widespread utility of imaging modalities calls for a deeper understanding of potential radiologic findings in this disease and identifying the most compatible imaging protocol with safety precautions. Although initially used for respiratory tract evaluation, imaging modalities have also been used for cardiovascular, neurologic, and gastrointestinal evaluation of patients with COVID-19. In this narrative review article, we provide multimodality and multisystemic review of imaging techniques and features that can aid in the diagnosis and management of COVID-19 patients.

Review of Multi-Modal Imaging in Urea Cycle Disorders: The Old, the New, the Borrowed, and the Blue.

The urea cycle disorders (UCD) are rare genetic disorder due to a deficiency of one of six enzymes or two transport proteins that act to remove waste nitrogen in form of ammonia from the body. In this review, we focus on neuroimaging studies in OTCD and Arginase deficiency, two of the UCD we have extensively studied. Ornithine transcarbamylase deficiency (OTCD) is the most common of these, and X-linked. Hyperammonemia (HA) in OTCD is due to deficient protein handling. Cognitive impairments and neurobehavioral disorders have emerged as the major sequelae in Arginase deficiency and OTCD, especially in relation to executive function and working memory, impacting pre-frontal cortex (PFC). Clinical management focuses on neuroprotection from HA, as well as neurotoxicity from other known and yet unclassified metabolites. Prevention and mitigation of neurological injury is a major challenge and research focus. Given the impact of HA on neurocognitive function of UCD, neuroimaging modalities, especially multi-modality imaging platforms, can bring a wealth of information to understand the neurocognitive function and biomarkers. Such information can further improve clinical decision making, and result in better therapeutic interventions. In vivo investigations of the affected brain using multimodal neuroimaging combined with clinical and behavioral phenotyping hold promise. MR Spectroscopy has already proven as a tool to study biochemical aberrations such as elevated glutamine surrounding HA as well as to diagnose partial UCD. Functional Near Infrared Spectroscopy (fNIRS), which assesses local changes in cerebral hemodynamic levels of cortical regions, is emerging as a non-invasive technique and will serve as a surrogate to fMRI with better portability. Here we review two decades of our research using non-invasive imaging and how it has contributed to an understanding of the cognitive effects of this group of genetic conditions.

Multimodality Imaging Review of Multiple Endocrine Neoplasia.

OBJECTIVE. The purpose of this article is to review the clinical manifestations, endocrine tumors types, and multimodality diagnostic tools available to physicians involved in the management of patients with multiple endocrine neoplasia (MEN) syndrome, in addition to discussing relevant imaging findings and appropriate imaging follow-up. CONCLUSION. Thorough knowledge of the spectrum of tumors associated with MEN gene mutations aids in the screening, diagnostic workup, and posttreatment monitoring of patients with MEN-related gene mutations.

Choroidal macrovessels: multimodal imaging findings and review of the literature

Background/aimsTo describe clinical and multimodal imaging features in a cohort of choroidal macrovessels.MethodsDemographics and multimodal imaging features of 16 eyes of 13 patients with choroidal macrovessels were reviewed. The multimodal...

Skeletal staging in Langerhans cell histiocytosis: a multimodality imaging review.

Evaluating the extent of skeletal disease in Langerhans cell histiocytosis (LCH) is a major predictor of patient outcome. Traditionally, whole-body skeletal staging consists of plain radiography and bone scintigraphy. However, more recently whole-body MRI has been shown to be accurate in detecting osseous and extra-osseous lesions, but no large-scale studies analysing its role within the diagnostic algorithm of LCH skeletal staging currently exist. In addition, FDG PET-CT provides useful information regarding disease activity and treatment response, but has an inherent radiation dose which is not ideal in children. Currently, radiographic skeletal survey remains the gold standard with cross-sectional imaging only performed for further characterisation. However, radiographs have shown a wide sensitivity range for skeletal staging and have clear limitations in detecting extra-skeletal disease, a crucial component of stratification in identifying 'at risk' organs. We aim to highlight the various appearances of bony LCH across all the imaging modalities for primary skeletal staging. We will also review the advantages, disadvantages, sensitivity and specificity of each, and establish their role in staging skeletal LCH. Recent studies using whole-body MRI have shown promising results, with radiographs and other modalities playing a more complementary role.

Multimodal imaging of chorioretinal folds induced by orbital vascular malformation in two cases.

To investigate the alterations in the retinal pigment epithelium (RPE) in the crest and trough portions of chorioretinal folds (CRFs) induced by an orbital vascular tumor. Review of multimodal imaging in two eyes of two patients with globe compression and CRFs from an orbital vascular tumor. Fundus photography demonstrated obliquely extending CRFs with alternating hyperpigmented and hypopigmented linear alterations in both eyes. Fundus autofluorescence (AF) imaging showed obliquely oriented hypoAF lines, incompletely alternating with hyperAF lines. In Case 1, the hyperAF lines had interspersed hypoAF segments and Case 2 had peripapillary mottling of AF. Fluorescein angiography (FA) showed alternating hyper and hypofluorescent lines in the late phase in Case 1. Optical coherence tomography (OCT) documented relative thinning of RPE at the folded crests in Case 1 and preservation of RPE in Case 2. Swept-source OCT angiography (SS-OCTA) demonstrated oblique hyporeflective lines in the outer retina and choriocapillaris layers in Case 2. These findings suggest that the crest of a CRF represents thinned or rarified RPE with hypoAF, transmission hyperfluorescence (FA), partially attenuated RPE layer (OCT), and isoreflectivity (SS-OCTA) while the trough represents compressed RPE with irregular hyperAF, transmission hypofluorescence (FA), thickened RPE layer (OCT), and hyporeflectivity (SS-OCTA). The anatomic and functional status of the RPE in CRFs based on multimodal imaging reveals normal to attenuated RPE with hypofunctionality at the fold crest and compacted, thickened RPE at the trough with segmental functional impairment on AF imaging. Anatomic information regarding CRFs is evident on OCT, FA, and SS-OCTA while the functional status is depicted on AF.

Multimodality imaging review of complex pelvic lesions in female pelvis.

Complex pelvic lesions can originate from various anatomical structures in the pelvis and pose a diagnostic dilemma due to a wide range of possible diagnoses. Accurate characterisation of these lesions would often require an algorithmic approach, which incorporates clinical findings, sequential use of multiple imaging modalities and a multiparametric approach. This approach usually aims at identifying key imaging features, which aid in anatomical localisation, morphology and tissue characterisation. There have been various attempts to standardise the lexicon used for describing adnexal masses in female patients; stratify their risk of cancer and suggest appropriate next steps in the management pathway. Through this review, we extend this approach to complex pelvic masses in female pelvis in general and will focus on optimal use of different imaging modalities to arrive at definitive diagnosis or meaningful differential diagnosis. We will also discuss potential pitfalls of imaging diagnosis and common mimics.

The Spectrum of Rib Neoplasms in Adults: A Practical Approach and Multimodal Imaging Review.

OBJECTIVE. Adult neoplasms of the ribs are a heterogeneous group consisting of both benign and aggressive entities. Rib neoplasms have a variety of overlapping imaging features, with much of the imaging data disjointed across the musculoskeletal, thoracic, and oncologic imaging literature. Arrival at accurate diagnosis can therefore be quite challenging. This article consolidates this information and introduces the reader to an algorithmic approach to rib lesion evaluation based on imaging. CONCLUSION. Rib neoplasms are a diverse group of benign and malignant entities, which often makes determining an accurate diagnosis challenging. Evaluation requires a multipronged approach that incorporates radiographic imaging features, nonradiographic imaging findings, lesion location, and clinical data.

Eight-year multimodal follow-up of recurrent idiopathic acute exudative polymorphous vitelliform maculopathy

To describe and analyze clinical findings in a patient with recurrent idiopathic acute exudative polymorphous vitelliform maculopathy (AEPVM), followed in detail, and to propose the diagnostic and follow-up algorithm. Retrospective observational analysis. A young adult male patient diagnosed with idiopathic AEPVM who developed two relapses in a 12-month period eight years after the initial onset. Review of clinical charts, multimodal imaging, and electrophysiology findings. The patient repeatedly underwent complete ophthalmic examinations, including best-corrected visual acuity testing (BCVA), slit-lamp and fundus examinations; digital fundus photography, time-domain optical coherence tomography (OCT) in 2009 (Stratus OCT, Carl Zeiss Meditec, USA) and spectral-domain OCT in 2017-2018 (Spectralis-OCT, Heidelberg Engineering, Germany), together with fundus autofluorescence (FAF), fluorescein angiography (FA), and indocyanine green angiography (ICGA), all with HRA2 (Heidelberg Engineering, Germany); microperimetry (MP-1 microperimeter, Nidek, Japan). Laser flare photometry (Kowa FM-600, Japan) and electrophysiology testing were also performed. Clinical features of long-lasting recurrent idiopathic AEPVM, and diagnostic and follow-up algorithm in such rare cases. Case report of a 25-year-old male Caucasian patient with typical features of AEPVM, including serous neuroepithelial detachment with irregular retinal elevations, ophthalmoscopically resembling retinal folds, with subsequent subretinal accumulation of characteristic yellow-white vitelliform deposits. Features in this case rarely described, or even not yet reported, include indocyanine- and fluorescein-negative intraretinal cystic changes, optic disc hyperfluorescence on FA, serous retinal elevations mimicking retinal folds, increased choroidal thickness, lack of rapid visual recovery, and very slow anatomical improvement of the relapses. Bimonthly fundus autofluorescence evaluation together with SD-OCT were the most informative diagnostic methods, demonstrating the evolution of pathological signs. AEPVM may be a recurrent or even chronic condition with uncertain long-term visual outcomes. It may have variable clinical presentations depending on the stage of the disease, and both clinical manifestations and imaging features of different stages of the pathologic process may overlap. Patients should be made aware that visual improvement occurs very slowly, if at all. Bimonthly fundus autofluorescence evaluation together with SD-OCT should be recommended in such cases.