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Mosaic KRAS variants and other RASopathy genes cause oculoectodermal, encephalo-cranio-cutaneous lipomatosis, and Schimmelpenning-Feuerstein-Mims syndromes, and a spectrum of vascular malformations, overgrowth and other associated anomalies, the latter of which are only recently being characterized. We describe eight individuals in total (six unreported cases and two previously reported cases) with somatic KRAS variants and variably associated features. Given the findings of somatic overgrowth (in seven individuals) and vascular or lymphatic malformations (in eight individuals), we suggest mosaic RASopathies (mosaic KRAS variants) be considered in the differential diagnosis for individuals presenting with asymmetric overgrowth and lymphatic or vascular anomalies. We expand the association with embryonal tumors, including the third report of embryonal rhabdomyosarcoma, as well as novel findings of Wilms tumor and nephroblastomatosis in two individuals. Rare or novel findings in our series include the presence of epilepsy, polycystic kidneys, and T-cell deficiency in one individual, and multifocal lytic bone lesions in two individuals. Finally, we describe the first use of targeted therapy with a MEK inhibitor for an individual with a mosaic KRAS variant. The purposes of this report are to expand the phenotypic spectrum of mosaic KRAS-related disorders, and to propose possible mechanisms of pathogenesis, and surveillance of its associated findings.

Cirrhosis is a heterogeneous disease that cannot be studied as a single entity and is classified in two main prognostic stages: compensated and decompensated cirrhosis. Portal hypertension, characterized by a pathological increase of the portal pressure and by the formation of portal-systemic collaterals that bypass the liver, is the initial and main consequence of cirrhosis and is responsible for the majority of its complications. A myriad of treatment options exists for appropriately managing the most common complications of portal hypertension, including acute variceal bleeding and refractory ascites. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Hemorrhage, resulting from gastric varies, can be challenging to treat, given the various precipitating etiologies. A wide variety of treatment options exist for managing the diverse range of the underlying disease processes. While cirrhosis is the most common cause for gastric variceal bleeding, occlusion of the portal or splenic vein in noncirrhotic states results in a markedly different treatment paradigm. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Acute obstructive uropathy is a medical emergency, which often is accompanied by acute renal failure or sepsis. Treatment options to resolve the acute obstructive process include conservative medical management, retrograde ureteral stenting, or placement of percutaneous nephrostomy or nephroureteral catheters. It is important to understand the various treatment options in differing clinical scenarios in order to guide appropriate consultation. Prompt attention to the underlying obstructive process is often imperative to avoid further deterioration of the patient's clinical status. A summary of the data and most up-to-date clinical trials regarding treatment options for urinary tract obstruction is outlined in this publication. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Hemoptysis, the expectoration of blood, ranges in severity from nonmassive to massive. This publication reviews the literature on the imaging and treatment of hemoptysis. Based on the literature, the imaging recommendations for massive hemoptysis are both a chest radiograph and CT with contrast or CTA with contrast. Bronchial artery embolization is also recommended in the majority of cases. In nonmassive hemoptysis, both a chest radiograph and CT with contrast or CTA with contrast is recommended. Bronchial artery embolization is becoming more commonly utilized, typically in the setting of failed medical therapy. Recurrent hemoptysis, defined as hemoptysis that recurs following initially successful cessation of hemoptysis, is best reassessed with a chest radiograph and either CT with contrast or CTA with contrast. Bronchial artery embolization is increasingly becoming the treatment of choice in recurrent hemoptysis, with the exception of infectious causes such as in cystic fibrosis. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Catheter-based hemodialysis is a necessary but unfortunate option for many patients with end-stage renal disease. Access to the central venous circulation is traditionally achieved via the jugular, subclavian, or femoral routes. However, chronic placement of indwelling hemodialysis catheters results in the slow exhaustion of the veins in the neck and chest due to venous thrombosis and occlusion. Once these conventional access sites are no longer available, it may become necessary to find additional, unconventional methods of access. Translumbar and transhepatic cannulation of the inferior vena cava reflect two unconventional modes of access into the central venous circulation. Because of the requisite technical expertise for placement and the comparatively high rate of complications, translumbar and transhepatic access for hemodialysis should only be considered in patients considered to have few or no other medical options for central venous access.

Biliary obstruction is a serious condition that can occur in the setting of both benign and malignant pathologies. In the setting of acute cholangitis, biliary decompression can be lifesaving; for patients with cancer who are receiving chemotherapy, untreated obstructive jaundice may lead to biochemical derangements that often preclude continuation of therapy unless biliary decompression is performed (see the ACR Appropriateness Criteria® topic on "Jaundice"). Recommended therapy including percutaneous decompression, endoscopic decompression, and/or surgical decompression is based on the etiology of the obstruction and patient factors including the individual's anatomy. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Uterine fibroids, also known as leiomyomas, are the most common benign tumor in women of reproductive age. When symptomatic, these patients can present with bleeding and/or bulk-related symptoms. Treatment options for symptomatic uterine leiomyomas include medical management, minimally invasive treatment such as uterine artery embolization, and surgical options, such as myomectomy. It is important to understand the role of these treatment options in various clinical scenarios so that appropriate consultation is performed. Furthermore, patients should be presented with the outcomes and complications of each of these treatment options. A summary of the data and clinical trials of the treatment options for symptomatic uterine leiomyomas is outlined in this article. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Background Medical management and surgical interventions for the lymphatic disorders chylothorax and plastic bronchitis are often unsuccessful. Single center data suggest that thoracic duct embolization is a more efficacious treatment for refractory lymphatic disorders, yet these outcomes have not been replicated. Case Presentation This case series describes a collaborative approach to the development of a successful lymphatic intervention program for congenital heart disease patients with persistent lymphatic disorders and defines a potential anatomic contraindication. Conclusions Successful lymphatic interventions can be performed at institutions with experienced providers working closely together to treat patients with complex congenital heart disease. Proper patient selection is essential and based on our results the presence of extensive lower body venous occlusion may be a contraindication and should be thoroughly investigated prior to attempts at thoracic duct embolization.

Management of primary and secondary hepatic malignancy is a complex problem. Achieving optimal care for this challenging population often requires the involvement of multiple medical and surgical disciplines. Because of the wide variety of potential therapies, treatment protocols for various malignancies continue to evolve. Consequently, development of appropriate therapeutic algorithms necessitates consideration of medical options, such as systemic chemotherapy; surgical options, such as resection or transplantation; and loco-regional therapies, such as thermal ablation and transarterial embolization techniques. This article provides a review of treatment strategies for the three most common subtypes of hepatic malignancy treated with loco-regional therapies: hepatocellular carcinoma, neuroendocrine metastases, and colorectal metastases. The ACR Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed every three years by a multidisciplinary expert panel. The guideline development and review include an extensive analysis of current medical literature from peer reviewed journals and the application of a well-established consensus methodology (modified Delphi) to rate the appropriateness of imaging and treatment procedures by the panel. In those instances where evidence is lacking or not definitive, expert opinion may be used to recommend imaging or treatment.

This chapter presents an overview of transhepatic catheter access, an acceptable but challenging vascular access technique that is often necessary in patients who have exhausted other endovascular options for central venous access. In patients with chronic total occlusion of internal jugular, subclavian, innominate, and femoral veins with coexistent occlusion of the infrahepatic inferior vena cava, transhepatic catheter placement is the last acceptable option for central venous access. Drawing on retrospective reviews of safety, efficacy, and complications implicit to the procedure, we present a simplified technique to successful transhepatic catheter placement.

Purpose The right internal jugular (RIJ) vein is preferred for placement of long-term venous catheters (CVC) for hemodialysis (HD). Use of left IJ vein potentially depletes the access site for arteriovenous fistula (AVF) or arteriovenous graft (AVF) placement because of the high rates of central venous stenosis. The right external jugular (EJ) vein is a viable alternative to the LIJ. The purpose of this study is to compare blood flow outcomes in a series of percutaneously placed external jugular (EJ), LIJ and RIJ HD catheters. Methods Using a prospectively collected database, we identified 46 hemodialysis patients who received a tunneled catheter during a 4-year period. Blood flow outcomes of RIJ, LIJ, and EJ catheters that were date-matched over the study period were compared. Using ANOVA, the blood flow outcomes of the 3 tunneled catheter techniques at 30-d and 90-d were compared. Results The 90-d blood flow outcomes of the 3 groups did not differ significantly. The 30-d blood flow was found to be 348.5± 56.62, 341± 22.42, and 365.7± 71.76 mL/min for RIJ, LIJ, and EJ respectively (PConclusions EJ blood flow outcomes at 30-d and 90-d were comparable to both LIJ and RIJ historic data. Further prospective investigation is required to define the role of EJ CVC placement as another potential long-term access modality.

Intraductal papillary mucinous neoplasms (IPMNs) of the pancreas are being diagnosed with increased frequency. CT scanning commonly serves as the primary imaging modality before surgery. We hypothesized MRCP provides better characterization of IPMN type/extent, which more closely matches actual pathology.Of 214 patients treated with IPMN (1991-2006), 30 had both preoperative CT and MRCP. Of these, 18 met imaging study criteria. Independent readers performed retrospective, blinded analyses using standardized criteria for IPMN type and extent.A ductal connection was detected on 73% of MRCP scans and only 18% of CT. IPMN type was classified differently in seven (39%); four (22%) of which were read on CT as having main duct involvement where this was not appreciated on MRCP or found on surgical pathology. MRCP showed multifocal disease in 13(72%) versus only 9(50%) on CT. A different disease distribution was seen in 9(50%). Finally, 101 branch lesions were identified on MRCP compared to 46 on CT.CT falls short of MRCP in detecting a ductal connection, estimating main duct involvement, and identification of small branch duct cysts. These factors influence diagnostic accuracy, cancer risk stratification and operative strategy. MRCP should be employed for optimal management of patients with IPMN.