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Background: Placenta accreta spectrum (PAS) disorders are characterized by an abnormal adherence of the placenta to the uterine myometrium. Magnetic resonance imaging (MRI) is an important adjunct in antenatal diagnosis. Objective: We sought to determine if there are patient and MRI characteristics that limit the accuracy of PAS diagnosis and degree of invasion. Study design: We conducted a retrospective cohort analysis of patients who were evaluated for PAS by MRI from 01/2007-12/2020. Patient characteristics evaluated included number of prior cesarean deliveries, history of dilation and curettage (D&C) or dilation and evacuation (D&E), short interval pregnancy

There is a wide spectrum of hereditary and acquired immunodeficiency disorders that are characterized by specific abnormalities involving a plethora of humoral, cellular, and phagocytic immunologic pathways. These include distinctive primary immunodeficiency syndromes due to characteristic genetic defects and secondary immunodeficiency syndromes, such as AIDS from HIV infection and therapy-related immunosuppression in patients with cancers or a solid organ or stem cell transplant. The gut mucosa and gut-associated lymphoid tissue (the largest lymphoid organ in the body), along with diverse commensal microbiota, play complex and critical roles in development and modulation of the immune system. Thus, myriad gastrointestinal (GI) symptoms are common in immunocompromised patients and may be due to inflammatory conditions (graft versus host disease, neutropenic enterocolitis, or HIV-related proctocolitis), opportunistic infections (viral, bacterial, fungal, or protozoal), or malignancies (Kaposi sarcoma, lymphoma, posttransplant lymphoproliferative disorder, or anal cancer). GI tract involvement in immunodeficient patients contributes to significant morbidity and mortality. Along with endoscopy and histopathologic evaluation, imaging plays an integral role in detection, localization, characterization, and distinction of GI tract manifestations of various immunodeficiency syndromes and their complications. Select disorders demonstrate characteristic findings at fluoroscopy, CT, US, and MRI that permit timely and accurate diagnosis. While neutropenic enterocolitis affects the terminal ileum and right colon and occurs in patients receiving chemotherapy for hematologic malignancies, Kaposi sarcoma commonly manifests as bull's-eye lesions in the stomach and duodenum. Imaging is invaluable in treatment follow-up and long-term surveillance as well.

There is a wide spectrum of benign and malignant mesenchymal neoplasms of the prostate, which account for less than 1% of all prostatic tumors. These include distinctive tumors that arise from the specialized prostatic stroma and site-agnostic neoplasms such as smooth muscle tumors, fibrous or myofibroblastic neoplasms, neurogenic tumors, vascular tumors, and a plethora of sarcomas. Select tumors show classic sites of origin within the prostate. While stromal tumors of uncertain malignant potential (STUMPs) commonly involve the peripheral zone at the prostate base, leiomyomas typically originate from the central prostate toward the apex. Some "prostatic" neoplasms such as gastrointestinal stromal tumors, solitary fibrous tumor (SFT), paragangliomas, and neurogenic tumors arise primarily from periprostatic soft tissues. Most mesenchymal tumors of the prostate and seminal vesicles manifest as large tumors that cause nonspecific symptoms; prostate-specific antigen level is not typically elevated. Diverse mesenchymal neoplasms demonstrate characteristic histopathologic and immunocytochemical features and variable cross-sectional imaging findings. While leiomyoma and SFT typically display low signal intensity on T2-weighted images, synovial sarcomas commonly show hemorrhage. Diagnosis is difficult because of the rarity and lack of awareness of the tumors and the significant overlap in histopathologic features. Select tumors show characteristic genetic abnormalities that allow the diagnosis to be established. For example, more than 90% of SFTs are characterized by a unique

Tuberous sclerosis complex (TSC) is a relatively rare autosomal dominant neurocutaneous disorder secondary to mutations in the TSC1 or TSC2 tumor suppressor genes. Although manifestation of the classic triad of seizures, intellectual disability, and facial angiofibromas may facilitate timely diagnosis of TSC, the multisystem features that may indicate TSC in the absence of these manifestations remain highly variable. In addition, patients with TSC are at risk of developing multiple benign and malignant tumors in various organ systems, resulting in increased morbidity and mortality. Thus, imaging plays a critical role in diagnosis, surveillance, and management of patients with TSC. It is crucial that radiologists be familiar with TSC and the various associated imaging features to avoid a delayed or incorrect diagnosis. Key manifestations include cortical dysplasias, subependymal nodules, subependymal giant cell astrocytomas, cardiac rhabdomyomas, lymphangioleiomyomatosis, and angiomyolipomas. Renal angiomyolipomas in particular can manifest with imaging features that mimic renal malignancy and pose a diagnostic dilemma. Other manifestations include dermatologic and ophthalmic manifestations, renal cysts, renal cell carcinomas, multifocal micronodular pneumocyte hyperplasia, splenic hamartomas, and other rare tumors such as perivascular epithelioid tumors. In addition to using imaging and clinical features to confirm the diagnosis, genetic testing can be performed. In this article, the molecular pathogenesis, clinical manifestations, and imaging features of TSC are reviewed. Current recommendations for management and surveillance of TSC are discussed as well. ©RSNA, 2021.

Hepatocellular adenomas (HCAs), hepatocellular carcinomas (HCCs), and intrahepatic cholangiocarcinomas (iCCAs) are a highly heterogeneous group of liver tumors with diverse pathomolecular features and prognoses. High-throughput gene sequencing techniques have allowed discovery of distinct genetic and molecular underpinnings of these tumors and identified distinct subtypes that demonstrate varied clinicobiologic behaviors, imaging findings, and complications. The combination of histopathologic findings and molecular profiling form the basis for the morphomolecular classification of liver tumors. Distinct HCA subtypes with characteristic imaging findings and complications include

Infections are the most commonly encountered complications in patients with cancer. The classical signs and symptoms of infections are often not present in this patient population, which makes the diagnosis more challenging. Host factors play a major role in the development and prognosis of infections in cancer patients; these can be related to the underlying type of malignancy (solid organ versus hematological), tumor burden, anatomic obstruction, altered integrity of barriers (skin or mucosa), treatment-related factors (from chemotherapy, radiation treatment, surgery, interventional procedures, and/or medical device placement) and the degree of immunosuppression. This article reviews common, as well as less common, imaging manifestations of infections and their potential mimics in the abdomen and pelvis in cancer patients and discusses their differentiating features, with the role of imaging in various organs in the abdomen and pelvis taking into consideration relevant clinical background information and the main risk factors.

Technologic advances in chromosomal analysis and DNA sequencing have enabled genome-wide analysis of cancer cells, yielding considerable data on the genetic basis of malignancies. Evolving knowledge of tumor genetics and oncologic pathways has led to a better understanding of histopathologic features, tumor classification, tumor biologic characteristics, and imaging findings and discovery of targeted therapeutic agents. Radiogenomics is a rapidly evolving field of imaging research aimed at correlating imaging features with gene mutations and gene expression patterns, and it may provide surrogate imaging biomarkers that may supplant genetic tests and be used to predict treatment response and prognosis and guide personalized treatment options. Multidetector CT, multiparametric MRI, and PET with use of multiple radiotracers are some of the imaging techniques commonly used to assess radiogenomic associations. Select abdominal malignancies demonstrate characteristic imaging features that correspond to gene mutations. Recent advances have enabled us to understand the genetics of steatotic and nonsteatotic hepatocellular adenomas, a plethora of morphologic-molecular subtypes of hepatic malignancies, a variety of clear cell and non-clear cell renal cell carcinomas, a myriad of hereditary and sporadic exocrine and neuroendocrine tumors of the pancreas, and the development of targeted therapeutic agents for gastrointestinal stromal tumors based on characteristic

OBJECTIVE. Imaging plays an important role in the diagnosis and staging of malignancies. Many common lymphoproliferative and other solid tumor malignancies can be viral-related. CONCLUSION. This re...

Cystic hepatobiliary neoplasms with mucin-producing epithelium-mucinous cystic neoplasm of the liver (MCN) and intraductal papillary neoplasm of the bile duct (IPNB)-are rare and distinct entities that have unique clinical, pathologic, and imaging features. They are differentiated pathologically by the presence of subepithelial ovarian-like hypercellular stroma (OLS), which is the defining histopathologic feature of MCN. MCN is commonly a benign, large, solitary, symptomatic, multiloculated cystic mass without biliary communication that occurs in middle-aged women. On the other hand, IPNBs are a heterogeneous spectrum of tumors, which are commonly associated with invasive carcinoma, occur in older patients, and can be differentiated from MCN by communication with the biliary tree, intraductal masses, associated biliary ductal dilatation, and absent OLS. Understanding of these rare neoplasms has grown and evolved over time and continues to today, but uncertainty and controversy persist, related to the rarity of these tumors, relatively recent designation as separate entities, inherent clinicopathologic heterogeneity, overlapping imaging features, and the fact that many prior studies likely included MCN and cystic IPNB together as a single entity. Confusion regarding these neoplasms is evident by historical inconsistencies and nonstandardized nomenclature through the years. Awareness of these entities is important for the interpreting radiologist to suggest a particular diagnosis or generate a meaningful differential diagnosis in the appropriate setting, and is of particular significance as MCN and cystic IPNB have overlapping imaging features with other more common hepatobiliary cystic masses but have different management and prognosis. Online supplemental material is available for this article. Work of the U.S. Government published under an exclusive license with the RSNA.

Testicular germ cell tumors (TGCTs) demonstrate a wide variety of histopathologic, genetic, pathogenetic, and immunocytochemical characteristics and various clinical-biologic profiles and prognoses. Most TGCTs arise from an intratubular precursor cell referred to as germ cell neoplasia in situ (GCNIS), which is an embryonic germ cell with the potential to differentiate into a plethora of embryonic and extraembryonic lineages. Advances in pathologic examination and genetics paved the way for the 2016 World Health Organization (WHO) classification system, which recognizes two pathogenetically distinct groups of TGCTs. Although postpubertal tumors originate from GCNIS, almost all prepubertal tumors belong to the non-GCNIS category. Molecular testing for chromosome 12p amplification helps to distinguish the two tumor categories. Imaging techniques such as US, CT, MRI, and fluorine 18 (

A wide spectrum of hereditary syndromes predispose patients to distinct pancreatic abnormalities, including cystic lesions, recurrent pancreatitis, ductal adenocarcinoma, nonductal neoplasms, and parenchymal iron deposition. While pancreatic exocrine insufficiency and recurrent pancreatitis are common manifestations in cystic fibrosis and hereditary pancreatitis, pancreatic cysts are seen in von Hippel-Lindau disease, cystic fibrosis, autosomal dominant polycystic kidney disease, and McCune-Albright syndrome. Ductal adenocarcinoma can be seen in many syndromes, including Peutz-Jeghers syndrome, familial atypical multiple mole melanoma syndrome, Lynch syndrome, hereditary breast and ovarian cancer syndrome, Li-Fraumeni syndrome, and familial pancreatic cancer syndrome. Neuroendocrine tumors are commonly seen in multiple endocrine neoplasia type 1 syndrome and von Hippel-Lindau disease. Pancreatoblastoma is an essential component of Beckwith-Wiedemann syndrome. Primary hemochromatosis is characterized by pancreatic iron deposition. Pancreatic pathologic conditions associated with genetic syndromes exhibit characteristic imaging findings. Imaging plays a pivotal role in early detection of these conditions and can positively affect the clinical outcomes of those at risk for pancreatic malignancies. Awareness of the characteristic imaging features, imaging-based screening protocols, and surveillance guidelines is crucial for radiologists to guide appropriate patient management. ©RSNA, 2021.

Urachal anomalies are classified into four types depending on the level of persistence of the embryonic urachal remnants between the urinary bladder and the umbilicus: patent urachus, umbilical-urachal sinus, urachal cyst, and vesico-urachal diverticulum. Due to the increasing use of cross-sectional imaging, urachal anomalies are frequently detected as incidental findings. Imaging plays a pivotal role in the initial diagnosis, evaluation of complications, treatment follow-up, and long-term surveillance of patients with urachal anomalies. Different urachal anomalies demonstrate characteristic imaging features that aid in a timely diagnosis and guide treatment. A patent urachus is visualized as an elongated tubular structure between the umbilicus and the urinary bladder. While umbilical-urachal sinus appears as focal dilatation at the umbilical end of the urachal remnant, the vesico-urachal diverticulum presents as a focal outpouching of the urinary bladder at anterosuperior aspect. Urachal cysts are identified as midline fluid-filled sacs most frequently located near the dome of the urinary bladder. Untreated urachal anomalies could progress into potential complications, including infection and malignancy. Knowledge regarding imaging features of urachal anomalies helps in timely diagnosis, treatment, follow-up, and early detection of complications.

Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare, heterogeneous neuroendocrine neoplasms of the autonomous nervous system of chromaffin cell origin that may arise within the adrenal medulla (PCCs) or the sympathetic and parasympathetic paraganglia (PGLs). Currently referred to by the umbrella term pheochromocytomas-paragangliomas (PPGLs), these distinct tumors are characterized by specific histopathology as well as biological and clinical profiles. PPGLs may occur as part of hereditary syndromes (40% of cases) or as sporadic tumors. Currently, there are 12 different hereditary syndromes with characteristic genetic abnormalities, at least 15 well-characterized driver genes and distinct tumor metabolic pathways. Based on the Cancer Genome Atlas (TCGA) taxonomic schemata, PPGLs have been classified into three main clusters of specific genetic mutations and tumor pathways with clinical, biochemical, and prognostic implications. Imaging plays a pivotal role in the initial diagnosis, tumor characterization, evaluation of treatment response, and long-term surveillance. While MDCT and MRI help in the anatomic localization, SPECT, and PET using different radiotracers are crucial in the functional assessment of these tumors. Surgery, chemotherapy, and radiotherapy are currently available treatment options for PPGLs; antiangiogenic drugs are also being used in treating metastatic disease. Evolving knowledge regarding the different genetic abnormalities involved in the pathogenesis of PPGLs has identified potential therapeutic targets that may be utilized in the discovery of novel drugs.

A wide spectrum of second cancers occur as late complications of radiation therapy (RT) used to treat various malignancies. In addition to the type and dose of radiation, lifestyle, environmental, and genetic factors are important to the development of second malignancies in cancer survivors. Typically, RT-induced malignancies (RTIMs) are biologically aggressive cancers with a variable period of 5-10 years for hematologic malignancies and 10-60 years for solid tumors between RT and the development of the second cancer. Although carcinomas and leukemias commonly develop after low-dose RT, sarcomas occur in tissues or organs that receive high-dose RT. Angiosarcomas and unclassified pleomorphic sarcomas are the two most common RT-associated sarcomas; other sarcomas include malignant peripheral nerve sheath tumors, leiomyosarcomas, osteosarcomas, chondrosarcomas, and dedifferentiated or pleomorphic liposarcomas. Select RTIMs show tumor genetic characteristics that allow accurate diagnosis. Nearly all cutaneous angiosarcomas after RT for breast cancer and 90% of RT-associated malignant peripheral nerve sheath tumors are characterized by

Cystic tumors of the liver are a heterogeneous group of neoplasms with characteristic histogenesis, clinico-biological features, and pathologic findings. They include both epithelial and mesenchymal liver tumors and commonly originate from the biliary epithelium. Mucinous cystic neoplasm (MCN) and intraductal papillary neoplasm of the bile duct (IPNB) are the most common cystic neoplasms of the liver. Cystic changes can develop in hepatocellular carcinoma (HCC) and hepatic metastases due to cystic degeneration and necrosis. Rarely, mass-forming intrahepatic cholangiocarcinoma and giant cavernous hemangioma may also show cystic appearance with necrotic areas. Rare hepatic tumors such as mesenchymal hamartoma, undifferentiated embryonal carcinoma, lymphangioma, and inflammatory myofibroblastic tumors are also cystic in nature. Select cystic hepatic tumors show characteristic cross-sectional imaging findings that permit an accurate diagnosis. Imaging is also pivotal in the follow-up and long-term surveillance of cystic hepatic tumors.

Pancreatic neuroendocrine neoplasms (panNENs) are heterogeneous neoplasms with neuroendocrine differentiation that show characteristic clinical, histomorphologic, and prognostic features; genetic alterations; and biologic behavior. Up to 10% of panNENs develop in patients with syndromes that predispose them to cancer, such as multiple endocrine neoplasia type 1, von Hippel-Lindau disease, tuberous sclerosis complex, neurofibromatosis type 1, and glucagon cell adenomatosis. PanNENs are classified as either functioning tumors, which manifest early because of clinical symptoms related to increased hormone production, or nonfunctioning tumors, which often manifest late because of mass effect. PanNENs are histopathologically classified as well-differentiated pancreatic neuroendocrine tumors (panNETs) or poorly differentiated pancreatic neuroendocrine carcinomas (panNECs) according to the 2010 World Health Organization (WHO) classification system. Recent advances in cytogenetics and molecular biology have shown substantial heterogeneity in panNECs, and a new tumor subtype, well-differentiated, high-grade panNET, has been introduced. High-grade panNETs and panNECs are two distinct entities with different pathogenesis, clinical features, imaging findings, treatment options, and prognoses. The 2017 WHO classification system and the eighth edition of the American Joint Committee on Cancer staging system include substantial changes. Multidetector CT, MRI, and endoscopic US help in anatomic localization of the primary tumor, local-regional spread, and metastases. Somatostatin receptor scintigraphy and fluorine 18-fluorodeoxyglucose PET/CT are helpful for functional and metabolic assessment. Knowledge of recent updates in the pathogenesis, classification, and staging of panNENs and familiarity with their imaging findings allow optimal patient treatment. ©RSNA, 2020.

There is a wide spectrum of pancreatic neoplasms with characteristic genetic abnormalities, tumor pathways, and histopathology that primarily determine tumor biology, treatment response, and prognosis. Although most pancreatic tumors are sporadic, 10% of neoplasms occur in the setting of distinct hereditary syndromes. Detailed studies of these rare syndromes have allowed researchers to identify a myriad of specific genetic signatures of pancreatic tumors. A better understanding of tumor genomics may have significant clinical implications in the diagnosis and management of patients with pancreatic tumors. Evolving knowledge has paved the way to screening paradigms and protocols in individuals at higher risk of developing pancreatic tumors.

There is a wide spectrum of mendelian disorders that predispose patients to an increased risk of benign as well as malignant tumors. Hereditary cancer syndromes are characterized by the early onset of diverse, frequently advanced malignancies in specific organ systems in multiple family members, posing significant challenges to diagnosis and management. A better understanding of the genetic abnormalities and pathophysiology that underlie these disorders has led to contemporary paradigms to screen, allowing early diagnosis, and has improved targeted therapies to aid in management. This article reviews select hereditary cancer syndromes with an emphasis on imaging-based screening and surveillance strategies.

Hereditary gastrointestinal (GI) cancer syndromes due to specific germline mutations are characterized by an increased risk of GI tract malignancies, extra-GI tract cancers, and benign abnormalities. These syndromes include Lynch syndrome, familial adenomatous polyposis, juvenile polyposis syndrome, Peutz-Jeghers syndrome, Cowden syndrome, hereditary diffuse gastric cancer, and hereditary pancreatic cancer. Timely identification of the responsible genes will help predict future cancer risks in these patients and their family members. Early detection of cancers is possible with appropriate screening methods; risk-reducing measures will help in cancer prevention. Select malignancies and benign conditions associated with these syndromes have distinctive imaging features that can aid in classifying the syndromes. Imaging also plays a pivotal role in screening and surveillance of patients as well as their at-risk relatives and is invaluable for follow-up of treated malignancies. The American College of Gastroenterology has established specific guidelines for diagnosis and management of hereditary GI cancer syndromes. Knowledge of the imaging features of various pathologic conditions and screening strategies will guide appropriate management of patients and at-risk family members. ©RSNA, 2019.

Germ cell tumors, because they contain immature and mature elements, can differentiate into different tissue types. They can exhibit unusual imaging features or manifest in a syndromic fashion. The authors describe these features and assign them to one of the following categories: (a) unusual manifestations of metastatic disease (growing teratoma syndrome, choriocarcinoma syndrome, ossified metastases, and gliomatosis peritonei); (b) autoimmune manifestations (sarcoidlike reaction and paraneoplastic syndromes); (c) endocrine syndromes (sex hormone production, struma ovarii, and struma carcinoid); or (d) miscellaneous conditions (ruptured dermoid cyst, squamous cell carcinoma arising from a mature teratoma, Currarino triad, fetus in fetu, pseudo-Meigs syndrome, and pancreatitis). Rare conditions associated with germ cell tumors demonstrate characteristic imaging findings that can help lead to the appropriate diagnosis and management recommendations. When evaluating for potential metastatic disease, alternative benign diagnoses should be considered (eg, growing teratoma syndrome, ossified metastases, ruptured dermoid cyst, gliomatosis peritonei, and sarcoidlike reaction), which may impact management. Germ cell tumors may also lead to life-threatening complications such as extensive hemorrhage from choriocarcinoma metastases or the rupture of mature teratomas, cases in which timely diagnosis is crucial. Autoimmune and endocrine manifestations such as paraneoplastic encephalitis, autoimmune hemolytic anemia, and hyperthyroidism may occur owing to the presence of germ cell tumors and can create a diagnostic dilemma for clinicians. Knowledge of the syndromic and unusual imaging findings associated with germ cell tumors helps guide appropriate management. ©RSNA, 2019.

Recent advances in pathology and genetics have improved our understanding of the pathogenesis of inherited and sporadic malignancies. Detailed studies of hereditary cancer syndromes-which contribute to 5%-10% of the overall cancer burden-have shed new light on the important role of genetic abnormalities in tumor metabolism, oncologic pathways, and clinicobiologic behavior. Many inherited cancer syndromes are characterized by development of pathognomonic histotypes of neoplasms in specific target organs. Cross-sectional imaging plays an integral role in diagnosis, screening, surveillance, and treatment of patients with a wide spectrum of cancer syndromes. This article focuses on the imaging spectrum of select hereditary cancer syndromes, featuring imaging features of associated common and uncommon tumors and conditions in each syndrome, along with screening and surveillance recommendations for each condition. MRI has proved to be a useful screening modality in such patients, as these patients are often young and require prolonged screening; MRI has the added advantage of better soft-tissue contrast without ionizing radiation. The whole-body MRI protocol is also briefly discussed. The radiologist is sometimes the first physician to encounter such patients, and knowledge of these syndromes can help identify these patients earlier and impact their care by timely diagnosis and intervention. This also benefits the family members, as they can also undergo genetic testing and obtain an early diagnosis and screening. ©RSNA, 2019.

The human gastrointestinal tract houses trillions of microbes. The gut and various types of microorganisms, including bacteria, viruses, fungi, and archaea, form a complex ecosystem known as the gut microbiota, and the whole genome of the gut microbiota is referred to as the gut microbiome. The gut microbiota is essential for homeostasis and the overall well-being of a person and is increasingly considered an adjunct "virtual organ," with a complexity level comparable to that of the other organ systems. The gut microbiota plays an essential role in nutrition, local mucosal homeostasis, inflammation, and the mucosal immune system. An imbalanced state of the gut microbiota, known as dysbiosis, can predispose to development of various gastrointestinal malignancies through three speculated pathogenic mechanisms

Life expectancies for solid organ recipients as well as graft survival rates for these patients have improved over the years because of advanced immunosuppressive therapies; however, with chronic use of these drugs, posttransplant malignancy has become one of the leading causes of morbidity for them. The risk of carcinogenesis in transplant recipients is significantly higher than for the general population and cancers tend to manifest at an advanced stage. Posttransplant malignancies are thought to develop by three mechanisms: de novo development, donor-related transmission, and recurrence of a recipient's pretransplant malignancy. Although nonmelanoma skin cancer, Kaposi sarcoma, posttransplant lymphoproliferative disorder, anogenital cancer, and lung cancer are malignancies that are thought to arise de novo, malignant melanoma and cancers that arise in the renal allograft are frequently donor related. Hepatocellular carcinomas and cholangiocarcinomas have a greater tendency to recur in liver transplant recipients. An altered or deranged immune system caused by chronic immunosuppression is considered to be one of the major contributing factors to carcinogenesis. The proposed pathogenic mechanisms for oncogenesis include impaired immunosurveillance of neoplastic cells, weakened immune activity against oncogenic viruses, and direct carcinogenic effects of immunosuppressive agents. Imaging plays an important role in screening, follow-up, and long-term surveillance in patients with malignancies because key imaging features can guide in their timely diagnosis. However, some benign entities such as transplant-related renal fibrosis, biliary necrosis, and infectious nodules in the lungs mimic malignancies and require pathologic confirmation. Management strategies that can improve malignancy-related morbidity and mortality in transplant recipients include prevention of risk factors, appropriate modulation of immunosuppressive agents, prophylaxis against infection-related malignancies, and use of intensive targeted screening programs. (©)RSNA, 2016.

Availability of novel immunosuppressive drugs is the most important factor responsible for significant improvement in long-term graft survival rates after solid organ transplantation. However, chronic immunosuppression predisposes patients to a myriad of potentially life-threatening complications. In addition to drug-related adverse effects, transplant recipients are at increased risk of developing opportunistic microbial infections and a spectrum of unique cancers, many of which are caused by oncogenic viruses. Cross-sectional imaging studies play a crucial role in the timely diagnosis and management of post-transplant infections and malignancies.

Hepatocellular carcinoma and cholangiocarcinoma are the most common primary liver malignancies in adults (comprising >85%); however, liver is also host to some unusual primary malignant tumors. Some of these tumors show distinct demographic, clinicopathologic, and imaging features. Imaging features of these uncommon primary malignant liver tumors are presented with an attempt to correlate them with histopathology.

Introduction After identifying and correcting pitfalls related to image acquisition, radiologists should focus their attention on the potential errors that can occur during image interpretation. Among them, thefirst andmost important one is inappropriate characterization of a focus of normal renal tissue, congenital variant, or a benign infectious or inflammatory condition as a renal neoplasmon imaging studies. Secondly, identification of fat within a solid renalmass can create problems in distinguishing benign angiomyolipoma (AML) from malignant entities such as renal cell carcinoma (RCC) and liposarcoma. Thirdly, inappropriate assessment of contrast enhancement in solid or cystic masses, especially in hyperattenuating and heavily calcified renal masses, may pose significant problems that result in a faulty interpretation of a benign neoplasm as a malignant one and vice versa. In this article, we would review potential errors that radiologistsmay encounter during interpretation of ultrasound (US), multiple detector computed tomography (MDCT), and magnetic resonance imaging (MRI) studies in patients with renal masses and provide possible solutions to overcome these pitfalls.

Acute cholecystitis (AC) is a life-threatening emergency that commonly occurs as a complication of gallstones. Severe right upper quadrant pain, abdominal guarding, fever, and a positive Murphy's sign with an elevated white blood cell count are the classical clinical manifestations of AC. Although ultrasonography is typically the initial diagnostic examination in patients with suspected AC, computed tomography and magnetic resonance imaging are commonly performed to identify complications; cholescintigraphy is recommended in patients with equivocal findings on the other imaging modalities, as this technique has the highest diagnostic accuracy in the diagnosis of AC. Imaging studies are also helpful in the timely detection of complications associated with AC. Although laparoscopic cholecystectomy is considered the gold-standard treatment for AC, percutaneous gallbladder drainage with or without cholecystostomy tube placement is a safe, effective management technique for surgically high-risk patients with multiple medical conditions. This treatment can be used as either a bridging therapy, with elective cholecystectomy performed at a later time after improvement of the patient's condition, or as definitive treatment in surgically unfit patients. Radiologists play a pivotal role in the initial diagnosis and management of patients with AC.

Pancreatic ductal adenocarcinoma (PDAC) is a genetically heterogeneous, biologically aggressive malignancy with a uniformly poor prognosis. While most pancreatic cancers arise sporadically, a small subset of PDACs develop in patients with hereditary and familial predisposition. Detailed studies of the rare hereditary syndromes have led to identification of specific genetic abnormalities that contribute to malignancy. For example, germline mutations involving BRCA1, BRCA2, PRSS1, and mismatch repair genes predispose patients to PDAC. While patients with Lynch syndrome develop a rare "medullary" variant of adenocarcinoma, intraductal papillary mucinous tumors are observed in patients with McCune-Albright syndrome. It is now well established that PDACs originate via a multistep progression from microscopic and macroscopic precursors due to cumulative genetic abnormalities. Improved knowledge of tumor genetics and oncologic pathways has contributed to a better understanding of tumor biology with attendant implications on diagnosis, management, and prognosis. In this article, the genetic landscape of PDAC and its precursors will be described, the hereditary syndromes that predispose to PDAC will be reviewed, and the current role of imaging in screening and staging assessment, as well as the potential role of molecular tumor-targeted imaging for evaluation of patients with PDAC and its precursors, will be discussed. Keywords: Abdomen/GI, Genetic Defects, Oncology, Pancreas Supplemental material is available for this article. © RSNA, 2020.

Cystic neoplasms in the pancreas are encountered frequently on imaging, often detected incidentally during evaluation for other conditions. They can have a variety of clinical and imaging presentations, and similarly, wide-ranging prognostic and treatment implications. In the majority, imaging helps in diagnosis of pancreatic cystic neoplasms (PCNs) and guides management decisions. But, a significant minority of the PCNs remain indeterminate. There have been multiple recent advances in biomarkers and molecular genetics which will likely prove helpful in risk stratification of PCNs. Several prominent national and international societies, as well as consensus groups have put forth recommendations to help guide management of PCNs. The purpose of this article is to discuss the role of imaging in evaluation of PCNs, review the recent advances in molecular genetics and pancreatic cyst fluid analysis, and analyze the pros and cons of major evidence-based and consensus guidelines for management of PCNs.

Purpose To determine the clinical significance and potential mechanisms of segmental liver ischemia and infarction following elective creation of a transjugular intrahepatic portosystemic shunt (TIPS). Materials and Methods A retrospective review of 374 elective TIPS creations between March 2006 and September 2014 was performed, yielding 77 contrast-enhanced scans for review. Patients with imaging evidence of segmental perfusion defects were identified. Model for End-stage Liver Disease scores, liver volume, and percentage of liver ischemia/infarct were calculated. Clinical outcomes after TIPS creation were reviewed. Results Ten patients showed segmental liver ischemia/infarction on contrast-enhanced imaging after elective TIPS creation. Associated imaging findings included thrombosis of the posterior division (n = 7) and anterior division (n = 3) of the right portal vein (PV). The right hepatic vein was thrombosed in 5 patients, as was the middle hepatic vein in 3 and the left hepatic vein in 1. One patient had acute thrombosis of the shunt and main PV. Three patients developed acute liver failure: 2 died within 30 days and 1 required emergent liver transplantation. One patient died of acute renal failure 20 days after TIPS creation. A large infarct in a transplant recipient resulted in biloma formation. Five patients survived without additional interventions with follow-up times ranging from 3 months to 5 years. Conclusions Segmental perfusion defects are not an uncommon imaging finding after elective TIPS creation. Segmental ischemia was associated with thrombosis of major branches of the PVs and often of the hepatic veins. Clinical outcomes varied significantly, from transient problems to acute liver failure with high mortality rates.

Approximately 20% of choledochal cysts (CC) present in adult patients and they are commonly associated with a high risk of complications, including malignancy. Additionally, children who underwent internal drainage procedures for CCs can develop complications during adulthood despite treatment. Concepts regarding classification and pathogenesis of the CCs have been evolving. While new subtypes are being added to the widely accepted Todani classification system, simplified classification schemes have also been proposed to guide appropriate management. The exact etiology of CCs is currently unknown. The two leading theories involve either the presence of an anomalous pancreatico-biliary junction with associated reflux of pancreatic juice into the biliary system or, more recently, some form of antenatal biliary obstruction with resulting proximal bile duct dilation. Imaging studies play an important role in the initial diagnosis, surgical planning, and long-term surveillance of CCs.

Harmful mutations of the BRCA tumor suppressor genes result in a greater lifetime risk for malignancy-breast and ovarian cancers in particular. An increased risk for male breast, fallopian tube, primary peritoneal, pancreatic, prostate, and colon cancers also has been reported. The BRCA gene is inherited in an autosomal dominant pattern and tends to be highly penetrant; thus, there is an increased incidence of these cancers in affected families. Compared with sporadic tumors, BRCA-associated malignancies have unique manifestations, clinical features, and pathologic profiles. Manifestation at an early patient age, high-grade tumors, and an aggressive clinical course are common features of BRCA-associated malignancies. Understanding the behavior of these cancers aids in identification of affected individuals and families, who can then make informed decisions regarding their future health. Enhanced screening, prophylactic surgery, and chemoprevention are options for managing cancer risk factors in these individuals. Imaging has an important role in the screening, evaluation, staging, and follow-up of BRCA-associated malignancies. Supplemental screening of BRCA mutation carriers often begins at an early age and is critical for early and accurate cancer diagnoses. The authors review the etiopathogenesis and imaging features of BRCA-associated malignancies, the importance of a multidisciplinary approach to determining the diagnosis, and the treatment of patients who have these mutations to improve their outcomes.

Graft-versus-host disease (GVHD) is a common complication of hematopoietic stem cell transplant (HSCT). GVHD predominantly affects the skin, gastrointestinal system and hepatobiliary systems. Imaging findings in the gastrointestinal tract include bowel wall thickening with mucosal enhancement, mesenteric edema, and vascular engorgement. In the hepatobiliary system, hepatosplenomegaly, periportal edema, bile duct dilatation, and gallbladder and biliary wall thickening are seen. Although the imaging findings of GVHD are nonspecific, with a known history of HSCT, GVHD should be considered.GVHD is a serious complication of HSCT, which involves multiple organ systems, with imaging manifestations most commonly seen in the gastrointestinal tract and hepatobiliary system. Knowledge of the imaging manifestations of GVHD, which alone may be relatively nonspecific, taken in conjunction with clinical history including the timing and type of HSCT, laboratory values, stool studies, and dermatologic findings can increase radiologist confidence in suggesting this diagnosis.

Bile duct strictures in adults are secondary to a wide spectrum of benign and malignant pathologic conditions. Benign causes of bile duct strictures include iatrogenic causes, acute or chronic pancreatitis, choledocholithiasis, primary sclerosing cholangitis, IgG4-related sclerosing cholangitis, liver transplantation, recurrent pyogenic cholangitis, Mirizzi syndrome, acquired immunodeficiency syndrome cholangiopathy, and sphincter of Oddi dysfunction. Malignant causes include cholangiocarcinoma, pancreatic adenocarcinoma, and periampullary carcinomas. Rare causes include biliary inflammatory pseudotumor, gallbladder carcinoma, hepatocellular carcinoma, metastases to bile ducts, and extrinsic bile duct compression secondary to periportal or peripancreatic lymphadenopathy. Contrast material-enhanced magnetic resonance (MR) imaging with MR cholangiopancreatography is extremely helpful in the noninvasive evaluation of patients with obstructive jaundice, an obstructive pattern of liver function, or incidentally detected biliary duct dilatation. Some of these conditions may show characteristic findings at MR imaging-MR cholangiopancreatography that help in making a definitive diagnosis. Although endoscopic retrograde cholangiopancreatography with tissue biopsy or surgery is needed for the definitive diagnosis of many of these strictures, certain MR imaging characteristics of the narrowed segment (eg, thickened wall, long-segment involvement, asymmetry, indistinct outer margin, luminal irregularity, hyperenhancement relative to the liver parenchyma) may favor a malignant cause. Awareness of the various causes of bile duct strictures in adults and familiarity with their appearances at MR imaging-MR cholangiopancreatography are important for accurate diagnosis and optimal patient management.

Neuroendocrine (NE) neoplasms of the genitourinary (GU) tract in adults are rare tumors with distinct histopathology and variable biological behavior and imaging findings. They may be primary or metastatic in origin. The spectrum of primary GU tract NE neoplasms includes carcinoid, small cell carcinoma, large cell NE carcinoma, and paraganglioma. The tumors commonly show positivity to specific immunohistochemical markers and characteristic dense-core granules at the ultra-structural level. Although imaging findings are nonspecific and accurate differentiation from the more common malignancies of the individual organs is not possible, cross-sectional imaging modalities play an important role in the diagnosis, staging, and surveillance of these tumors. Somatostatin receptor scintigraphy (octreotide scan) may be useful in the detection and treatment of metastatic disease in select patients. Knowledge of the various NE tumors of the adult GU tract and familiarity with their pathological and imaging findings permit optimal patient management.

Adrenal collision tumors (ACTs) refer to coexistence of two adjacent, but histologically distinct neoplasms involving the adrenal gland without histologic admixture at interface. ACTs include adenoma with myelolipoma, adenoma with metastases, hemangioma with adenoma, and adrenocortical carcinoma with myelolipoma. In addition, hemorrhage into a pre-existing adrenal mass can mimic an ACT, and it is important to differentiate these two pathologies. Accurate characterization of ACTs is difficult, but critical, for correct staging of patients with malignancies and to guide percutaneous biopsy. Magnetic resonance imaging (MRI) and multidetector computed tomography imaging techniques may depict different tumor components separately; however, biopsy may be required in selected patients for confirmation. [18F]Fluorodeoxyglucose-positron emission tomography (PET) shows increased uptake in the malignant component of ACTs, and guides percutaneous biopsy. Even in patients requiring percutaneous biopsy for a definite diagnosis, imaging findings can help in guiding the appropriate component to be biopsied. Knowledge of imaging findings of different ACTs and their mimics on MRI, computed tomography, and PET help in optimal patient management.

Cancers of the female genital system, particularly endometrial and ovarian cancers, can be associated with hereditary cancer syndromes such as hereditary breast and ovarian cancer and Lynch syndrome. Cancers that are found in the setting of a hereditary cancer syndrome are often unique in presentation, clinical features, and pathologic profiles when compared with sporadic tumors. This article reviews the hereditary cancer syndromes associated with gynecological malignancies, as well as the imaging findings and staging system of endometrial and ovarian cancers. These associations are important for proper patient screening, diagnosis, and treatment.

Tumors and tumorlike conditions of the anus and perianal region originate from the anal canal and anal margin or result from direct extension of tumors from adjacent organs. The anatomy of the anal canal is complex, and its different histologic characteristics can lead to diverse pathologic conditions. The anal canal extends from the anorectal junction to the anal verge. The World Health Organization classification of anal canal tumors includes (a) anal intraepithelial neoplasia, the precursor of squamous cell carcinoma (SCC), and (b) invasive tumors. Invasive tumors are further classified on the basis of cell type as epithelial tumors (SCC, adenocarcinoma, mucinous adenocarcinoma, small cell carcinoma, and undifferentiated carcinoma), nonepithelial tumors, carcinoid tumors, melanoma, and secondary tumors (direct spread from rectal, cervical, or prostate carcinoma). The anal margin, or perianal skin, lies outside the anal verge and encompasses a radius of 5 cm from the anal verge. Tumors in the anal margin are classified according to the World Health Organization classification of skin tumors. Anal margin tumors include SCC, anal intraepithelial neoplasia, also known as Bowen disease, adenocarcinoma and its precursor Paget disease, basal cell carcinoma, and verrucous carcinoma (Buschke-Lowenstein tumor), which is a rare variant of SCC. Imaging plays an important role in the evaluation, staging, and follow-up of patients with anal and perianal tumors. However, because of the overlap in imaging features among these diverse entities, a definitive diagnosis is best established at histopathologic examination. Nevertheless, familiarity with the pathogenesis, imaging features, and treatment of these tumors can aid radiologic diagnosis and guide appropriate patient treatment. (©)RSNA, 2016.

Immunoglobulin G4-related disease (IgG4-RD) is a novel, immune-mediated, multisystem disease characterized by the development of tumefactive lesions in multiple organs. IgG4-RD encompasses many fibroinflammatory diseases that had been thought to be confined to single organs. Delayed diagnosis or misdiagnosis as malignancies leading to aggressive treatment may be averted by identification of the multisystem nature of IgG4-RD. Most cases show exquisite response to steroid therapy; steroid-resistant cases are being treated by novel therapeutic agents, including B-cell depleting agents such as rituximab. Cross-sectional imaging studies play a pivotal role in the initial diagnosis, assessing response to therapy and long-term surveillance.

Introduction There has been an exponential increase in the incidental detection of renal masses that is commensurate with the increasing use of cross-sectional studies for evaluation of awide spectrum of patient symptoms. This increased detection of renal lesions necessitates better characterization to allowproper management. Ultrasound or ultrasonography (US), multidetector-row computed tomography (MDCT), and magnetic resonance imaging (MRI) are the modalities currently available for renal lesion detection, localization, and characterization. Standardized, multiphase CT and MRI protocols have been developed to allow reliable and reproducible lesion characterization. However, suboptimal image quality or nonstandard imaging techniquesmay create challenges in both the detection of masses as well as their accurate diagnosis. In this article, we present potential image acquisition–related pitfalls and possible solutions in the detection and assessment of renal masses in adult patients. Improved knowledge regarding such potential pitfalls may help radiologists to choose a different imaging modality or repeat the same study with technique modifications.