Performance of CT With Adrenal-Washout Protocol in Heterogeneous Adrenal Nodules: A Multiinstitutional Study.

BACKGROUND. CT with adrenal-washout protocol (hereafter, adrenal-protocol CT) is commonly performed to distinguish adrenal adenomas from other adrenal tumors. However, the technique's utility among heterogeneous nodules is not well established, and the optimal method for placing ROIs in heterogeneous nodules is not clearly defined. OBJECTIVE. The purpose of our study was to determine the diagnostic performance of adrenal-protocol CT to distinguish adenomas from nonadenomas among heterogeneous adrenal nodules and to compare this performance among different methods for ROI placement. METHODS. This retrospective study included 164 patients (mean age, 59.1 years; 61 men, 103 women) with a total of 164 heterogeneous adrenal nodules evaluated using adrenal-protocol CT at seven institutions. All nodules had an available pathologic reference standard. A single investigator at each institution evaluated the CT images. ROIs were placed on portal venous phase images using four ROI methods: standard ROI, which refers to a single large ROI in the nodule's center; high ROI, a single ROI on the nodule's highest-attenuation area; low ROI, a single ROI the on nodule's lowest-attenuation area; and average ROI, the mean of the three ROIs on the nodule's superior, middle, and inferior thirds using the approach for the standard ROI. ROIs were then placed in identical locations on unenhanced and delayed phase images. Absolute washout was determined for all methods. RESULTS. The nodules comprised 82 adenomas and 82 nonadenomas (36 pheochromocytomas, 20 metastases, 12 adrenocortical carcinomas, and 14 nodules with other pathologies). The mean nodule size was 4.5 ± 2.8 (SD) cm (range, 1.6-23.0 cm). Unenhanced CT attenuation of 10 HU or less exhibited sensitivity and specificity for adenoma of 22.0% and 96.3% for standard-ROI, 11.0% and 98.8% for high-ROI, 58.5% and 84.1% for low-ROI, and 30.5% and 97.6% for average-ROI methods. Adrenal-protocol CT overall (unenhanced attenuation ≤ 10 HU or absolute washout of ≥ 60%) exhibited sensitivity and specificity for adenoma of 57.3% and 84.1% for the standard-ROI method, 63.4% and 51.2% for the high-ROI method, 68.3% and 62.2% for the low-ROI method, and 59.8% and 85.4% for the average-ROI method. CONCLUSION. Adrenal-protocol CT has poor diagnostic performance for distinguishing adenomas from nonadenomas among heterogeneous adrenal nodules regardless of the method used for ROI placement. CLINICAL IMPACT. Adrenal-protocol CT has limited utility in the evaluation of heterogeneous adrenal nodules.

Terpene Synthase Gene Family in Chinese Chestnut (Castanea mollissima BL.) Harbors Two Sesquiterpene Synthase Genes Implicated in Defense against Gall Wasp Dryocosmus kuriphilus.

Chinese chestnut (Castanea mollissima BL.) is a well-known fruit tree that has been cultivated in East Asia for millennia. Leaves and buds of the plant can become seriously infested by the gall wasp Dryocosmus kuriphilus (GWDK), which results in gall formation and associated significant losses in fruit production. Herbivore-induced terpenes have been reported to play an important role in plant-herbivory interactions, and in this study, we show that upon herbivory by GWDK, four terpene-related compounds were significantly induced, while the concentrations of these four compounds in intact buds were relatively low. Among these compounds, (E)-nerolidol and (E, E)-α-farnesene have frequently been reported to be involved in plant herbivory defenses, which suggests direct and/or indirect functions in chestnut GWDK defenses. Candidate terpene synthase (TPS) genes that may account for (E)-nerolidol and (E, E)-α-farnesene terpene biosynthesis were characterized by transcriptomics and phylogenetic approaches, which revealed altered transcript levels for two TPSs: CmAFS, a TPS-g subfamily member, and CmNES/AFS, a TPS-b clade member. Both genes were dramatically upregulated in gene expression upon GWDK infestation. Furthermore, Agrobacterium tumefaciens-mediated transient overexpression in Nicotiana benthamiana showed that CmAFS catalyzed the formation of (E, E)-α-farnesene, while CmNES/AFS showed dual (E)-nerolidol and (E, E)-α-farnesene synthase activity. Biochemical assays of the recombinant CmAFS and CmNES/AFS proteins confirmed their catalytic activity in vitro, and the enzymatic products were consistent with two of the major volatile compounds released upon GWDK-infested chestnut buds. Subcellular localization demonstrated that CmAFS and CmNES/AFS were both localized in the cytoplasm, the primary compartment for sesquiterpene synthesis. In summary, we show that two novel sesquiterpene synthase genes CmAFS and CmNES/AFS are inducible by herbivory and can account for the elevated accumulation of (E, E)-α-farnesene and (E)-nerolidol upon GWDK infestation and may be implicated in chestnut defense against GWDK herbivores.

The terpene synthase (TPS) gene family in kiwifruit shows high functional redundancy and a subset of TPS likely fulfil overlapping functions in fruit flavour, floral bouquet and defence.

Volatile terpenes are important compounds that influence fruit flavour and aroma of kiwifruit. Terpenes in plants also impact on the floral bouquet and defence against pests and pathogens in leaves and fruit. To better understand the overlapping roles that terpenes may fulfil in plants, a systematic gene, chemical and biochemical analysis of terpenes and terpene synthases (TPS) was undertaken in Red5 kiwifruit (Actinidia spp.). Analysis of the Red5 genome shows it contains only 22 TPS gene models, of which fifteen encode full-length TPS. Thirteen TPS can account for the major terpene volatiles produced in different tissues of Red5 kiwifruit and in response to different stimuli. The small Red5 TPS family displays surprisingly high functional redundancy with five TPS producing linalool/nerolidol. Treatment of leaves and fruit with methyl jasmonate enhanced expression of a subset of defence-related TPS genes and stimulated the release of terpenes. Six TPS genes were induced upon herbivory of leaves by the economically important insect pest Ctenopseustis obliquana (brown-headed leaf roller) and emission, but not accumulation, of (E)- and (Z)-nerolidol was strongly linked to herbivory. Our results provide a framework to understand the overlapping biological and ecological roles of terpenes in Actinidia and other horticultural crops.

The hops (Humulus lupulus) genome contains a mid-sized terpene synthase family that shows wide functional and allelic diversity.

BACKGROUND: Hops (Humulus lupulus L.) are a dioecious climbing perennial, with the dried mature "cones" (strobili) of the pistillate/female inflorescences being widely used as both a bittering agent and to enhance the flavour of beer. The glandular trichomes of the bract and bracteole flowering structures of the cones produce an abundance of secondary metabolites, such as terpenoids, bitter acids and prenylated phenolics depending on plant genetics, developmental stage and environment. More knowledge is required on the functional and allelic diversity of terpene synthase (TPS) genes responsible for the biosynthesis of volatile terpenes to assist in flavour-directed hop breeding. RESULTS: Major volatile terpene compounds were identified using gas chromatography-mass spectrometry (GC-MS) in the ripe cones of twenty-one hop cultivars grown in New Zealand. All cultivars produced the monoterpene ß-myrcene and the sesquiterpenes α-humulene and ß-caryophyllene, but the quantities varied broadly. Other terpenes were found in large quantities in only a smaller subset of cultivars, e.g. ß-farnesene (in seven cultivars) and α-pinene (in four). In four contrasting cultivars (Wakatu™, Wai-iti™, Nelson Sauvin™, and 'Nugget'), terpene production during cone development was investigated in detail, with concentrations of some of the major terpenes increasing up to 1000-fold during development and reaching maximal levels from 50-60 days after flowering. Utilising the published H. lupulus genome, 87 putative full-length and partial terpene synthase genes were identified. Alleles corresponding to seven TPS genes were amplified from ripe cone cDNA from multiple cultivars and subsequently functionally characterised by transient expression in planta. Alleles of the previously characterised HlSTS1 produced humulene/caryophyllene as the major terpenes. HlRLS alleles produced (R)-(-)-linalool, whilst alleles of two sesquiterpene synthase genes, HlAFS1 and HlAFS2 produced α-farnesene. Alleles of HlMTS1, HlMTS2 and HlTPS1 were inactive in all the hop cultivars studied. CONCLUSIONS: Alleles of four TPS genes were identified and shown to produce key aroma volatiles in ripe hop cones. Multiple expressed but inactive TPS alleles were also identified, suggesting that extensive loss-of-function has occurred during domestication and breeding of hops. Our results can be used to develop hop cultivars with novel/improved terpene profiles using marker-assisted breeding strategies to select for, or against, specific TPS alleles.

Virtual Mentoring: A Guide to Navigating a New Age in Mentorship.

Mentorship is a fundamental part of professional and personal growth. Over time, the fabric of mentorship has been transforming from typical one-on-one mentor-mentee relationship to other types including peer, group, speed, and virtual mentoring. When the COVID-19 pandemic hit, it caused major disruptions in many facets of life and career, including mentoring. In response to the COVID-19 pandemic, live meetings were canceled, and social distancing measures were enacted at many institutions. Thus, the traditional set-up, with a face-to-face mentor and mentee interaction, was impossible. Many virtual platforms were utilized to navigate through these restrictions. In this review, we highlight challenges in mentorship during the COVID-19 pandemic and how we implemented different strategies to promote mentorship.

Vascular Anomaly Syndromes in the ISSVA Classification System: Imaging Findings and Role of Interventional Radiology in Management.

Vascular anomalies encompass a spectrum of tumors and malformations that can cause significant morbidity and mortality in children and adults. Use of the International Society for the Study of Vascular Anomalies (ISSVA) classification system is strongly recommended for consistency. Vascular anomalies can occur in isolation or in association with clinical syndromes that involve complex multifocal lesions affecting different organ systems. Thus, it is critical to be familiar with the differences and similarities among vascular anomalies to guide selection of the appropriate imaging studies and possible interventions. Syndromes associated with simple vascular malformations include hereditary hemorrhagic telangiectasia, blue rubber bleb nevus syndrome, Gorham-Stout disease, and primary lymphedema. Syndromes categorized as vascular malformations associated with other anomalies include Klippel-Trenaunay-Weber syndrome, Parkes Weber syndrome, Servelle-Martorell syndrome, Maffucci syndrome, macrocephaly-capillary malformation, CLOVES (congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and scoliosis, skeletal, and spinal anomalies) syndrome, Proteus syndrome, Bannayan-Riley-Ruvalcaba syndrome, and CLAPO (capillary malformations of the lower lip, lymphatic malformations of the face and neck, asymmetry of the face and limbs, and partial or generalized overgrowth) syndrome. With PHACES (posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects and/or coarctation of the aorta, eye abnormalities, and sternal clefting or supraumbilical raphe) syndrome, infantile hemangiomas associated with other lesions occur. Diagnostic and interventional radiologists have important roles in diagnosing these conditions and administering image-guided therapies-embolization and sclerotherapy, and different ablation procedures in particular. The key imaging features of vascular anomaly syndromes based on the 2018 ISSVA classification system and the role of interventional radiology in the management of these syndromes are reviewed. Online supplemental material is available for this article. ©RSNA, 2022.

Alcohol acyl transferase genes at a high-flavor intensity locus contribute to ester biosynthesis in kiwifruit.

Volatile esters are key compounds contributing to flavor intensity in commonly consumed fruits including apple (Malus domestica), strawberry (Fragaria spp.), and banana (Musa sapientum). In kiwifruit (Actinidia spp.), ethyl butanoate and other esters have been proposed to contribute fruity, sweet notes to commercial cultivars. Here, we investigated the genetic basis for ester production in Actinidia in an A. chinensis mapping population (AcMPO). A major quantitative trait loci for the production of multiple esters was identified at the high-flavor intensity (HiFI) locus on chromosome 20. This locus co-located with eight tandemly arrayed alcohol acyl transferase genes in the Red5 genome that were expressed in a ripening-specific fashion that corresponded with ester production. Biochemical characterization suggested two genes at the HiFI locus, alcohol acyl transferase 16-b/c (AT16-MPb/c), probably contributed most to the production of ethyl butanoate. A third gene, AT16-MPa, probably contributed more to hexyl butanoate and butyl hexanoate production, two esters that segregated in AcMPO. Sensory analysis of AcMPO indicated that fruit from segregating lines with high ester concentrations were more commonly described as being "fruity" as opposed to "beany". The downregulation of AT16-MPa-c by RNAi reduced ester production in ripe "Hort16A" fruit by >90%. Gas chromatography-olfactometry indicated the loss of the major "fruity" notes contributed by ethyl butanoate. A comparison of unimproved Actinidia germplasm with those of commercial cultivars indicated that the selection of fruit with high concentrations of alkyl esters (but not green note aldehydes) was probably an important selection trait in kiwifruit cultivation. Understanding ester production at the HiFI locus is a critical step toward maintaining and improving flavor intensity in kiwifruit.

Neurofibromatosis from Head to Toe: What the Radiologist Needs to Know.

Neurofibromatosis type 1 (NF1) and neurofibromatosis type 2 (NF2) are autosomal dominant inherited neurocutaneous disorders or phakomatoses secondary to mutations in the NF1 and NF2 tumor suppressor genes, respectively. Although they share a common name, NF1 and NF2 are distinct disorders with a wide range of multisystem manifestations that include benign and malignant tumors. Imaging plays an essential role in diagnosis, surveillance, and management of individuals with NF1 and NF2. Therefore, it is crucial for radiologists to be familiar with the imaging features of NF1 and NF2 to allow prompt diagnosis and appropriate management. Key manifestations of NF1 include café-au-lait macules, axillary or inguinal freckling, neurofibromas or plexiform neurofibromas, optic pathway gliomas, Lisch nodules, and osseous lesions such as sphenoid dysplasia, all of which are considered diagnostic features of NF1. Other manifestations include focal areas of signal intensity in the brain, low-grade gliomas, interstitial lung disease, various abdominopelvic neoplasms, scoliosis, and vascular dysplasia. The various NF1-associated abdominopelvic neoplasms can be categorized by their cellular origin: neurogenic neoplasms, interstitial cells of Cajal neoplasms, neuroendocrine neoplasms, and embryonal neoplasms. Malignant peripheral nerve sheath tumors and intracranial tumors are the leading contributors to mortality in NF1. Classic manifestations of NF2 include schwannomas, meningiomas, and ependymomas. However, NF2 may have shared cutaneous manifestations with NF1. Lifelong multidisciplinary management is critical for patients with either disease. The authors highlight the genetics and molecular pathogenesis, clinical and pathologic features, imaging manifestations, and multidisciplinary management and surveillance of NF1 and NF2. Online supplemental material is available for this article. ©RSNA, 2022.

Review of uterine fibroids: imaging of typical and atypical features, variants, and mimics with emphasis on workup and FIGO classification.

Uterine fibroids are the most common gynecologic neoplasm. Although non-degenerated fibroids are easily identifiable on imaging, degenerated fibroids, fibroid variants, and fibroids with unusual growth patterns can constitute a diagnostic dilemma. Identification of these abnormal morphologic features can alter the diagnosis of presumed uterine fibroids and hence change management plans. This article reviews the typical and atypical radiologic imaging features of uterine fibroids, with an emphasis on the pitfalls, mimics, and radiologically identifiable features that can alter clinical management plans.

Gastrointestinal bleeding: imaging and interventions in cancer patients.

Gastrointestinal bleeding (GIB) among cancer patients is a major source of morbidity and mortality. Although a wide variety of etiologies contribute to GIB, special considerations should be made for cancer-related factors such as the type of malignancy, location and extent of disease, hemostatic parameters, and treatment effects. Key imaging modalities used to evaluate GIB include computed tomography angiography (CTA), radionuclide imaging, and catheter-based angiography. Understanding the cancer and treatment history and recognizing the associated imaging manifestations are important for identifying the source and potential causes of GIB in cancer patients. This article will review the common clinical presentations, causes, imaging manifestations, and angiographic management of GIB in cancer patients.

Acing the Fundamentals of Radiology: An Online Series for Medical Students and Interns.

PURPOSE: The current undergraduate radiology education predominantly integrates radiology with other disciplines during preclerkship years and is often taught by nonradiologists. Early exposure to radiology and profound understanding of scientific fundamentals of imaging modalities and techniques are essential for a better understanding and interest in the specialty. Furthermore, the COVID-19 pandemic-related impact on in-person medical education aggravated the need for alternative virtual teaching initiatives to provide essential knowledge to medical students. METHODS: The authors designed an online 7-session course on the principles of imaging modalities for medical students and fresh graduates in the United States and abroad. The course was delivered online and taught by radiologists from different US institutions. Pretests and posttests were delivered before and after each session, respectively, to assess change in knowledge. At the end of the course, a survey was distributed among students to collect their assessment and feedback. RESULTS: A total of 162 students and interns initially enrolled in the program by completing a sign-up interest form. An average of 65 participants attended each live session, with the highest attendance being 93 live attendees. An average of 44 attendees completed both the pretest and posttest for each session. There was a statistically significant increase in posttest scores compared with pretest scores ( P < 0.01) for each session; on average, the posttest scores were 48% higher than the pretest scores. A total of 84 participants answered the end-of-course survey. A total of 11% of the respondents described themselves as first year, 17% as second year, 18% as third year, 21% as fourth year, and 33% as "other." Attendees were enrolled in medical schools across 21 different countries with 35% of the respondents studying medicine in the United States. More than 76% of the respondents stated that they "strongly agree" that the program increased their understanding of radiology, increased their interest in radiology, and would be useful in their clinical practice in the future. Eighty-three percent of the respondents stated that they "strongly agree" that "this course was a worthwhile experience." Particularly, more than 84% of the respondents stated that among the most important components in enhancing their understanding of radiology were "the interpretation of normal imaging" and "interpretation of clinical cases." Ninety-two percent of the respondents stated that "the amount of effort to complete the requirements for this program was just right." Participants were also asked to rate each of the 8 sessions using the following scale: poor = 1 point, fair = 2, good = 3, and excellent = 4. The average rating for all 8 sessions was 3.61 points (SD = 0.55), which translates to 96% of the sessions being rated good or excellent. Eighty percent of the participants reported that the topics presented in the program were "excellent and clinically important to learn," and 20% of the participants reported that the topics presented were "good and somewhat important to learn." The participants were asked to evaluate their confidence regarding basic radiology skills before and after the program using the following scale: not confident at all = 1 point, somewhat confident = 2, moderately confident = 3, and very confident = 4. Figure 2 summarizes the responses of the participants. CONCLUSIONS: An online course to teach the fundamentals of imaging modalities could be delivered through a webinar format to medical students and interns in several countries to address the potential gaps in radiology education, therefore increasing their understanding of the different imaging modalities and their proper use in medicine.

Analyzing the Landscape of the 2021 Virtual Match: A Nationwide Survey of Radiology Programs' Stakeholders.

RATIONALE AND OBJECTIVES: We aimed to provide insights into the adaptive strategies, benefits, and challenges faced by the radiology programs during the 2021 residency virtual Match. Furthermore, we explored the potential impacts of related topics, such as diversity and social media use on the Match process and outcomes. MATERIALS AND METHODS: A cross-sectional survey of 31 questions was designed and distributed via e-mails to individuals involved radiology programs match process during the 2021 Match. Descriptive statistics were used to analyze the results of most questions. Two questions comparing the changes in factors influencing the selection of applicants on a Likert scale of 1-5 were analyzed using paired t-test and Wilcoxon signed-rank test where p-value <0.05 was considered statistically significant. RESULTS: Responses from 125 participants were analyzed. The following factors carried less weight in evaluating applicants during 2021 Match: away rotations (p < 0.01), no failed attempts in USMLE Step 1/CK (p < 0.01), grades in radiology clerkship (p < 0.04), and class rank/quartile (p < 0.04), while personal statements were more important (p < 0.03). Out of the 125 respondents, 80 (64%) and 58 (47%) strongly or somewhat agree on the effectiveness of virtual interviews in gauging applicants' candidacy and showing their programs' advantages, respectively. Advantages of virtual interviews included decreased cost, time flexibility, less faculty burden, and an increased number of offered interviews according to 81% (101/125), 46% (58/125), 40%, (50/125), and 34% (43/125), respectively. The most helpful platforms that showcased program advantages were program websites followed by Twitter and Instagram. CONCLUSION: Most radiology programs were able to adjust to the virtual interview process, and the majority agree on their effectiveness citing many benefits. However, there were mixed opinions if it could be sustained in future cycles.

Current update on molecular cytogenetics, diagnosis and management of gastrointestinal stromal tumors.

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal (GI) tract and are thought to arise from precursors of the interstitial cells of Cajal. GISTs can arise anywhere in the GI tract, but most commonly originate from the stomach and small intestine. The majority of GISTs occur as a result of activating mutations in two receptor protein tyrosine kinases: KIT and/or platelet-derived growth factor receptor-α. Mutational analyses allow for predicting patient prognosis and treatment response. Clinical presentations can vary from no symptoms, typical in the case of small incidentally found tumors, to GI bleeding, abdominal discomfort, and ulcer-related symptoms when the tumor is enlarged. Imaging plays a critical role in the diagnosis and management of these tumors with multiphasic computed tomography serving as the imaging modality of choice. Magnetic resonance imaging and positron emission tomography-computed tomography can serve as imaging adjuncts in lesion characterization, especially with liver metastases, and subsequent staging and assessment for treatment response or recurrence. Surgical resection is the preferred management for small GISTs, while tyrosine kinase inhibitors - imatinib mesylate and sunitinib malate - serve as crucial molecular-targeted therapies for locally advanced and metastatic GISTs. This review article highlights the clinical presentation, pathology and molecular cytogenetics, imaging features, and current management of GISTs.

Current update on imaging for pancreatic neuroendocrine neoplasms.

Pancreatic neuroendocrine neoplasms (panNEN) are a heterogeneous group of tumors with differing pathological, genetic, and clinical features. Based on clinical findings, they may be categorized into functioning and nonfunctioning tumors. Adoption of the 2017 World Health Organization classification system, particularly its differentiation between grade 3, well-differentiated pancreatic neuroendocrine tumors (panNET) and grade 3, poorly-differentiated pancreatic neuroendocrine carcinomas (panNEC) has emphasized the role imaging plays in characterizing these lesions. Endoscopic ultrasound can help obtain biopsy specimen and assess tumor margins and local spread. Enhancement patterns on computed tomography (CT) and magnetic resonance imaging (MRI) may be used to classify panNEN. Contrast enhanced MRI and diffusion-weighted imaging have been reported to be useful for characterization of panNEN and quantifying metastatic burden. Current and emerging radiotracers have broadened the utility of functional imaging in evaluating panNEN. Fluorine-18 fluorodeoxyglucose positron emission tomography (PET)/CT and somatostatin receptor imaging such as Gallium-68 1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid-octreotate PET/CT may be useful for improved identification of panNEN in comparison to anatomic modalities. These new techniques can also play a direct role in optimizing the selection of treatment for individuals and predicting tumor response based on somatostatin receptor expression. In addition, emerging methods of radiomics such as texture analysis may be a potential tool for staging and outcome prediction in panNEN, however further investigation is required before clinical implementation.

Features of primary pancreatic lymphoma: A bi-institutional review with an emphasis on typical and atypical imaging features.

BACKGROUND: Primary pancreatic lymphoma (PPL) is a rare neoplasm. Being able to distinguish it from other pancreatic malignancies such as pancreatic ductal adenocarcinoma (PDAC) is important for appropriate management. Unlike PDAC, PPL is highly sensitive to chemotherapy and usually does not require surgery. Therefore, being able to identify PPL preoperatively will not only direct physicians towards the correct avenue of treatment, it will also avoid unnecessary surgical intervention. AIM: To evaluate the typical and atypical multi-phasic computed tomography (CT) imaging features of PPL. METHODS: A retrospective review was conducted of the clinical, radiological, and pathological records of all subjects with pathologically proven PPL who presented to our institutions between January 2000 and December 2020. Institutional review board approval was obtained for this investigation. The collected data were analyzed for subject demographics, clinical presentation, laboratory values, CT imaging features, and the treatment received. Presence of all CT imaging findings including size, site, morphology and imaging characteristics of PPL such as the presence or absence of nodal, vascular and ductal involvement in these subjects were recorded. Only those subjects who had a pre-treatment multiphasic CT of the abdomen were included in the study. RESULTS: Twenty-nine cases of PPL were diagnosed between January 2000 and December 2020 (mean age 66 years; 13 males/16 females). All twenty-nine subjects were symptomatic but only 4 of the 29 subjects (14%) had B symptoms. Obstructive jaundice occurred in 24% of subjects. Elevated lactate dehydrogenase was seen in 81% of cases, whereas elevated cancer antigen 19-9 levels were present in only 10% of cases for which levels were recorded. The vast majority (90%) of tumors involved the pancreatic head and uncinate process. Mean tumor size was 7.8 cm (range, 4.0-13.8 cm). PPL presented homogenous hypoenhancement on CT in 72% of cases. Small volume peripancreatic lymphadenopathy was seen in 28% of subjects. Tumors demonstrated encasement of superior mesenteric vessels in 69% of cases but vascular stenosis or occlusion only manifested in 5 out of the twenty-nine individuals (17%). Mild pancreatic duct dilatation was also infrequent and seen in only 17% of cases, whereas common bile duct (CBD) dilation was seen in 41% of subjects. Necrosis occurred in 10% of cases. Size did not impact the prevalence of pancreatic and CBD dilation, necrosis, or mesenteric root infiltration (P = 0.525, P = 0.294, P = 0.543, and P = 0.097, respectively). Pancreatic atrophy was not present in any of the subjects. CONCLUSION: PPL is an uncommon diagnosis best made preoperatively to avoid unnecessary surgery and ensure adequate treatment. In addition to the typical CT findings of PPL, such as homogeneous hypoenhancement, absence of vascular stenosis and occlusion despite encasement, and peripancreatic lymphadenopathy, this study highlighted many less typical findings, including small volume necrosis and pancreatic and bile duct dilation.

Tuberous Sclerosis: Current Update.

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.