Biliary Disorders, Anomalies, and Malignancies in Children.

Biliary abnormalities in children are uncommon, and the spectrum of biliary disorders is broader than in adult patients. Unlike in adults, biliary disorders in children are rarely neoplastic and are more commonly rhabdomyosarcoma rather than cholangiocarcinoma. Pediatric biliary disorders may be embryologic or congenital, such as anatomic gallbladder anomalies, anomalous pancreaticobiliary tracts, various cholestatic processes, congenital cystic lesions, or genetic conditions. They may also be benign, such as biliary filling anomalies, biliary motility disorders, and biliary inflammatory and infectious disorders. Distinguishing these entities with a single imaging modality is challenging. US is the primary imaging modality for initial evaluation of biliary abnormalities in children, due to its wide availability, lack of ionizing radiation, and low cost and because it requires no sedation. Other examinations such as MRI, CT, and nuclear medicine examinations may provide anatomic and functional information to narrow the diagnosis further. Hepatobiliary-specific contrast material with MRI can provide better assessment of biliary anatomy on delayed images than can traditional MRI contrast material. MR cholangiopancreatography (MRCP) allows visualization of the intra- and extrahepatic biliary ducts, which may not be possible with endoscopic retrograde cholangiopancreatography (ERCP). Suspected biliary atresia requires multiple modalities for diagnosis and timely treatment. Determining the type of choledochal cyst calls for a combination of initial US and MRCP. Many benign and malignant biliary masses require biopsy for definitive diagnosis. Knowledge of the imaging appearances of different pediatric biliary abnormalities is necessary for appropriate imaging workup, providing a diagnosis or differential diagnosis, and guiding appropriate management. ©RSNA, 2024 Test Your Knowledge questions for this article are available in the supplemental material.

Oncologic Emergencies in the Chest, Abdomen, and Pelvis.

Oncology patients can present with acute, life-threatening conditions that may arise either due to underlying malignancy or secondary to cancer therapy. Select oncologic emergencies show characteristic imaging findings on radiographs, ultrasound, computed tomography, and MRI that helps in timely diagnosis. Radiologists need to be aware of typical imaging findings in such patients in an emergency setting and should be able to guide the clinicians for proper patient management. Appropriate knowledge of the treatment and its timing is pivotal in diagnosing treatment-related complications.

Prostate artery embolization has long term efficacy for treatment of severe lower urinary tract symptoms from giant prostatic hyperplasia.

BACKGROUND: Patients with severe lower urinary tract symptoms (LUTS) from giant prostatic hyperplasia (GPH): prostate volume greater than 200 mL that do not respond to medical therapy may not be eligible for surgical treatments due to morbidities, technical challenges, and patient preference. This retrospective investigation examined the long-term efficacy and safety of prostatic arterial embolization (PAE) as a treatment option for severe LUTS due to GPH in a large patient cohort. METHODS: Of 529 patients who underwent PAE between January 2016 and January 2020, 72 patients had severe LUTS from GPH and were retrospectively evaluated. PAE was performed with two embolic agents in sequence: 100-250 µm particles followed by 2 mm and 3 mm coils. Clinical assessment was performed with international prostate symptoms score (IPSS), quality of life (QoL), peak flow rate (Qmax), post-void residual volume (PVR), and prostate specific antigen (PSA) measurements before and 12 months and 24 months after PAE. Prostate volume (PV) was measured by multiparametric magnetic resonance (MR) imaging before and 12 months and 24 months after PAE. RESULTS: Patients with severe LUTS from GPH experienced significant clinical improvements in IPSS, QoL, Qmax, PVR, PSA, and PV at 12 months and 24 months after PAE. Mean IPSS decreased from 26.5 to 18.0 (P < 0.01) to 10.5 (P < 0.01). Mean QoL decreased from 6.0 to 4.0 (P < 0.01) to 2.0 (P < 0.01). Mean Qmax increased from 8.0 to 14 mL/s (P < 0.01) to 18 mL/s (P < 0.01). Mean PVR decreased from 198.0 to 152.0 mL (P < 0.01) to 90 mL (P < 0.01). Mean PV decreased from 303.0 mL to 258.0 mL (P < 0.01) to 209.0 mL (P < 0.01). Mean PSA decreased from 11.2 ng/mL to 9.5 ng/mL (P < 0.05) to 7.9 ng/mL (P < 0.05). No major complications occurred. CONCLUSIONS: PAE is a safe treatment with long term efficacy for severe LUTS from GPH. PAE may be a viable therapeutic option for patients with severe LUTS from GPH whom fail medical therapy and are not candidates for surgical treatments.

Magnetic resonance enterography, colonoscopy, and fecal calprotectin correlate in colonic Crohn's disease.

BACKGROUND: Fecal calprotectin (FCP), magnetic resonance enterography (MRE), and colonoscopy are complementary biometric tests that are used to assess patients with Crohn's Disease (CD). While prior studies have evaluated the association between combinations of these tests, no study has established a correlation between all three: FCP, MRE, and colonoscopy. We prospectively investigated if there is correlation between these three tests, which may result in improved clinical outcomes that can then be used to streamline patient monitoring and treatment modification. METHODS: One hundred fifty-six patients with colonic CD were prospectively examined between March 2017 and December 2018. FCP levels, MRE, and colonoscopy were assessed in parallel on all 156 patients. Clinical CD activity was measured with the Crohn's Disease Activity Index (CDAI). CD activity with FCP was measured with a quantitative immunoassay. CD activity on MRE was measured with the Magnetic Resonance Index of Activity (MaRIA). CD activity on colonoscopy was measured with the Crohn's Disease Endoscopic Index of Severity (CDEIS). RESULTS: One hundred twelve patients (72%) had active disease (Crohn's Disease Activity Index > 150) and 44 patients (28%) were in clinical remission disease (Crohn's Disease Activity Index < 150). FCP levels, MaRIA, and CDEIS are highly correlated with positive and significant Pearson and Spearman coefficients, respectively (P < 0.0001), in univariate analyses. Regression analysis (multivariate analyses) demonstrates significant, positive correlation between FCP and MaRIA (r = 1.07, P < 0.0001) and between FCP and CDEIS (r = 0.71, P = 0.03), and between. MaRIA and CDEIS (r = 0.63, P = 0.01). CONCLUSIONS: FCP levels significantly correlate with the degree of active inflammation in patients with colonic Crohn's Disease. Improved clinical results may be achieved by using a biometric strategy that incorporates FCP, colonoscopy, and MRE together. This strategy may in-turn be used in the future to streamline monitoring disease activity and adjustment of therapy to improve long term patient outcomes.

Radiogenomics of clear cell renal cell carcinoma: preliminary findings of The Cancer Genome Atlas-Renal Cell Carcinoma (TCGA-RCC) Imaging Research Group.

PURPOSE: To investigate associations between imaging features and mutational status of clear cell renal cell carcinoma (ccRCC). MATERIALS AND METHODS: This multi-institutional, multi-reader study included 103 patients (77 men; median age 59 years, range 34-79) with ccRCC examined with CT in 81 patients, MRI in 19, and both CT and MRI in three; images were downloaded from The Cancer Imaging Archive, an NCI-funded project for genome-mapping and analyses. Imaging features [size (mm), margin (well-defined or ill-defined), composition (solid or cystic), necrosis (for solid tumors: 0%, 1%-33%, 34%-66% or >66%), growth pattern (endophytic, <50% exophytic, or ≥50% exophytic), and calcification (present, absent, or indeterminate)] were reviewed independently by three readers blinded to mutational data. The association of imaging features with mutational status (VHL, BAP1, PBRM1, SETD2, KDM5C, and MUC4) was assessed. RESULTS: Median tumor size was 49 mm (range 14-162 mm), 73 (71%) tumors had well-defined margins, 98 (95%) tumors were solid, 95 (92%) showed presence of necrosis, 46 (45%) had ≥50% exophytic component, and 18 (19.8%) had calcification. VHL (n = 52) and PBRM1 (n = 24) were the most common mutations. BAP1 mutation was associated with ill-defined margin and presence of calcification (p = 0.02 and 0.002, respectively, Pearson's χ (2) test); MUC4 mutation was associated with an exophytic growth pattern (p = 0.002, Mann-Whitney U test). CONCLUSIONS: BAP1 mutation was associated with ill-defined tumor margins and presence of calcification; MUC4 mutation was associated with exophytic growth. Given the known prognostic implications of BAP1 and MUC4 mutations, these results support using radiogenomics to aid in prognostication and management.

Pediatric and adult primary sarcomas of the kidney: a cross-sectional imaging review.

There is a wide pathological spectrum of kidney sarcomas that show characteristic histology, ontogeny, and clinical-biological behavior. While leiomyosarcomas commonly arise from the capsule, solitary fibrous tumors and clear cell sarcomas typically show renal sinus and medullary epicenter, respectively. Although distribution and imaging findings of some sarcomas may be characteristic, definitive diagnosis warrants histopathological examination following surgery. Renal sarcomas manifest advanced disease at presentation and portend poor prognosis.