Image-Guided Precision Medicine in the Diagnosis and Treatment of Pheochromocytomas and Paragangliomas.

In this comprehensive review, we aimed to discuss the current state-of-the-art medical imaging for pheochromocytomas and paragangliomas (PPGLs) diagnosis and treatment. Despite major medical improvements, PPGLs, as with other neuroendocrine tumors (NETs), leave clinicians facing several challenges; their inherent particularities and their diagnosis and treatment pose several challenges for clinicians due to their inherent complexity, and they require management by multidisciplinary teams. The conventional concepts of medical imaging are currently undergoing a paradigm shift, thanks to developments in radiomic and metabolic imaging. However, despite active research, clinical relevance of these new parameters remains unclear, and further multicentric studies are needed in order to validate and increase widespread use and integration in clinical routine. Use of AI in PPGLs may detect changes in tumor phenotype that precede classical medical imaging biomarkers, such as shape, texture, and size. Since PPGLs are rare, slow-growing, and heterogeneous, multicentric collaboration will be necessary to have enough data in order to develop new PPGL biomarkers. In this nonsystematic review, our aim is to present an exhaustive pedagogical tool based on real-world cases, dedicated to physicians dealing with PPGLs, augmented by perspectives of artificial intelligence and big data.

Predicting death or recurrence of portal hypertension symptoms after TIPS procedures.

BACKGROUND: Accurate prediction of portal hypertension recurrence after transjugular intrahepatic portosystemic shunt (TIPS) placement will improve clinical decision-making. PURPOSE: To evaluate if perioperative variables could predict disease-free survival (DFS) in cirrhotic patients with portal hypertension (PHT) treated with TIPS. MATERIALS AND METHODS: We recruited 206 cirrhotic patients with PHT treated with TIPS, randomly assigned to training (n = 138) and validation (n = 68) sets. We recorded 7 epidemiological, 4 clinical, and 9 radiological variables. TIPS-distal end positioning (TIPS-DEP) measured the distance between the distal end of the stent and the hepatocaval junction on contrast-enhanced CT scans. In the training set, the signature was defined as the random forest for survival algorithm achieving the lowest error rate for the prediction of DFS which was landmarked 4 weeks after the TIPS procedure. In the training set, a simple to use scoring system was derived from variables selected by the signature. The primary endpoint was to assess if TIPS-DEP was associated with DFS. The secondary endpoint was to validate the scoring system in the validation set. RESULTS: Overall, patients with TIPS-DEP ≥ 6 mm (n = 49) had a median DFS of 24.5 months vs. 72.8 months otherwise (n = 157, p = 0.004). In the training set, the scoring system was calculated by adding age ≥ 60 years old, Child-Pugh B or C, and TIPS-DEP ≥ 6 mm (1 point each) since the signature showed high DFS probability at 6.5 months post-landmark in patients that did not meet these criteria: 86%, 80%, and 78%, respectively. The hazard ratio [95 CI] between patients determined to be low-risk (< 2 points) and high-risk (≥ 2 points) was 2.30 [1.35-3.93] (p = 0.002) in the training set and 2.01 [0.94-4.32] (p = 0.072) in the validation set. CONCLUSION: TIPS-DEP is an actionable radiological biomarker which can be combined with age and Child-Pugh score to predict death or PHT symptom recurrence after TIPS procedure. KEY POINTS: • TIPS-DEP measurement was the third most important but only actionable variable for predicting DFS. • TIPS-DEP < 6 mm was associated with a DFS probability of 78% at 6.5 months post-landmark. • A simple scoring system calculated using age, Child-Pugh score, and TIPS-DEP predicted DFS after TIPS.

Endovascular therapies for pulmonary embolism.

PURPOSE: The aim of this article is to define the place of new endovascular methods for the management of pulmonary embolisms (PE), on the basis of a multidisciplinary consensus. METHOD AND RESULTS: Briefly, from the recent literature, for high-risk PE presenting with shock or cardiac arrest, systemic thrombolysis or embolectomy is recommended, while for lowrisk PE, anticoagulation alone is proposed. Normo-tense patients with PE but with biological or imaging signs of right heart dysfunction constitute a group known as "at intermediate risk" for which the therapeutic strategy remains controversial. In fact, some patients may require more aggressive treatment in addition to the anticoagulant treatment, because approximately 10% will decompensate hemodynamically with a high risk of mortality. Systemic thrombolysis may be an option, but with hemorrhagic risks, particularly intra cranial. Various hybrid pharmacomechanical approaches are proposed to maintain the benefits of thrombolysis while reducing its risks, but the overall clinical experience of these different techniques remains limited. Patients with high intermediate and high risk pulmonary embolism should be managed by a multidisciplinary team combining the skills of cardiologists, resuscitators, pneumologists, interventional radiologists and cardiac surgeons. Such a team can determine which intervention - thrombolysis alone or assisted, percutaneous mechanical fragmentation of the thrombus or surgical embolectomy - is best suited to a particular patient. CONCLUSIONS: This consensus document define the place of endovascular thrombectomy based on an appropriate risk stratification of PE.

Combining Volumetric and Wall Shear Stress Analysis from CT to Assess Risk of Abdominal Aortic Aneurysm Progression.

Background Despite known limitations, the decision to operate on abdominal aortic aneurysm (AAA) is primarily on the basis of measurement of maximal aneurysm diameter. Purpose To identify volumetric and computational fluid dynamics parameters to predict AAAs that are likely to progress in size. Materials and Methods This study, part of a multicenter prospective registry (NCT01599533), included 126 patients with AAA. Patients were sorted into stable (≤10-mL increase in aneurysm volume) and progression (>10-mL increase in aneurysm volume) groups. Initial AAA characteristics of the derivation cohort were analyzed (maximal diameter and surface, thrombus and lumen volumes, maximal wall pressure, and wall shear stress [WSS]) to identify relevant parameters for a logistic regression model. Model and maximal diameter diagnostic performances were assessed in both cohorts and for AAAs smaller than 50 mm by using area under the receiver operating characteristic curve (AUC). Results Eighty-one patients were included (mean age, 73 years ± 7 years [standard deviation]; 78 men). The derivation and validation cohorts included, respectively, 50 and 31 participants. In the derivation cohort, there was higher mean lumen volume and lower mean WSS in the progression group compared with the stable group (60 mL ± 14 vs 46 mL ± 18 [P = .005] and 66% ± 6 vs 53% ± 9 [P = .02], respectively). Mean lumen volume and mean WSS at baseline were correlated to total volume growth (r = 0.47 [P = .002] and -0.42 [P = .006], respectively). In the derivation cohort, a regression model including lumen volume and WSS to predict aneurysm enlargement was superior to maximal diameter alone (AUC, 0.78 vs 0.52, respectively; P = .003); although no difference was found in the validation cohort (AUC, 0.79 vs 0.71, respectively; P = .51). For AAAs smaller than 50 mm, a regression model that included both baseline WSS and lumen volume performed better than maximal diameter (AUC, 0.79 vs 0.53, respectively; P = .01). Conclusion Combined analysis of lumen volume and wall shear stress was associated with enlargement of abdominal aortic aneurysms at 1 year, particularly in aneurysms smaller than 50 mm in diameter. © RSNA, 2020 Online supplemental material is available for this article. See also the editorial by Mitsouras and Leach in this issue.

Imaging-guided precision medicine in non-resectable gastro-entero-pancreatic neuroendocrine tumors: A step-by-step approach.

The majority of gastroenteropancreatic (GEP) neuroendocrine tumors (NETs) are diagnosed at a non-resectable stage due to non-specific clinical syndromes, late manifestations from mass effects, or incidental detection of a clinically silent disease. Management strategies include curative or cytoreduction surgery, imaging-guided intervention, chemotherapy, immunotherapy, and radionuclide therapies. In this step-by-step review, we provide a structured approach for standardized reading and reporting of medical imaging studies covering content and terminology. This review explains which imaging studies should be used for different NETs and what should be reported when interpreting these studies. This standardized data collection guide should enable precision medicine for the management of patients with GEP-NETs of neuroectodermal origin: gastrointestinal-NETs (giNETs) and pancreatic NETs (pNETs). To improve outcomes from GEP-NETs, it contains a comprehensive evaluation of imaging aids for determining surgical non-resectability, and serves as a surrogate measure for tumor differentiation and proliferation, assessing the spatial and temporal heterogeneity of the tumor sites with prognostic and therapeutic implications.

Prognostic impact of myocardial perfusion single photon emission computed tomography in patients with major extracardiac findings by computed tomography for attenuation correction.

BACKGROUND: Attenuation correction computed tomography (CT) contributes to an improvement in the diagnostic accuracy of myocardial perfusion imaging (MPI) by single photon emission tomography (SPECT). The aim of this study was to explore the prognosis of patients with major findings by CT according to the results of MPI. METHODS AND RESULTS: 1506 patients who underwent MPI by SPECT were retrospectively included. Attenuation correction CT images were systematically analyzed for major and minor abnormalities. 830 (55.1%) and 212 (14.1%) patients had minor and major extracardiac findings, respectively. Among patients with major extracardiac findings, the abnormality was previously unknown in 113 (53.3%) patients. 90 (41.9%) had abnormal MPI, 73 (34.4%) had a myocardial infarction scar, 55 (25.9%) had myocardial ischemia, and 38 (17.7%) patients had both myocardial infarction scar and myocardial ischemia. Among the 201 patients available for survival analysis, there were 67 (31.2%) deaths over a follow-up period of 3.2±1.3 years. There was no significant impact on survival arising from MPI, whatever the result. The results were the same among the 103 patients with previously unknown major extracardiac findings. CONCLUSION: Extracardiac findings by CT during MPI are frequent. Patients with major extracardiac findings have a poor mid-term outcome, whatever the results of the myocardial perfusion imaging. Extracardiac findings should be systematically checked when attenuation correction CT is performed.

Pituitary Stalk Interruption Syndrome from Infancy to Adulthood: Clinical, Hormonal, and Radiological Assessment According to the Initial Presentation.

BACKGROUND: Patients with pituitary stalk interruption syndrome (PSIS) are initially referred for hypoglycemia during the neonatal period or growth retardation during childhood. PSIS is either isolated (nonsyndromic) or associated with extra-pituitary malformations (syndromic). OBJECTIVE: To compare baseline characteristics and long-term evolution in patients with PSIS according to the initial presentation. STUDY DESIGN: Sixty-seven patients with PSIS were included. Data from subgroups were compared: neonates (n = 10) versus growth retardation patients (n = 47), and syndromic (n = 32) versus nonsyndromic patients (n = 35). RESULTS: Neonates displayed a more severe hormonal and radiological phenotype than children referred for growth retardation, with a higher incidence of multiple hormonal deficiencies (100% versus 34%; P = 0.0005) and a nonvisible anterior pituitary lobe (33% versus 2%; P = 0.0017). Regular follow-up of growth might have allowed earlier diagnosis in the children with growth retardation, as decreased growth velocity and growth retardation were present respectively 3 and 2 years before referral. We documented a progressive worsening of endocrine impairment throughout childhood in these patients. Presence of extra-pituitary malformations (found in 48%) was not associated with more severe hormonal and radiological characteristics. Growth under GH treatment was similar in the patient groups and did not vary according to the pituitary MRI findings. CONCLUSIONS: PSIS diagnosed in the neonatal period has a particularly severe hormonal and radiological phenotype. The progressive worsening of endocrine impairment throughout childhood justifies periodic follow-up to check for additional hormonal deficiencies.