Radiomics of Intrahepatic Cholangiocarcinoma and Peritumoral Tissue Predicts Postoperative Survival: Development of a CT-Based Clinical-Radiomic Model.

BACKGROUND: For many tumors, radiomics provided a relevant prognostic contribution. This study tested whether the computed tomography (CT)-based textural features of intrahepatic cholangiocarcinoma (ICC) and peritumoral tissue improve the prediction of survival after resection compared with the standard clinical indices. METHODS: All consecutive patients affected by ICC who underwent hepatectomy at six high-volume centers (2009-2019) were considered for the study. The arterial and portal phases of CT performed fewer than 60 days before surgery were analyzed. A manual segmentation of the tumor was performed (Tumor-VOI). A 5-mm volume expansion then was applied to identify the peritumoral tissue (Margin-VOI). RESULTS: The study enrolled 215 patients. After a median follow-up period of 28 months, the overall survival (OS) rate was 57.0%, and the progression-free survival (PFS) rate was 34.9% at 3 years. The clinical predictive model of OS had a C-index of 0.681. The addition of radiomic features led to a progressive improvement of performances (C-index of 0.71, including the portal Tumor-VOI, C-index of 0.752 including the portal Tumor- and Margin-VOI, C-index of 0.764, including all VOIs of the portal and arterial phases). The latter model combined clinical variables (CA19-9 and tumor pattern), tumor indices (density, homogeneity), margin data (kurtosis, compacity, shape), and GLRLM indices. The model had performance equivalent to that of the postoperative clinical model including the pathology data (C-index of 0.765). The same results were observed for PFS. CONCLUSIONS: The radiomics of ICC and peritumoral tissue extracted from preoperative CT improves the prediction of survival. Both the portal and arterial phases should be considered. Radiomic and clinical data are complementary and achieve a preoperative estimation of prognosis equivalent to that achieved in the postoperative setting.

Vertebral artery dissection caused by atlantoaxial dislocation in a patient with Marfan syndrome.

A small number of case reports have documented a link between atlantoaxial dislocation (AAD) and vertebral artery dissection (VAD), but this association has never been described in patients with hereditary connective tissue disorders. We present a case of an 18-year-old female patient, diagnosed with Marfan syndrome since the age of one, who underwent brain MRA for intracranial aneurysm screening revealing tortuosity of the internal carotid and vertebral arteries as well as atlantoaxial dislocation. Since the patient was asymptomatic, a wait-and-see approach was chosen, but a follow-up MRA after 18 months showed the appearance of a dissecting pseudoaneurysm of the V3 segment of the left vertebral artery. Despite the patient being still asymptomatic, it was decided to proceed with C1-C2 stabilization to prevent further vascular complications. Follow-up imaging showed realignment of the atlantoaxial joint and reduction of the dissecting pseudoaneurysm of the left vertebral artery. In our patient, screening MRA has led to the discovery of asymptomatic arterial and skeletal abnormalities which, if left untreated, might have led to severe cerebrovascular complications. Therefore, AAD correction or close monitoring with MRA should be provided to MFS patients with this craniovertebral junction anomaly, even if asymptomatic.

Quantitative measurement of dural ectasia: associations with clinical and genetic characteristics in Marfan syndrome.

PURPOSE: Dural ectasia (DE) may significantly impact Marfan syndrome (MFS) patients' quality of life due to chronic lower back pain, postural headache and urinary disorders. We aimed to evaluate the association of quantitative measurements of DE, and their evolution over time, with demographic, clinical and genetic characteristics in a cohort of MFS patients. METHODS: We retrospectively included 88 consecutive patients (39% females, mean age 37.1 ± 14.2 years) with genetically confirmed MFS who underwent at least one MRI or CT examination of the lumbosacral spine. Vertebral scalloping (VS) and dural sac ratio (DSR) were calculated from L3 to S3. Likely pathogenic or pathogenic FBN1 variants were categorized as either protein-truncating or in-frame. The latter were further classified according to their impact on the cysteine content of fibrillin-1. RESULTS: Higher values of the systemic score (revised Ghent criteria) were associated with greater DSR at lumbar (p < 0.001) and sacral (p = 0.021) levels. Patients with protein-truncating variants exhibited a greater annual increase in lumbar (p = 0.039) and sacral (p = 0.048) DSR. Mutations affecting fibrillin-1 cysteine content were linked to higher VS (p = 0.009) and DSR (p = 0.038) at S1, along with a faster increase in VS (p = 0.032) and DSR (p = 0.001) in the lumbar region. CONCLUSION: Our study shed further light on the relationship between genotype, dural pathology, and the overall clinical spectrum of MFS. The identification of protein-truncating variants and those impacting cysteine content may therefore suggest closer patient monitoring, in order to address potential complications associated with DE.

Spectrum of imaging findings of primary bone lymphoma in pediatric patients.

Primary bone lymphoma, a rare oncologic entity, may initially present with minimal symptoms. Presenting symptoms range from local pain and mild systemic symptoms to large palpable masses and pathologic fractures. The term "primary bone lymphoma" indicates the finding of bone involvement without other organ sites for at least 6 months. Although some radiological features may raise suspicion about this tumor form, there are no pathognomonic imaging findings, and the diagnosis will likely be delayed for a long time. The most critical radiological feature is soft tissue involvement associated with a preserved cortical layer, much more than expected for an infiltrating lesion. Anyway, very different radiological findings may be displayed in patients with primary bone lymphoma. Although these radiological features of primary bone lymphoma have been discussed in the literature by various authors, there is little data concerning imaging in pediatric patients. This paper aims to depict the possible spectrum of imaging features of primary bone lymphoma in the pediatric age, providing an exemplification pictorial essay extracted from a single institution experience in the year range period 2006-2022.

Analysis of serological treatment response to doxycycline versus benzathine penicillin in syphilis infections, a retrospective single-center study.

Doxycilicine is the second-line treatment of choice for infectious syphilis when treatment with penicillin G is not feasible. To date, difficulties in the penicillin supply chain make it necessary to evaluate and resort to antibiotic therapies which are currently considered a second-line choice. Moreover, systematic studies comparing the two treatments in affected patients are still few, and many do not consider late and indeterminate latent infections. The objective of this study was to assess the differences in the serological response of the treatment of syphilis infections with benzathine penicillin compared with doxycycline. We built an in-house database with all patients diagnosed with syphilis infection from January 2010 to January 2020 in the STD Centre of the S.Orsola-Malpighi Polyclinic of the University of Bologna, located in the North-east of Italy. We recorded all the principal independent (demographic, social status, reinfection rare, HIV infections, comorbidities, sexual behaviors, and initial TPHA values) and dependent variables (RPR values). We then extrapolated all patients treated with doxycycline (100 mg of doxycycline twice daily for 14 days for infections diagnosed within the first year and a 28 days course for infections older than 1 year or undetermined) and matched in 1:1 ratio numbers with a homogeneous group of patients treated with penicillin G (2.4 million units in a single dose intramuscularly for infections diagnosed within the first year and a cycle consisting in of 2.4 million units administered in a single dose per week for 3 weeks for infections older than 1 year or undetermined) We then analyzed the serological trends and outcomes in the primary, secondary and early latent groups versus late latent and undetermined infections. We retrieved 41 patients for each group with homogeneous initial characteristics. At the end of the 24-month observation period, a slight difference in a valid RPR reduction rate emerged, with a greater success rate emerged in patients receiving penicillin than those with doxycycline (26 vs. 22, p 0.615). Indeed, patients with latent or indeterminate syphilis treated with doxycycline appear to have a higher rate of serofast than those treated with penicillin. Linear regression analysis showed no strong correlation between the analyzed independent variables and the observed outcomes. Doxycycline had a slightly lower, though not statistically different, success rate when compared with penicillin in treating primary syphilis, but appeared to have a reduced success rate in attaining resolution in late and undetermined syphilis infection.

Atlantoaxial rotatory subluxation/fixation and Grisel's syndrome in children: clinical and radiological prognostic factors.

Atlantoaxial rotatory subluxation/fixation (AARS/F) is a rare cause of torticollis in children. The aim of our study is to investigate all possible clinical and radiological prognostic factors in children with AARS/F. We retrospectively reviewed all cervical spine CT scans of children with AARS/F treated in our Hospital over the last 15 years. AARS/F was classified according to Fielding and Hawkins classification and C1-C2 rotation-degree was calculated. Moreover, two orthopedic surgeons reviewed all clinical reports of these children. All patients were conservatively treated (cervical traction/neck collar). An early recovery was considered in patients with complete clinical/radiological healing at 3 months follow-up, while a late recovery was considered in patients with disease persistence or relapse at 3 months follow-up or earlier. Fifty-five patients with diagnosis of AARS/F were included in the study (mean age = 8.5 years old - 25F, 30M). In 9/55 subjects (16.4%), a late recovery was observed. The presence of a concomitant infection or inflammation in the head and neck region (Grisel's syndrome) was significantly associated with a late recovery (p < .001). Also, the type of AARS/F (p = .019), according to the Fielding and Hawkins classification, and C1-C2 rotation-degree (p = .027) were significantly correlated with the recovery time.Conclusion: In patients with AARS/F, the presence of a concomitant infection/inflammation in the head and neck region is the most important prognostic factor and it is associated with a late recovery. The Fielding and Hawkins classification and C1-C2 rotation-degree well correlate with patients' recovery time. What is Known: • Atlantoaxial rotatory subluxation/fixation (AARS/F) is a rare cause of torticollis in children and CT is the most useful imaging tool for diagnosis and classification of AARS/F. • Conservative treatments are effective in the majority of pediatric patients with AARS/F. What is New: • The presence of a concomitant infection/inflammation in the head and neck region associated with AARS/F (Grisel's syndrome) is the most important prognostic factor and it is associated with a late recovery. • C1-C2 rotation-degrees, as well as Fielding and Hawkins classification system, well correlate with patients' recovery time.

Homogeneous blue lumps of the nipple-areola complex in pubertal girls.

Retroareolar cysts are benign breast lesions caused by the obstruction and consequent dilatation of Montgomery tubercles. Herein, we report two cases of premenarchal girls who developed retroareolar cysts. Their course and differential diagnosis are discussed.

Congenital fibrous hamartoma of the tip of the tongue: A novel peculiar entity.

Congenital fibrous hamartoma of the tip of the tongue, a peculiar and novel entity, consists of one or two asymptomatic pearly or yellowish nodules, not exceeding 0.5 cm in maximum diameter, at the tip of the tongue, ventrally or dorsally. Unlike other localizations, congenital fibrous hamartoma of the tip of the tongue is not associated with cleft lip or palate, or with feeding problems. Surgical excision should be avoided, reserved only for dubious cases, since the lesions are benign and stable over time.

Keep CALME (childhood asymmetry labium majus enlargement) and follow up.

CALME is the abbreviation of "childhood asymmetry labium majus enlargement" and denotes a physiological, non-neoplastic swelling of labium majus soft tissues. It is caused by the expansion of one or more normal vulvar tissue components and is devoid of a peripheral capsule. A few reports in the literature have raised awareness of this benign condition and stressed the need to avoid invasive procedures with possible permanent sequelae. The etiopathogenesis of CALME is still debated, although the hypothesis of a hormone-driven major labia stromal hyperplasia is favored over the assumption of a benign neoplastic process. This article aims to provide a detailed review of the clinical, histological, and instrumental findings of CALME and provide a step-based algorithm to approach and manage genito-inguinal masses in pediatric patients.

Bei CALME (childhood asymmetry labium majus enlargement): die Ruhe bewahren und nachverfolgen.

CALME steht für childhood asymmetry labium majus enlargement und bezeichnet eine physiologische, nichtneoplastische Schwellung des Weichteilgewebes der Labia majora. CALME wird durch die Vergrößerung einer oder mehrerer normaler vulvärer Gewebeanteile verursacht und weist keine äußere Kapsel auf. Vereinzelte Berichte in der Literatur haben das Augenmerk auf diese gutartige Veränderung gelenkt und die Notwendigkeit betont, invasive Eingriffe mit möglichen Dauerfolgen zu vermeiden. Die Ätiopathogenese von CALME ist nach wie vor umstritten, wobei die Hypothese einer hormonell bedingten stromalen Hyperplasie der großen Schamlippen gegenüber der Annahme eines gutartigen neoplastischen Prozesses favorisiert wird. Dieser Artikel zielt darauf ab, einen detaillierten Überblick über die klinischen, histologischen und apparativen Befunde bei CALME zu geben und einen abgestuften Algorithmus zur Herangehensweise und zum Management von genito-inguinalen Geschwülsten bei pädiatrischen Patienten zu erstellen.

Percutaneous management of postoperative Bile leak after hepato-pancreato-biliary surgery: a multi-center experience.

BACKGROUND: Bile leak (BL) after hepato-pancreato-biliary (HPB) surgery is associated with significant morbidity and mortality. Aim of this study was to evaluate effectiveness and safety of percutaneous transhepatic approach (PTA) to drainage BL after HPB surgery. METHODS: Between 2006 and 2018, consecutive patients who were referred to interventional radiology units of three tertiary referral hospitals were retrospectively identified. Technical success and clinical success were analyzed and evaluated according to surgery type, BL-site and grade, catheter size and biochemical variables. Complications of PTA were reported. RESULTS: One-hundred-eighty-five patients underwent PTA for BL. Technical success was 100%. Clinical success was 78% with a median (range) resolution time of 21 (5-221) days. Increased clinical success was associated with patients who underwent hepaticresection (86%,p = 0,168) or cholecystectomy (86%,p = 0,112) while low success rate was associated to liver-transplantation (56%,p < 0,001). BL-site,grade, catheter size and AST/ALT levels were not associated with clinical success. ALT/AST high levels were correlated to short time resolution (17 vs 25 days, p = 0,037 and 16 vs 25 day, p = 0,011, respectively) Complications of PTA were documented in 21 (11%) patients. CONCLUSION: This study based on a large cohort of patients demonstrated that PTA is a valid and safe approach in BL treatment after HPB surgery.

Ultrasound features of the subungual glomus tumor and squamous cell carcinomas.

BACKGROUND: Nail tumors often pose a significant challenge for the clinician, as they are burdened by a high delay in diagnosis and paucity of clinical signs in the early stage. In most cases, the onset of pain or nail plate dystrophy leads to imaging investigations such as plain radiography, magnetic resonance imaging, and ultrasonography (US) when surgery is pending. MATERIAL AND METHODS: A 2-year monocentric study evaluated the histologically confirmed cases of subungual glomus tumor (GT) and squamous cell carcinoma (SCC) with a pre-surgical US assessment, and reviewed their sonography features. A total of 7 digital GTs and 6 SCCs of the nail were collected. RESULTS: GT and SCC are easily distinguishable on US. Compared to SCC, GT was positively associated with an ovoid shape, well-defined borders, bone cup-scalloping without cortical erosion, and a rich intralesional vascular pattern (P < 0.05). Vice versa, SCC was significantly correlated with irregular shape, ill-circumscribed margins, an infiltrative growth pattern with peripheral hypoechoic foci, posterior acoustic shadow, cortical erosion, and a highly vascular multipolar peripheral pattern (P < 0.05). CONCLUSION: The rapid assessment of GT and SCC sonographic features may contribute to reduce diagnostic delay, expedite management, and decrease the risk of surgical relapse.

Prognostic value of posttreatment 18F-FDG PET/CT and predictors of metabolic response to therapy in patients with locally advanced cervical cancer treated with concomitant chemoradiation therapy: an analysis of intensity- and volume-based PET parameters.

PURPOSE: To investigate the prognostic value of posttreatment 18F-FDG PET/CT in patients with locally advanced cervical cancer (LACC) treated with concomitant chemoradiation therapy (CCRT). The secondary aim was to assess the possible role of intensity-based and volume-based PET parameters including SUVmax, SUVmean, MTV and TLG, and clinical parameters including age, pathology, FIGO stage and nodal involvement as factors predicting response to treatment. METHODS: This retrospective study included 82 patients affected by LACC treated with CCRT. All patients underwent 18F-FDG PET/CT both before and after treatment. The posttreatment PET/CT scans were used to classify patients as complete metabolic responders (CMR) or non-complete metabolic responders (N-CMR) according to the EORTC criteria. Kaplan-Meier analysis was used to evaluate differences in overall survival (OS) between the CMR and N-CMR groups. Student's t test, Pearson's chi-squared test and logistic regression were used to investigate the possible value of PET and clinical parameters as predictors of metabolic response to therapy. RESULTS: Kaplan-Meier analysis showed a highly significant difference in OS between the CMR and N-CMR groups (log-rank test p < 0.0001). Significant independent predictors of response to therapy were MTV (p = 0.019, odds ratio = 1.015, 95% CI = 1.002-1.028, Nagelkerke R2 = 0.110), TLG (p = 0.045, odds ratio = 1.001, 95% CI = 1.000-1.002, Nagelkerke R2 = 0.081) and nodal involvement (p = 0.088, odds ratio = 2.361, 95% CI = 0.879-6.343, Nagelkerke R2 = 0.051). CONCLUSION: 18F-FDG PET/CT-based response assessment using the EORTC criteria reliably predicts OS in LACC patients treated with CCRT. In our cohort of patients, pretreatment MTV and TLG and nodal involvement were predictors of response to therapy. MTV was the best predictor of response. However, its additional risk value seems to be low (MTV odds ratio = 1.015).

In Vivo Total Ankle Arthroplasty Kinematic Evaluation: A Prospective Radiostereometric Analysis.

Ankle osteoarthritis (OA) represents a significant social burden and is one of the main causes of chronic disability in a rapidly growing part of the world's population. Total ankle arthroplasty (TAA) has become increasingly popular despite the poor results obtained with the first dedicated designs. The purpose of this paper was to evaluate the ankle kinematics, in vivo and under weight-bearing conditions, of a TAA through a dynamic model-based radiostereometric analysis (MB-RSA). The clinical evaluation was performed by administering the American Orthopaedic Foot and Ankle Society ankle-hindfoot score and Short Form-36 questionnaires. The kinematic evaluation was conducted through MB-RSA during the execution of an open kinetic chain and a closed kinetic chain motor task. Double radiographic images of the ankle joint were processed using dedicated software to obtain a 3D reconstruction of the ankle prosthetic components' motion. Eighteen patients (five females) completed the clinical and instrumental preoperative and postoperative evaluations (age 59.1 ± 10.3). All clinical scores showed a marked improvement (p < 0.005). During the closed kinetic chain motor tasks, the ankle showed a total range of motion (ROM) in dorsi-plantarflexion of 19.84°. The parameters in varus-valgus were recorded. Physiological motion can be achieved in TAA, characterized by a wide range of motion and coupling of movements on the three planes. The results of the present work may help to understand the real movement of a widespread TAA model and possibly to improve future designs and instrumentation.

Impact of Sarcopenia on the Survival of Patients with Hepatocellular Carcinoma Treated with Sorafenib.

BACKGROUND AND AIMS: Sarcopenia has been associated with poor outcomes in patients with cirrhosis and hepatocellular carcinoma. We investigated the impact of sarcopenia on survival in patients with advanced hepatocellular carcinoma treated with Sorafenib. METHODS: A total of 328 patients were retrospectively analyzed. All patients had an abdominal CT scan within 8 weeks prior to the start of treatment. Two cohorts of patients were analyzed: the "Training Group" (215 patients) and the "Validation Group" (113 patients). Sarcopenia was defined by reduced skeletal muscle index, calculated from an L3 section CT image. RESULTS: Sarcopenia was present in 48% of the training group and 50% of the validation group. At multivariate analysis, sarcopenia (HR: 1.47, p = 0.026 in training; HR 1.99, p = 0.033 in validation) and MELD > 9 (HR: 1.37, p = 0.037 in training; HR 1.78, p = 0.035 in validation) emerged as independent prognostic factors in both groups. We assembled a prognostic indicator named "SARCO-MELD" based on the two independent prognostic factors, creating three groups: group 1 (0 prognostic factors), group 2 (1 factor) and group 3 (2 factors), the latter with significantly worse survival and shorter time receiving treatment.

Palliative Arterial Embolization for Metastases of the Sternum.

BACKGROUND: To investigate the safety and efficacy of palliative arterial embolization for metastases of the sternum. MATERIALS AND METHODS: This study included 10 consecutive patients (5 M, 5 F; mean age 58.1; range 37-70) with metastases of the sternum from different primary tumors, treated with palliative arterial embolization using NBCA-Lipiodol between January 2007 and June 2022. Four patients received a second embolization at the same site, for a total of 14 embolizations. Data on technical and clinical success, as well as changes in tumor size, were collected. All embolization-related complications were evaluated according to the CIRSE classification system for complications. RESULTS: Post-embolization angiography showed occlusion of more than 90% of the pathological feeding vessels in all procedures. Pain score and analgesic drug consumption were reduced by 50% in all 10 patients (100%, p < 0.05). The mean duration of pain relief was 9.5 months (range 8 to 12 months, p < 0.05). Metastatic tumor size was reduced from a mean of 71.5 cm3 (range 41.6 to 90.3 cm3) pre-embolization to a mean of 67.9 cm3 (range 38.5 to 86.1 cm3) at the 12-month follow-up (p < 0.05). None of the patients experienced embolization-related complications. CONCLUSION: Arterial embolization is safe and effective as a palliative treatment for patients with metastases of the sternum who did not benefit from radiation therapy or experienced recurrence in symptoms.

Update of the Bologna Experience in Radioembolization of Intrahepatic cholangiocarcinoma.

BACKGROUND: Intrahepatic cholangiocarcinoma (ICC) is the second most common primitive liver cancer and is rising in incidence worldwide. Given its low survival and resectability rates, locoregional therapies such as Yttrium-90 transarterial radioembolization (Y-TARE) are increasingly being employed. This retrospective study aim was to confirm and update our previous results about overall survival (OR), safety, and efficacy of Y-TARE in patients with unresectable/recurrent ICC. MATERIALS AND METHODS: OS was evaluated as primary endpoint while radiological tumor response at 3 months, based on Response Evaluation Criteria in Solid Tumors (RECIST) 1.1, was considered as secondary endpoint. RESULTS: Over a total of 49 patients, the overall median survival was 16 months (27-41 months, 95% confidence interval [CI]) from Y-TARE procedure. A significantly longer survival was recorded in naive patients compared to patients previously submitted to any type of liver-directed treatment and radical surgery (18 vs 14 months, P=.015 and 28 vs 14 months, P=.001, respectively). Target lesion and overall objective response for RECIST 1.1 criteria were 64.6% and 52.1%, respectively. Low rates of postprocedural and late complications were recorded. CONCLUSIONS: In unresectable and recurrent ICC, Y-TARE confirms its safety and its potential in increasing OS, especially in naive patients.