Decreased Diversity and Abundance of Marine Invertebrates at CO2 Seeps in Warm-Temperate Japan.

Japan has many coastal carbon dioxide seeps as it is one of the most volcanically active parts of the world. These shallow seeps do not have the spectacular aggregations of specialist fauna seen in deep-sea vent systems but they do have gradients in seawater carbonate chemistry that are useful as natural analogues of the effects of ocean acidification on marine biodiversity, ecosystem function and fisheries. Here, we compare macroinvertebrate diversity and abundance on rocky habitats at ambient (mean ≤ 410 µatm) and high (mean 971-1484 µatm) levels of seawater pCO2 in the warm-temperate region of Japan, avoiding areas with toxic sulphur or warm-water conditions. We show that although 70% of intertidal taxa and 40% of shallow subtidal taxa were able to tolerate the high CO2 conditions, there was a marked reduction in the abundance of corals, bivalves and gastropods in acidified conditions. A narrower range of filter feeders, grazers, detritivores, scavengers and carnivores were present at high CO2 resulting in a simplified coastal system that was unable to retain the high standing stocks of marine carbon biomass found in ambient conditions. It is clear that cuts in CO2 emissions would reduce the risks of climate change and ocean acidification impacts on marine biodiversity, shellfish production and biomass in the rocky coastal shores of this region.

Spinal cord compression as tumor onset: an unusual case report of Hodgkin lymphoma in a teenager.

BACKGROUND: Spinal cord compression (SCC) is an uncommon, severe complication of Hodgkin lymphoma (HL), occurring in 0.2% of cases at the onset and in 6% during disease progression. We present a teenager with SCC with clinical onset of HL; her pre-existing neurological abnormalities covered the presence of an epidural mass, which could have misled us. CASE PRESENTATION: A 13-year-old girl presented with a three-month history of lower back pain and degrading ability to walk. She suffered from a chronic gait disorder due to her preterm birth. A magnetic resonance imaging of the spine revealed an epidural mass causing collapse of twelfth thoracic vertebra and thus compression and displacement of the spinal cord. Histological examination with immunohistochemical analysis of the epidural mass demonstrated a classic-type Hodgkin lymphoma. Early pathology-specific treatment allowed to avoid urgent surgery, achieve survival and restore of neurological function. CONCLUSIONS: Children and adolescents with back pain and neurological abnormalities should be prioritized to avoid diagnostic delay resulting in potential loss of neurological function. SCC requires a prompt radiological assessment and an expert multidisciplinary management.

Bilateral adrenal neuroblastoma in the infant: Is it an image-defined risk factor?

The treatment of neuroblastoma is based on the International Neuroblastoma Risk Group stratification considering life-threatening symptoms, image-defined risk factors (IDRFs), presence and site of metastases, biology, and histopathology. The authors present an infant with bilateral nonmetastatic adrenal neuroblastoma with favorable biology. Both tumors were resectable and without IDRFs, but bilateral resection was considered mutilating, so it was decided to operate one side only. The authors suggest considering bilaterality among IDRFs.

Long-term local disease control in a recurrent soft-tissue sarcoma of the thigh treated by radiofrequency ablation.

In 2008, we performed radiofrequency ablation (RFA) in an elderly patient with a large recurrent soft-tissue sarcoma of the thigh, previously treated with surgery and radiotherapy. After ablation, a marked shrinkage of tumor was obtained. Further local recurrences occurred during follow-up, all safely treated by RFA, with local control of the disease maintained until 6-year follow-up. RFA was safe, effective, and repeatable for soft-tissue sarcoma recurrences, and allowed long-term local control of the disease.

Preliminary Results in Unresectable Head and Neck Cancer Treated by Radiofrequency and Microwave Ablation: Feasibility, Efficacy, and Safety.

PURPOSE: To retrospectively determine whether the application of thermal ablation to recurrent and advanced head and neck cancer (HNC) could allow for local tumor control. MATERIALS AND METHODS: From 2002 to 2014, 22 patients (17 men and 5 women; mean age, 64 y; age range, 42-88 y) with unresectable HNC lesions treated with thermal ablation were evaluated. Patients were followed at 3 and 6 months after treatment, every 6 months for 5 years, and yearly thereafter with computed tomography and/or magnetic resonance imaging. The mean follow-up period was 32.2 months (range, 3-51 mo). RESULTS: Three of 22 patients with primary lesions are still alive; two of these patients were treated with radiofrequency (RF) ablation and one was treated with microwave (MW) ablation. Of the 19 remaining patients, four were treated with MW ablation and 15 were treated with RF ablation. Imaging revealed partial response in eight patients, and complete response was observed in the remaining 14. There were two major complications after ablation treatment. The mean survival time was 32.9 months ± 3.205 (standard error; 95% confidence interval [CI], 26.6-39.2 mo). The survival time for MW ablation cases (36 mo ± 5.185; 95% CI, 25.8-46.16 mo) was longer than for RF ablation (32.2 mo ± 3.911; 95% CI, 24.5-39.8 mo), although the CI overlap between the groups is large. CONCLUSIONS: Percutaneous thermal ablation is a promising alternative treatment for local control of incurable HNC.

Magnetic Resonance Imaging of Central Nervous System Manifestations of Type 1 Neurofibromatosis: Pictorial Review and Retrospective Study of Their Frequency in a Cohort of Patients.

Background: type 1 neurofibromatosis (NF1) is the most common neurocutaneous disorder, and it is an inherited condition that causes a tumour predisposition. Central nervous system (CNS) manifestations are a significant cause of morbidity and mortality in NF1. We provide a pictorial review of neuroradiological features of NF1, with emphasis on magnetic resonance imaging (MRI), and we assess the frequency of those features on a cohort of NF1 patients. Methods: we retrospectively evaluated all patients with a diagnosis of NF1 who underwent MRI of the spine and brain in our centre over a period of almost 5 years. A total of 74 patients were enrolled, 28 males and 46 females, with a mean age of 21 ± 12.67 years. The frequency of CNS manifestations encountered in our cohort of NF1 patients was assessed and compared with the data found in other studies published in the literature. Results: many of our findings were in line with the literature, and possible interpretations for those that turned out to be different were suggested in the discussion. Conclusion: imaging plays a central role in the diagnosis and management of NF1, and the knowledge of CNS manifestations could be critical for its early detection and identification, such as for treatment planning and prognostic implications.

Congenital Lung Malformations: A Pictorial Review of Imaging Findings and a Practical Guide for Diagnosis.

The term congenital lung malformation (CLM) is used to describe a wide range of pathological conditions with different imaging and clinical manifestations. These anomalies stem from abnormal embryological lung development, potentially occurring across various stages of prenatal life. Their natural history can be variable, presenting in a wide range of severity levels and encompassing asymptomatic individuals who remain so until adulthood, as well as those who experience respiratory distress in the neonatal period. Through the PubMed database, we performed an extensive review of the literature in the fields of congenital lung abnormalities, including their diagnostic approach and findings. From our RIS-PACS database, we have selected cases with a final diagnosis of congenital lung malformation. Different diagnostic approaches have been selected, including clinical cases studied using plain radiograph, CT scan, prenatal ultrasound, and MR images. The most encountered anomalies can be classified into three categories: bronchopulmonary anomalies (congenital pulmonary airway malformations (CPAMs), congenital lobar hyperinflation, bronchial atresia, and bronchogenic cysts), vascular anomalies (arteriovenous malformation), and combined lung and vascular anomalies (scimitar syndrome and bronchopulmonary sequestration). CLM causes significant morbidity and mortality; therefore, the recognition of these abnormalities is necessary for optimal prenatal counseling and early peri- and postnatal management. This pictorial review aims to report relevant imaging findings in order to offer some clues for differential diagnosis both for radiologists and pediatric consultants.

Consensus Guidelines of Irreversible Electroporation for Pancreatic Tumors: Protocol Standardization Using the Modified Delphi Technique.

Since no uniform treatment protocol for pancreatic irreversible electroporation (IRE) exists, the heterogeneity throughout literature complicates the comparison of results. To reach agreement among experts, a consensus study was performed. Eleven experts, recruited according to predefined criteria regarding previous IRE publications, participated anonymously in three rounds of questionnaires according to a modified Delphi technique. Consensus was defined as having reached ≥80% agreement. Response rates were 100, 64, and 64% in rounds 1 to 3, respectively; consensus was reached in 93%. Pancreatic IRE should be considered for stage III pancreatic cancer and inoperable recurrent disease after previous local treatment. Absolute contraindications are ventricular arrhythmias, implantable stimulation devices, congestive heart failure NYHA class 4, and severe ascites. The inter-electrode distance should be 10 to 20 mm and the exposure length should be 15 mm. After 10 test pulses, 90 treatment pulses of 1,500 V/cm should be delivered continuously, with a 90-µs pulse length. The first postprocedural contrast-enhanced computed tomography should take place 1 month post-IRE, and then every 3 months. This article provides expert recommendations regarding patient selection, procedure, and follow-up for IRE treatment in pancreatic malignancies through a modified Delphi consensus study. Future studies should define the maximum tumor diameter, response evaluation criteria, and the optimal number of preoperative FOLFIRINOX cycles.

Natural history of neurofibromatosis type 2 with onset before the age of 1 year.

Neurofibromatosis type 2 (NF2) with onset before the first year of life has been anecdotally reported in the literature. We (a) prospectively (years 1997-2012) followed up three unrelated NF2 children, all harbouring NF2 gene mutations whose onset of disease was before age 1 year, and (b) systematically reviewed published reports on NF2 in the youngest age group (i.e. onset <1 year). The present three children had (1) small (<1 cm), bilateral vestibular schwannomas (VSs) detected (as an incidental finding) at magnetic resonance imaging (MRI) by the age of 4 to 5 months that were asymptomatic for 10 to 14 years, with sudden and rapid (<12 months) progression in two cases at the age of 11 and 15 years, respectively; (2) development of large numbers of skin NF2 plaques mainly in atypical locations (i.e. face, hands, legs and knees), which reverted to normal skin appearance at the time of VSs progression; (3) lens opacities (n = 1) and NF2 retinal changes (n = 2) detected as early as age of 3-4 months; (4) diffuse (asymptomatic) high signal lesions at brain MRI in the periventricular regions (alike cortical dysplasia); and (5) unaffected first-degree relatives who did not harbour NF2 gene abnormalities. This represents the youngest NF2 group with the longest prospective follow-up so far reported. NF2 may present as a congenital form with bilateral VSs presenting as early as the first months of life and with natural history different to that which occurs in classical NF2.

Case report: A gain-of-function of hamartin may lead to a distinct "inverse TSC1-hamartin" phenotype characterized by reduced cell growth.

Mutations of TSC1 and TSC2 genes cause classical Tuberous Sclerosis Complex (TSC), a neurocutaneous disorder characterized by a tendency to develop hamartias, hamartomas, and other tumors. We herein report on a girl, now aged 5 years, who presented a previously unreported, distinct clinical phenotype consisting of primary microcephaly (head circumference = 40 cm, -5.6 standard deviations), brain anomalies including hypoplasia of the corpus callosum (with a residual draft of the genu), simplified parieto-temporal gyral pattern, colpocephaly with ectasia of the temporal ventricular horns, intellectual disability, and a general pattern of reduced growth (with weight and height < 3rd centiles). No classical features of TSC were recorded; the girl harbored a novel missense variant in TSC1 (c.611G > A). We hypothesize that her clinical phenotype could be related to a "gain-of-function" of the TSC1 protein product hamartin, causing an increase in the effects of the protein on inhibition of its intracellular targets (i.e., mTORC or RAC1 pathways), resulting in a distinct "inverse TSC1-hamartin" phenotype characterized by reduced growth of cells instead of the more classical predisposition to increased cell growth.

Congenital and acquired anomalies of the basilar artery: A pictorial essay.

INTRODUCTION: The basilar artery is one of the two cases in our body where an arterial vessel is formed by the union of two others - the vertebral arteries. It provides vascular supply to essential structures for the main vital functions; the posterior cerebral arteries originate from it as terminal branches, and form part of the anastomotic circle of Willis. IMAGING FINDINGS: Congenital and acquired anomalies of the basilar trunk are described. We provide a schematic and detailed representation of normal anatomical variants - mainly represented by the fenestrated basilar artery or the persistence of carotid-basilar anastomosis; course anomalies are also illustrated, with reference to neuro-vascular conflicts and dolichoectasia. Among congenital anomalies, this pictorial review also shows the variants of the basilar origin, such as in the case of basilar trunk arising from only one of the two vertebral arteries, and the calibre changes - which are represented by aneurysm and hypoplasia. The latter appears to be a risk factor for posterior circulation stroke, when associated with a bilateral posterior foetal variant.Among the acquired forms, this pictorial essay describes some clinical cases of dissections, non-congenital aneurysms, thrombosis and tumour with vascular encasing or compression of basilar artery. CONCLUSION: CT angiography and MRI allow us to study the posterior intracranial circulation in detail, providing useful pre-treatment information. Therefore, knowledge of congenital or acquired anomalies of the basilar artery is essential for radiologists, neuroradiologists and neurosurgeons.

An overview of the irreversible electroporation for the treatment of liver metastases: When to use it.

Tumour ablation is an established therapy for local treatment of liver metastases and hepatocellular carcinoma. Most commonly two different kind of thermic ablation, radiofrequency ablation and microwave ablation, are used in clinical practice. The aim of both is to induce thermic damage to the malignant cells in order to obtain coagulative necrosis of the neoplastic lesions. Our main concerns about these procedures are the collateral thermic damage to adjacent structures and heat-sink effect. Irreversible electroporation (IRE) is a recently developed, non-thermal ablation procedure which works applying short pulses of direct current that generate an electric field in the lesion area. The electric field increase the transmembrane potential, changing its permeability to ions.Irreversible electroporation does not generate heat, giving the chance to avoid the heat-sink effect and opening the path to a better treatment of all the lesions located in close proximity to big vessels and bile ducts. Electric fields produced by the IRE may affect endothelial cells and cholangiocytes but they spare the collagen matrix, preserving re-epithelization process as well as the function of the damaged structures. Purpose of the authors is to identify the different scenarios where CT-guided percutaneous IRE of the liver should be preferred to other ablative techniques and why.

Case report: Incidence and prognostic value of brain MRI lesions and elevated cerebrospinal fluid protein in children with Guillain-Barré syndrome.

Background: Guillain-Barrè syndrome (GBS) is an acute immune-mediated disorder affecting peripheral nerves and nerve roots with a variable clinical course and outcome. Epidemiologic analyses have revealed that the incidence of the syndrome increases linearly among the age. The clinical diagnosis of GBS is based on the family history, physical and neurological examination, electrodiagnostic exams, and cerebrospinal fluid analysis with the classical presence of albumin-cytologic dissociation. Prognosis is associated with the severity of clinical signs and the type of peripheral nerves involved. Methods: This study aims to clarify which clinical features can be used for prognostic purposes. We evaluated the correlation between (1) brain MRI lesions and grade of disability; (2) brain MRI lesions and elevated cerebrospinal fluid (CSF) protein; and (3) increased levels of CSF protein and grade of disability. Statistical analysis extracted from these data indicated a good correlation to be a prognostic indicator in children affected by GBS. We found little evidence regarding laboratory tests, imaging, and prognosis. We enrolled 12 continuous patients who met the Brighton criteria for GBS in this retrospective study. Each patient was clinically evaluated at the time of disease onset to assess the GBS disability score and after 2 weeks. Results: We estimated Pearson's correlation index to evaluate the possible correlation between MRI and disability and CSF protein levels and disability. The correlation coefficient was 0.92 and 0.85, respectively. In addition, we developed a graph to see the trend of the disability values, proteins in the CSF, and damage assessed with MRI in the 12 patients. It seems that these parameters have a parallel trend and a good correlation in each patient. Finally, we calculated the correlation between MRI and CSF protein values, with an r-value of 0.87. The values suggest a correlation among the MRI score, CSF protein, and prognosis. Conclusion: The MRI and CSF laboratory parameters can be important tools for the clinician not only for diagnosis but also to evaluate the possible worsening of general conditions or the need to prepare measures to support life parameters. Patients who need ventilatory support could be established early from patients who have less severe GBS and can begin rehabilitation earlier. We suggest MRI should be performed routinely in children with GBS to be able to estimate the evolution of the clinical condition.

Report of Two Cases of Pediatric IgG4-Related Lymphadenopathy (IgG4-LAD): IgG4-Related Disease (IgG4-RD) or a Distinct Clinical Pathological Entity?

IgG4-related disease (IgG4-RD) is a recently discovered immune-mediated fibroinflammatory condition, uncommon in the pediatric population, that could involve multiple organs and induce cancer-like lesions and organ damage. Its main features are multiple injuries in different sites, a dense lymphoplasmacytic infiltrate rich in IgG4 plasma cells, storiform fibrosis, and often high serological concentrations of IgG4. Autoimmune pancreatitis is the most common manifestation, mainly in adults. Two cases of IgG4-RD in children with lymph node localization of disease are reported. Localized or systemic lymph node involvement is common, but lymph node enlargement as the first and only manifestation of IgG4-RD is unusual, and therefore, hard to differentiate from other diseases. IgG4-related lymphadenopathy (IgG4-LAD) is most likely a distinct disease, described as isolated lymphadenopathy, related to the presence of elevated numbers of IgG4-positive plasma cells. Both disorders are likely to be misdiagnosed in children because they are characterized by rare and polymorphic features. IgG4-RD and IgG4-LAD should be considered in the differential diagnosis of disorders characterized by lymphadenopathy of uncertain etiology.

Is the distance between mammillary bodies predictive of a thickened third ventricle floor?

Object The aim of this study was to correlate intraoperative endoscopic third ventriculostomy (ETV) findings in hydrocephalic patients with the MR imaging appearance of the mammillary bodies (MBs), the fundamental anatomical landmarks of the third ventricle floor (TVF) region. Methods The authors reviewed brain MR images and intraoperative ETV records in 23 patients with hydrocephalus as well as MR imaging data from 120 randomized control volunteers of various ages to define the normal intermammillary distance (IMD). Results In control volunteers, no measurable IMD ("kissing" configuration) was observed in 91 (85%) of 107 cases, and there was mild MB splitting (mean +/- standard deviation, 0.18 +/- 0.12 cm) in only 16 cases with age-related cerebral atrophy. Among the 21 patients with complete MR imaging and ETV data sets, 12 ETV procedures were hindered by anatomical anomalies such as a thickened TVF or an "upward ballooning" phenomenon. On preoperative MR imaging in these 12 patients, there was an increased IMD (0.55 +/- 0.41 cm) compared with that in the remaining 9 patients (0.27 +/- 0.25 cm) who had a normal thin TVF during ETV and in the control group (0.03 +/- 0.08 cm). Magnetic resonance imaging and ETV data concordantly displayed nonsplit MBs in 6 of 9 cases with a thin TVF and split MBs in 10 of 12 cases with a thick TVF. Conclusions The normal configuration of MBs is no measurable IMD, with mild splitting occurring in patients with age-related brain atrophy. In hydrocephalic patients, a thickened TVF was present almost exclusively with an increased IMD on preoperative MR imaging and separated MBs on endoscopic viewing. Large retrospective series are needed to confirm that a preoperative increased IMD is predictive of a thickened TVF during ETV.

CT-guided radiofrequency ablation in the treatment of recurrent rectal cancer.

OBJECTIVE: Locally recurrent rectal adenocarcinoma remains a therapeutic challenge that is unsatisfactorily managed by surgery and radiation therapy or chemotherapy. Palliative CT-guided radiofrequency ablation was used in 14 patients with recurrent rectal adenocarcinoma who had been previously treated with abdominoperineal resection and radiation therapy. Follow-up CT or MRI was performed at 3, 6, 12, and 24 months. Pain palliation was monitored by the brief pain inventory (BPI). CONCLUSION: One month after radiofrequency ablation, 11 patients reported satisfactory BPI mean scores reduction compared to baseline (from 7.6 to 3.4 and from 5.1 to 1.6 for worst and average pain, respectively). In two unresponsive patients, retreatment was successfully performed at 3 months. After 24 months, worst and average pain scores further decreased (to 2.6 and 0.8, respectively) in 10 patients, who, at imaging, showed an ablation zone covering the entire original lesion in two patients and incomplete ablation in eight. In our experience, radiofrequency ablation is a safe and effective palliative treatment for patients with recurrent rectal adenocarcinoma.

Mandatory Reporting of Coronary Artery Calcifications Incidentally Noted on Chest Multi-Detector Computed Tomography: A Multicentre Experience.

BACKGROUND: Coronary Artery Calcifications (CACs) are associated with coronary atherosclerosis and Cardiovascular (CV) events. In "non-cardiovascular" settings, CACs can be easily detected on chest Multi-Detector Computed Tomography (MDCT). Their evaluation may help to better stratify CV risk in the general population, especially for primary prevention. AIMS: We retrospectively evaluated the relationship between CAC distribution and CV risk, determined by Framingham Risk Score (FRS), in a cohort of patients who underwent chest MDCT performed for several clinical indications. METHOD: We retrospectively recruited 305 patients (194 men, 111 women; mean age 70.5 years) from 3 different Italian centres. Patients with coronary stent, pacemaker and/or CV devices were excluded from the study. Circumflex Artery (LCX), Left Main Coronary Artery (LMCA), left Anterior Descending artery (LAD) and right coronary artery (RCA) were analysed. RESULTS: From a total population of 305 patients, 119 (39%) had low FRS (<10%), 115 (38%) had intermediate FRS (10-20%), and 71 (23%) had high FRS (>20%). The study identified 842 CACs located in decreasing order as follows: RCA (34.5%), LAD (32.3%), LCX (28%) and LMCA (13%). Statistical two-step analysis subdivided patients into two clusters according to FRS (risk threshold = 12.38%): cluster I (mean 9.34) and cluster II (mean 15.09). A significant association between CAC distribution and cluster II was demonstrated. CACs were mostly detected in patients with intermediate FRS. All patients (100%) with the highest CV risk showed intermediate RCA and LMCA involvement. CONCLUSION: Radiologists can note the distribution of CACs on a chest MDCT and should mandatorily record them in their reports. Depending on CAC presence and location, these findings may have important clinical implications, mostly in asymptomatic patients with intermediate FRS. This information may reclassify a patients' CV risk and improve clinical management.

Concurrent chemotherapy alone versus irreversible electroporation followed by chemotherapy on survival in patients with locally advanced pancreatic cancer.

Pancreatic adenocarcinoma is one of the most fatal cancers, characterized by aggressive tumor growth and a short patient survival time between diagnosis and death. Safe and effective treatment options are limited, especially in cases when surgical resection is not possible. Irreversible electroporation (IRE) is a non-thermal ablation technique recently introduced in the treatment of pancreatic cancer. From 2013 to 2016, 29 cases of locally advanced pancreatic cancer (LAPC) treated with IRE were retrospectively analyzed and the median overall survival (OS) rates were compared with patients with the same diagnosis who received standard chemotherapy as reported in the literature. Literature was selected according to a predetermined protocol. Secondarily, preoperative and postoperative Karnofsky scores of the 29 IRE-treated patients were compared to determine improvement in quality-of-life. Median OS of IRE-treated patients was 14 months (SE 11 months, 95% CI range 9.86-18.14). For IRE-treated patients, the Karnofsky score increased from Tzero to T3m by a mean of 28.28 (SE 2.11, 95% CI range 23.95-32.60). In 27 patients, 6-month imaging follow-up showed a mean lesion volumetric decrease percentage of 40.32% (SE 2.76, 95% CI 34.63-46.01%). Treatment with IRE followed by chemotherapy substantially increases median OS rate and quality-of-life of LAPC-diagnosed patients when compared to patients treated with traditional methods, including chemotherapy. Further investigation of this multi-modal treatment is warranted.