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A 46-year-old man who had been diagnosed with eosinophilic otitis media (EOM) and eosinophilic chronic rhinosinusitis was referred to our department. He suffered from bilateral earache, clogged ear sensation, and otorrhea associated with EOM. He had been treated with a myringotomy and a ventilation tube (VT) insertion. However, his symptoms did not improve, and the VT repeatedly fell out. We performed canal wall down mastoidectomy via a retro-auricular incision to remove the presumed cholesterol granuloma (CG) and a long-term VT insertion. The VT fell out repeatedly. Therefore, a large VT that Komune devised was inserted. Four months after reinsertion, there was no evidence of recurrent otorrhea or fallout of a large VT. A large VT insertion could be useful in the severe case of EOM with CG. [ABSTRACT FROM AUTHOR]

Eosinophilia is defined as a condition with increased eosinophil cell counts in blood more than the standard value. In this regard, when extensive evaluation fails to reveal the underlying causes of this disease, hypereosinophilic syndrome (HES) diagnosis should be considered. Moreover, it is possible that the mutation of the tyrosine kinase genes, as the most common type of cryptic mutations, is associated with HES syndrome. We report a case of a 47-year-old man who was initially diagnosed with HES by Microscopic examination of bone marrow aspiration and peripheral blood cell analysis. After diagnosis, the patient was administered with cortisone acetate, leading to an initial remission. One month after the initial remission, the disease relapsed, and the patient eventually died. This case report provides the first report of HES, in which a novel variant of partial tandem duplication (PTD) was detected in the KMT2A gene. [ABSTRACT FROM AUTHOR]

Benign tumors that grow in the spinal canal are heterogeneous neoplasms with low incidence; from these, meningiomas and nerve sheath tumors (neurofibromas and schwannomas) account for 60%–70% of all primary spinal tumors. Benign spinal canal tumors provoke nonspecific clinical manifestations, mostly related to the affected level of the spinal cord. These tumors present a challenge for the patient and healthcare professionals, for they are often difficult to diagnose and the high frequency of posttreatment complications. In this review, we describe the epidemiology, risk factors, clinical features, diagnosis, histopathology, molecular biology, and treatment of extramedullary benign meningiomas, osteoid osteomas, osteoblastomas, aneurysmal bone cysts, osteochondromas, neurofibromas, giant cell tumors of the bone, eosinophilic granulomas, hemangiomas, lipomas, and schwannomas located in the spine, as well as possible future targets that could lead to an improvement in their management. [ABSTRACT FROM AUTHOR]

Eosinophilic myocarditis is a human condition that has been rarely documented in animals. We now report two unrelated porcine cases of idiopathic eosinophilic granulomatous myocarditis that resembled the human disease and which were associated with sudden death. The most relevant gross finding in both cases was marked cardiomegaly, accompanied by raised, multifocal to coalescent small white nodules (1–2 mm) and poorly demarcated multifocal pale areas in the epicardium. Histologically, there were multifocal to coalescent areas of cardiomyocyte loss with replacement by an intense inflammatory infiltrate of eosinophils and epithelioid macrophages, and proliferation of fibrous connective tissue. Immunohistochemistry for porcine circovirus type 2 (PCV2) and Toxoplasma gondii , in-situ hybridization and quantitative polymerase chain reaction tests for PCV2 and porcine circovirus type 3 and aerobic bacterial culture on myocardium samples were negative. [Display omitted] [ABSTRACT FROM AUTHOR]

Background: In many patients, low-dose computed tomography (CT) screening for lung cancer reveals asymptomatic pulmonary nodules. Lung resection surgery may be indicated in these patients; however, distinguishing malignancies from benign lesions preoperatively can be challenging.Methods: From 2013 to 2018, 4181 patients undergoing surgery for pulmonary nodules were reviewed at National Taiwan University Hospital, and 837 were diagnosed with benign pathologies. Only patients with pathological diagnosis as caseating granulomatous inflammation were included, sixty-nine patients were then analyzed for preoperative clinical and imaging characteristics, surgical methods and complications, pathogens, medical treatment and outcomes. Mycobacterial evidence was obtained from the culture of respiratory or surgical specimen.Results: Overall, 68% of the patients were asymptomatic before surgery. More than half of the nodules were in the upper lobes, and all patients underwent video-assisted thoracoscopic surgery (VATS). Some patients (14.5%) developed grade I complications, and the mean postoperative hospital stay was 4 days. The final pathology reports of 20% benign entities postoperatively, and caseating granulomatous inflammation accounted for a significant part. MTB and NTM were cultured from one-fourth of the patients respectively. All patients with confirmed MTB infection received antimycobacterial treatment, while the medical treatment in NTM-infected patients was decided by the infectious disease specialists. The mean follow-up period was 736 days, and no recurrence was found.Conclusion: Lung resection surgery is an aggressive but safe and feasible method for diagnosing MTB- or NTM-associated pulmonary nodules, and, potentially, an effective therapeutic tool for patients with undiagnosed MTB- or NTM-associated pulmonary nodules. [ABSTRACT FROM AUTHOR]

A middle meningeal artery (MMA) aneurysm is a type of intracranial aneurysm that has been rarely documented and is not completely understood in clinical medicine and especially in pediatrics, because juvenile cases have been extremely uncommon. Vascular conditions have occasionally been diagnosed in osteoarchaeological remains, providing evidence of their occurrence in previous eras. In the present report, we have described a lesion that was highly consistent with a case of an MMA aneurysm observed in an Etruscan child, whose remains had been exhumed during archaeological excavations in the necropolis of Pontecagnano (Salerno, southern Italy). The remains date back to the seventh to sixth century BC. Macroscopic and radiological examinations revealed an oval depression in the endocranial area corresponding to the parietal branch of the right MMA. The localized thinning of the bone and the absence of a bone reaction were compatible with a long-lasting compression of vascular origin highly consistent with an MMA aneurysm. Primary malignant bone tumors, bone metastasis, benign neoplastic conditions, and other non-neoplastic conditions, including calvarial venous malformations, eosinophilic granuloma, and sarcoidosis, could be excluded from the differential diagnosis, although the occurrence of other pathologies, such as dural arteriovenous fistulas, could not be rejected. Although it was not possible to definitely establish whether the MMA aneurysm was symptomatic, it could not be ruled out that rupture and subsequent extradural hemorrhage and/or intracranial hemorrhage could have caused the death of the child. From a paleopathological viewpoint, the case illustrated provides an interesting historical perspective on a rare neurovascular disorder that continues to be debated in modern clinical research. [ABSTRACT FROM AUTHOR]

Although few case reports of human fascioliasis have been reported from different parts of India, there is no case reported from the Kashmir valley to date. Herein we report two cases of human fascioliasis. Both patients presented with fever, marked eosinophilia, and liver lesions on imaging. Hepatobiliary imaging showed vague features like mild biliary dilatation and liver lesions representing burrows. A liver biopsy in one of the patients revealed eosinophilic granuloma. Both patients were diagnosed definitively with endoscopic retrograde cholangiopancreatography (ERCP) by demonstrating live adult fasciola worms. Any patient presenting with fever, marked eosinophilia, and liver lesions on imaging should be evaluated for fascioliasis. [ABSTRACT FROM AUTHOR]

To determine the differential diagnosis of dermal and sub-dermal soft tissue masses which do not extend to the deep fascia that are referred to a specialist musculoskeletal oncology service. Review of all patients referred to a specialist musculoskeletal oncology service over a 13-month period with a dermal or sub-dermal soft tissue mass which did not extend to the deep fascia based on MRI appearances. Data collected included age, gender, site and maximal lesion size, and final histological diagnosis for biopsied and/or excised lesions. 56 patients were included, 21 (37.5%) males and 35 (62.5%) females with mean age 49.75 years (range 5–86 years). 3 patients had 2 lesions making a total of 59 lesions, 11 (18.6%) dermal and 48 (81.4%) sub-dermal. Histological diagnosis was available in 44 (74.6%) cases, of which 11 (25%) were non-neoplastic, 23 (52.3%) were benign neoplasms, and 10 (22.7%) were malignant neoplasms. Although older age at presentation and greater mean maximal tumour dimension were seen with malignant lesions, these did not reach statistical significance (p = 0.154 and 0.102 respectively). There was also no relationship between skin involvement from sub-dermal lesions and malignancy, but 5 of 6 dermal lesions which showed an exophytic growth pattern were malignant. The differential diagnosis of dermal and sub-dermal lesions which do not reach the deep fascia is wide with a malignant lesion being the cause in almost one-quarter of cases. • The differential diagnosis of dermal and sub-dermal lesions that do not reach the deep fascia is wide. • Almost a quarter of cases represent malignant lesions. • Almost a quarter of cases represent non-neoplastic lesions. • Older age at presentation and greater mean maximal tumour dimensions were present more frequently with malignant lesions. [ABSTRACT FROM AUTHOR]

A histiocytosis is a group of immunoproliferative disorders of clonal cells. The management protocols are still evolving, with chemotherapy as the mainstay of treatment. This study aims to evaluate the feasibility, safety, efficacy, and complication profile of stereotactic radiosurgery for intracranial histiocytosis. The authors reviewed PubMed, Scopus, Web of Science, and Embase for "radiosurgery" and "histiocytosis" in the English/Japanese language following preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines. The patient profile, radiosurgical parameters (dose and isodose), target volume, and mode of radiosurgery (Gamma knife, LINAC radiosurgery, etc.) were collected. Its use as primary or adjuvant therapy, clinical and radiological outcome was also evaluated. We identified 7 studies (9 patients); mean age: 41.9 years (24–57 years). Six patients received Gamma-knife radiosurgery, whereas 3 received CyberKnife radiosurgery. The Langerhans cell histiocytosis variants were eosinophilic granuloma in 3, whereas 4 were not defined. Two cases had Rosai-Dorfman disease, and 2 different yet pathogenetically related histiocytic disorders. Four patients harbored lesions in the pituitary stalk and posterior pituitary, 2 patients in the petrous region, 1 patient had a pontine lesion, and 2 patients had multiple lesions. The dose delivered ranged from 8 to 28 Gy. A total of 18 lesions (9 patients) were followed for 81.67 patient-years: 7 (39%) disappeared, 8 (44.4%) showed radiological reduction, and 2 (11%) remained stable. One lesion (5%) showed an increase in size needed surgical excision. There were no adverse effects. The role of stereotactic radiosurgery needs to be further evaluated as the current cohort with only 9 cases (2 are Rosai-Dorfman disease) is insufficient to make conclusions. It may be a viable alternative in localized disease, along with chemotherapy and targeted surgery. [ABSTRACT FROM AUTHOR]

Previous reports on skeletal pathology in captive snakes did not clarify the relationship of lesions to captive or free-ranging status. In this study, post-cranial skeletons from 7,950 captive and free-ranging snakes in nine of the major North American collections were examined macroscopically, microscopically and radiologically for the presence of pathology. Micro-computerized tomography was performed on two skeletons on which surface microscopy failed to provide a definitive diagnosis. Osteomyelitis was identified in the vertebrae of 24 snakes and spondyloarthropathy in one snake. Neoplasia or congenital anomalies were not found. Osteomyelitis was four times as common in Viperidae than in Colubridae and twice as common as in Boidae, independent of captive or free-ranging status. A lytic lesion in the vertebrae of one snake was suggestive of fungal disease. The progression of bone lesions was greater in captive snakes, but the prevalence was independent of captive or free-ranging status. The results of this study indicate that bone alterations, previously proposed as neoplastic disease, in archival snake skeletons were most likely of infectious aetiology, and establish macroscopic examination as a useful technique for characterizing skeletal pathology in snakes. [ABSTRACT FROM AUTHOR]

• MicroRNA miR-155-5p, a non-protein-coding and endogenous small RNA, is highly overexpressed in Angiostrongylus cantonensis infection. • MiR-155-5p knockdown may attenuate A. cantonensis -induced eosinophilic meningitis (EM). • MiR-155-5p targets SOCS1 and upregulates MMP9 and TSLP expression through activating STAT3 and NF-κB signal. • A positive feedback loop is formed between miR-155-5p, STAT3, and NF-κB in A. cantonensis infection. Angiostrongylus cantonensis infection is a major cause of eosinophilic meningitis (EM). Severe cases or cases that involve infants and children present poor prognoses. MicroRNAs (miRNAs), which are important regulators of gene expression in many biological processes, were recently found to be regulators of the host response to infection by parasites; however, their roles in brain inflammation caused by A. cantonensis are still unclear. The current study confirmed that miR-155-5p peaked at 21 days after A. cantonensis infection, and its expression was positively correlated with the concentration of excretory and secretory products (ESPs). We found that miR-155-5p knockdown lentivirus successfully ameliorated brain injury and downregulated the expression of major basic protein (MBP) in vivo, and the number of eosinophils in CSF (and the percentage of eosinophils in peripheral blood were also decreased in the miR-155-5p knockdown group. Moreover, the expression of several eosinophilic inflammation cytokines such as CCL6/C10, ICAM-1, and MMP9, declined after the miR-155-5p knockdown. SOCS1 protein, which is an important negative regulator of inflammation activation, was identified as a direct miR-155-5p target. We further detected the effect of miR-155-5p knockdown on phosphorylated-STAT3 and phosphorylated-p65 proteins, which were found to be negatively regulated by SOCS1 and play an important role in regulating the inflammatory response. We found that miR-155-5p knockdown decreased the activity of p-STAT3 and p-p65, thereby leading to lower expression of MMP9 and TSLP proteins, which were closely related to the chemotaxis and infiltration of eosinophils. Interestingly, the inhibition of p-STAT3 or p-p65 was found to induce the downregulation of miR-155-5p in an opposite manner. These observations suggest that a positive feedback loop was formed between miR-155-5p, STAT3, and NF-κB in A. cantonensis infection and that miR-155-5p inhibition might provide a novel strategy to attenuate eosinophilic meningitis. [ABSTRACT FROM AUTHOR]

The aim of this study was to evaluate the diagnostic accuracy of navigation-guided core needle biopsy for skull base and parapharyngeal lesions. Twenty patients with skull base and parapharyngeal lesions were included in this study. The preoperative design and intraoperative real-time image guiding was done using an optical navigation system. A spring-loaded semi-automatic biopsy gun and biopsy needle were used for specimen harvesting. Accuracy was established on the basis of the postoperative pathology. All patients underwent needle biopsy successfully without any immediate or delayed complications. The subzygomatic approach was adopted in all cases. The number of passes ranged from three to five. The diagnostic accuracy was 90% (18/20). Navigation-guided core needle biopsy offers an easy approach for the diagnosis of skull base and parapharyngeal lesions, with a high yield of specimens and good patient tolerance. [ABSTRACT FROM AUTHOR]

Therapeutic allogeneic mesenchymal stromal cells (MSCs) are currently in clinical trials to evaluate their effectiveness in treating many different disease indications. Eventual commercialization for broad distribution will require further improvements in manufacturing processes to economically manufacture MSCs at scales sufficient to satisfy projected demands. A key contributor to the present high cost of goods sold for MSC manufacturing is the need to create master cell banks from multiple donors, which leads to variability in large-scale manufacturing runs. Therefore, the availability of large single donor depots of primary MSCs would greatly benefit the cell therapy market by reducing costs associated with manufacturing. We have discovered that an abundant population of cells possessing all the hallmarks of MSCs is tightly associated with the vertebral body (VB) bone matrix and only liberated by proteolytic digestion. Here we demonstrate that these vertebral bone-adherent (vBA) MSCs possess all the International Society of Cell and Gene Therapy-defined characteristics (e.g., plastic adherence, surface marker expression and trilineage differentiation) of MSCs, and we have therefore termed them vBA-MSCs to distinguish this population from loosely associated MSCs recovered through aspiration or rinsing of the bone marrow compartment. Pilot banking and expansion were performed with vBA-MSCs obtained from 3 deceased donors, and it was demonstrated that bank sizes averaging 2.9 × 108 ± 1.35 × 108 vBA-MSCs at passage 1 were obtainable from only 5 g of digested VB bone fragments. Each bank of cells demonstrated robust proliferation through a total of 9 passages, without significant reduction in population doubling times. The theoretical total cell yield from the entire amount of bone fragments (approximately 300 g) from each donor with limited expansion through 4 passages is 100 trillion (1 × 1014) vBA-MSCs, equating to over 105 doses at 10 × 106 cells/kg for an average 70-kg recipient. Thus, we have established a novel and plentiful source of MSCs that will benefit the cell therapy market by overcoming manufacturing and regulatory inefficiencies due to donor-to-donor variability. [ABSTRACT FROM AUTHOR]

• Aporocotylid Cardicola laruei infects the heart of wild sciaenid Cynoscion nebulosus. • Prevalence of infection by adult flukes (61%) and granulomas (84%) peak in summer. • Female seatrout are infected by more flukes than males. • Seatrout ventricle is damaged by epithelialized granulomas and blister-like extrusions through the epicardium. • Granulomas may be used to translocate live Cardicola eggs as occurs in schistosomes. The sciaenid Spotted Seatrout (Cynoscion nebulosus) are infected by blood flukes (Cardicola spp.). A 2 year survey in estuaries of South Carolina, USA, showed that adult flukes and granulomas occurred throughout the year but their prevalence was highest in summer (61% and 84%, respectively), indicating an unusually high level of infection for wild fish. Granulomas remained after adult flukes could no longer be found. PCR-Restriction Fragment Length Polymorphism (RFLP) of a subsample of specimens allowed identification of Cardicola laruei as the only species infecting these seatrout during the period of study. Mean intensity of infection by flukes was higher in female seatrout, suggesting endocrine and/or immune system involvement. The prevalence of granulomas declined sharply in winter, indicating possible mortality of infected seatrout as this species is known to be cold-sensitive. Granulomas were studied using histology, immunohistochemistry, and transmission electron microscopy. Eggs were encapsulated by an inner core of dark epithelioid cells, and an outer core of large epithelioid cells undergoing epithelialization. Fibrosis was observed around granulomas and some granulomas detached from the surrounding damaged myocardium. Numerous inflammatory cells appeared mobilised around granulomas and pathology could be severe, in some cases showing grossly visible blister-like extrusions scattered in the damaged epicardium. At the gross level, some granulomas possessing eggs with live miracidia were observed at the surface of the epicardium. These findings suggest that granulomas carrying both dead and live eggs can clear the fish heart by host-mediated transport through the myocardium, as is known to occur in related human Schistosoma infections. [ABSTRACT FROM AUTHOR]

Tuberculosis is a common disease; however, the prevalence of calvarial tuberculosis is very rare. Most cases of calvarial tuberculosis occur in young patients. We report a rare case of calvarial tuberculosis in an elderly patient. An 89-year-old woman presented with a forehead skin defect. Radiologic imaging showed bony erosion 20 × 10 mm in size with adjacent dural enhancement in the left frontal bone. The patient underwent surgical treatment. Pathology revealed chronic granulomatous inflammation with caseous necrosis, consistent with tuberculosis. Antituberculous medications were prescribed for 6 months. A careful assessment should be performed to obtain an appropriate diagnosis in cases of osteolytic lesions of the skull. [ABSTRACT FROM AUTHOR]

We document the causes and pathological findings in 50 cases of equine pneumonia and pleuritis in Southern Brazil. Suppurative (17/50), pyogranulomatous (14/50), aspiration (5/50), mycotic (4/50), bronchointerstitial (3/50), embolic (3/50) and eosinophilic granulomatous pneumonia (1/50) and pleuritis (3/50) were the main conditions identified. Streptococcus spp. were identified in 11 cases of suppurative pneumonia. Suppurative pneumonia was further divided into acute (8/17), subacute (6/17) and chronic (3/17) based on the morphological pattern of lesions. Rhodococcus equi was identified in all cases of pyogranulomatous pneumonia and exclusively affected young foals. [ABSTRACT FROM AUTHOR]

Intracranial bronchogenic cysts (BCs) are extremely rare. To our knowledge, this is the first report of a BC in which lesions involve the middle and posterior cranial fossa, as well as the infratemporal fossa. We present the case of a 38-year-old woman who suffered from a cranial nerve dysfunction for 2 years. Magnetic resonance imaging showed that there were skull base communication lesions across the middle and posterior fossa. The patient was operated on through an infratemporal fossa approach. The final diagnosis was BC after histopathologic examination and immunohistochemical verification. The patient's neurologic dysfunction was partially ameliorated at the half-year follow-up. Intracranial BCs are rare. However, they should be considered in the differential diagnosis for cystic lesions with edge enhancement or extracranial extension. [ABSTRACT FROM AUTHOR]

Sarcoidosis is a granulomatous disease most often affecting the lungs, but extrapulmonary manifestations are also common. While virtually any organ system can be involved, skeletal manifestations are present in 1%−13% of cases. Skull lesions are even more rare. We found 14 case reports describing symptomatic skull lesions in patients with no prior history of sarcoid to better understand the symptomology and disease progression. Here we present the case of a 57-year-old female with history of sarcoidosis and new-onset forgetfulness, confusion, and headaches who was found to have multiple skull lesions with epidural extension. On histopathologic examination of the excised lesion, noncaseating granulomas were observed and diagnoses of skull sarcoidosis and neurosarcoid were made. Since the patient was actively treated with corticosteroids, the plan was to initiate infliximab. Sarcoidosis affecting the skull and central nervous system is exceedingly rare and can mimic many pathologies including metastatic bone disease, multiple myeloma, and eosinophilic granulomatosis. With a wide differential, surgical specimen is needed for concrete diagnosis and treatment. While the rates of skeletal involvement are low, a skeletal survey might be an important step in monitoring disease burden in patients, especially as lesions can be asymptomatic. [ABSTRACT FROM AUTHOR]

Proliferative disorders of lymphatic origin in animals are mostly congenital or occur within the first few months of life. Involvement of internal organs is extremely infrequent. A seven-year-old entire female mixed-breed dog was presented with apathy and poor appetite. Abdominal ultrasonography revealed a focally enlarged spleen with an anechoic round lesion. Splenectomy was performed and pathological examination demonstrated a sponge-like, compressible tumour composed of endothelium-lined vascular cystic spaces filled with eosinophilic proteinaceous material lacking erythrocytes. Immunohistochemical stains showed that cyst-lining cells were strongly positive for CD31 and factor VIII and focally positive for lymphatic vessel endothelial hyaluronan receptor 1 (LYVE-1). Based on these findings, the lesion was identified as splenic cystic lymphangioma. To the best of our knowledge, this is the first description of solitary lymphangioma of the spleen in animals. Image 1 [ABSTRACT FROM AUTHOR]

Study Objectives: To describe sleep manifestations, polysomnographic (PSG) findings, and specific sleep disorders in children with Eosinophilic Esophagitis (EoE).Methods: This retrospective study included children with EoE who were referred to sleep clinics. Clinical manifestations, PSG variables, and diagnosis of sleep disorders were analyzed. Sleep architecture of patients with EoE was compared to control subjects.Results: In sum, 81 children with EoE met the criteria for entry into the analysis with a mean age of 10.1 ± 4.4 years. Of those, 46 children (57%) presented in the sleep clinic with active EoE symptoms, while 35 (43%) children did not have active EoE symptoms at presentation. Several sleep complaints were common in children with EoE, including snoring (62, 76.5%), restless sleep (54, 66.6%), legs jerking or leg discomfort (35, 43.2%) and daytime sleepiness (47, 58.0%). Comparing sleep architecture with controls, children with EoE had significantly higher NREM2 (P= < 0.001), lower NREM3 (P= < 0.001), lower rapid eye movement (REM) (P = 0.017), increased periodic leg movements (PLM) index (P= < 0.001) and increased arousal index (P = 0.007). There were no significant differences in the sleep efficiency between the EoE and control subjects. Common sleep diagnoses included obstructive sleep apnea (OSA, 30, 37.0%) and periodic limb movements disorder (PLMD, 20, 24.6%). Of note, we found a much higher percentage of PLMD in active EoE compared to inactive EoE (P = 0.004).Conclusions: Children with EoE have frequent sleep complaints and several sleep disorders identified from the sleep study, including sleep-disordered breathing and PLMD. Analysis of sleep architecture demonstrates significant sleep fragmentation as evidenced by decreased slow-wave sleep and REM sleep and increased arousal index. [ABSTRACT FROM AUTHOR]

Rosai-Dorfman disease (RDD) is a rare, benign histiocytosis disorder with only approximately 100 reported central nervous system cases in the literature. Even less common is skull base involvement of RDD, with about 41 reported cases. Radiographically, RDD can appear similar to a meningioma; the true diagnosis is only found with histologic analysis. Although "benign," RDD can lead to significant neurologic morbidity from the disease or unnecessary surgical and nonsurgical treatment. While rare, intracranial RDD has been treated with chemotherapy or radiotherapy, with surgery playing a limited role to relieve mass effect. Surgical approaches to the skull base are invasive and pose risk, especially for a benign and self-limiting disease like RDD. Here we present the case of a 63-year-old woman with a presumed spheno-orbital meningioma for which the patient previously underwent radiation therapy. On presentation to our facility, the patient noted pressure headaches and blurry vision, and imaging demonstrated progression of her disease. For these reasons, surgical debulking and biopsy were undertaken. This report demonstrates the role of EEA for tissue biopsy and decompression of the affected cranial nerves and orbit. This paradigm seems to provide a safe and effective way to manage patients with compressive symptoms, while also allowing for tissue sampling. [ABSTRACT FROM AUTHOR]

L'histiocytose langerhansienne (HL) est une maladie rare pouvant toucher tous les tissus. Les premières manifestations de l'HL sont souvent orales (ulcérations buccales, mobilités dentaires). Nous rapportons un cas rare d'HL unifocale localisée au condyle mandibulaire et mimant un désordre de l'appareil manducateur. Une femme de 44 ans présentait un tableau de désordre temporo-mandibulaire gauche associé à un œdème pré-auriculaire gauche douloureux. Le bilan d'imagerie retrouvait une lésion ostéolytique du condyle mandibulaire gauche. Un curetage avec biopsie a permis de faire le diagnostic d'HL. Six mois plus tard, la patiente n'avait plus de douleur. Les atteintes crâniofaciales de l'HL concernent majoritairement les os, pouvant provoquer : douleur, œdème, fracture, compressions d'organes nobles. Les autres atteintes sont buccales, cutanée, ganglionnaire ou oculaire. Les formes isolées sont généralement bénignes, et leur traitement se discute entre abstention et chirurgie non agressive. Langerhans cell histiocytosis (HL) is a rare disease that can affect all tissues. Oral manifestations such as mucosal ulcer and tooth mobility are often the first signs of the disease. We report a rare case of mandibular condyle unifocal HL mimicking a temporomandibular joint disorder. A 44-year-old patient presented with a left temporomandibular disorder with painful left preauricular swelling. The imaging assessment found a bone lesion of the left mandibular condyle. A curettage with biopsy was used to diagnose HL. Six months later, the patient had no more pain. The craniofacial clinical expressions of HL mainly concern the bones, which can cause: pain, swelling, fracture, compression of noble organs. The other sites are: oral cavity, skin, lymph nodes, or eyes. Isolated forms are generally benign, and their treatment is discussed between abstention and non-aggressive surgery. [ABSTRACT FROM AUTHOR]

Spine stereotactic radiosurgery (SSRS) is a noninvasive treatment for metastatic spine lesions. MD Anderson Cancer Center reports a quality assurance (QA) failure rate approaching 15% for SSRS cases, which we hypothesized is due to difficulties in accurately calculating dose resulting from a large number of small-area segments. Clinical plans typically use 9 beams with an average of 10 segments per beam and minimum segment area of 2-3 cm2. The purpose of this study was to identify a set of intensity-modulated radiation therapy (IMRT) planning parameters that attempts to optimize the balance among QA passing rate, plan quality, dose calculation accuracy, and delivery time for SSRS plans. Using Pinnacle version 9.10, we evaluated the effects of 2 IMRT parameters: maximum number of segments and minimum segment area. Initial evaluation of the data revealed that 5 segments per beam along with minimum segment area of 4 cm2 and 4 monitor units (MU) per segment (5-4-4 plans) was the most promising. IMRT QA was performed using a PTW OCTAVIUS 4D phantom with a 2D detector array. Our data showed no significant plan quality change with decreased number of segments and increased minimum segment area. The average coverage of GTV and CTV was 82.5 ± 13% (clinical) vs 82.5 ± 13% (5-4-4) and 92.3 ± 8% (clinical) vs 91.5 ± 8% (5-4-4). Maximum point dose to cord was 11.4 ± 3.5 Gy (clinical) vs 11.0 ± 4.0 Gy (5-4-4). Total plan delivery time was decreased by an average of 11.3% for the 5-4-4 plans. For IMRT QA, the gamma index passing rate (distance to agreement: 2.5 mm, local dose difference: 4%) for the original plans vs the 5-4-4 plans averaged 90.3% and 91.9%, respectively. In conclusion, IMRT parameters of 5 segments per beam and 4 cm2 minimum segment areas provided a better balance of plan quality, delivery efficiency, and plan dose calculation accuracy for SSRS. [ABSTRACT FROM AUTHOR]

Angiomatoid fibrous histiocytoma (AFH) is a rare low-grade malignant tumor mainly occurring in soft tissues, and its incidence in the bones is extremely rare. Although most of the existing reports focus on the pathological features of AFH, only a few describe its imaging features. To our knowledge, this is the first case of AFH in the skull, and it is distinguished from eosinophilic granuloma based on imaging results. A boy aged 10 years presented with a painless mass of parietal bone after trauma. Cranial computed tomography angiography showed local bone defects near the sagittal suture of the left parietal bone and a soft tissue mass with relatively uniform density in the same area. The signals of this mass were heterogeneous in all sequences of magnetic resonance imaging and presented septal enhancement after the injection of contrast agent. The patient underwent complete resection of the mass, and the histopathological and immunohistochemical diagnosis of the mass was AFH. No complications occurred after the operation and no recurrence occurred during the follow-up. To our knowledge, this is the first AFH that occured in the skull, and the main imaging manifestations of AFH are bone destruction with soft tissue mass. The characteristic features of AFH are its fibrillar component that showed low signal on T2-weighted imaging and septal or peripheral enhancement, and no dead bone in the mass. [ABSTRACT FROM AUTHOR]

Tics and obsessions-compulsions are consistent phenotypes of the Obsessive-Compulsive Tic Disorder (OCTD), frequently associated with male gender, sensory phenomena, and impulsive behaviors. These clinical symptoms were reported to influence eating behaviors, but literature also showed that food patterns or dietary supplements could alleviate the clinical spectra, thus suggesting the existence of a bidirectional association. We present a series of 9 boys with OCTD from Italy whose unhealthy food habits were corrected through a nutritional counseling. The education focused on promoting a balanced diet through non-specific Mediterranean dietary advices. After one month, YGTSS (Yale Global Tic Severity Scale) and Y-BOCS (Yale-Brown Obsessive Compulsive Scale) scored a significant reduction, but the quality of life diminished. First, we conclude that healthier dietary patterns may be associated with an amelioration of tics and obsessive-compulsive traits in boys who are diagnosed with the same conditions. Second, the impact of nutritional interventions on the quality of patients' life, especially if underage, should always be considered. Certainly, the treatment of OCTD must be multidisciplinary and should include neuropsychiatrists, clinical psychologists, and nutritionists. The nutritional counseling should be as comprehensive as possible to promote a balanced diet, and inform about nutritional side effects of drugs or potential food-drug interactions. [ABSTRACT FROM AUTHOR]

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystem necrotizing vasculitis associated with eosinophilia and extravascular granuloma and classically involving the upper and lower airways. There have only been a few reported cases of gynecologic involvement in EGPA. We present an 8-year-old girl diagnosed with EGPA with a vulvar granuloma in what is, to our knowledge, the first reported pediatric gynecologic manifestation of EGPA. Interestingly, the vulvar granuloma did not respond to initial immunosuppressant treatment with prednisone and methotrexate and required treatment regimen modification with mycophenolate mofetil resulting in granuloma resolution. EGPA in the pediatric population has a relatively high mortality rate compared with in the adult population thus it is important that vulvar granulomas associated with EGPA should be included in the differential diagnosis of a vulvar mass allowing for the prompt diagnosis and treatment of this potentially fatal disease in children. [ABSTRACT FROM AUTHOR]

We describe the potent effect of myriadenolide (Myr), a naturally occurring labdane diterpene, in promoting the production of eosinophils in cultured bone-marrow from several inbred mouse strains. This enhancing effect is lineage-selective and requires the eosinophil growth factors, Interleukin(IL)-5 or GM-CSF. Myr acts over a very low concentration range (10−10–10−14 M), if added at the beginning of the cell cultivation. Its enhancing effect increases between 24 h and 10 days of culture. We used both pharmacological and genetical tools to analyze its mechanism of action. Several lines of evidence show that the enhancing effect of Myr requires functional integrity of the 5-lipoxygenase (5-LO) pathway, and of CysLT 1 receptors, which transduce the effects of cysteinyl-leukotrienes generated through this pathway. Myr also protects developing eosinophils from apoptosis induced by exogenous prostaglandin E2 (PGE 2), but not by NO, indicating that it acts upstream of NO in the PGE 2 -initiated proapoptotic pathway which requires iNOS and CD95. Exposure to NO concentrations insufficient to induce apoptosis abolished the ability of eosinophils to respond to Myr, suggesting the involvement of a NO-sensitive cellular target. Myr has potential as a chemically defined research tool, which can be used to generate large numbers of eosinophils, thereby overcoming current limitations in the biochemical and molecular biological study of murine eosinophils, which has so far depended on complex, labor-intensive and long-term culture protocols for in vitro expansion. Potent enhancing effects of Myr on eosinophil production in bone marrow stimulated by GM-CSF and IL-5 are mediated by the 5-LO pathway. Unlabelled Image • Myriadenolide has a potent pro-eosinophilic effect in murine bone-marrow cultures at very low concentrations. • Its effectiveness requires functional integrity of the 5-lipoxygenase pathway and of type I cysteinyl-leukotriene receptors. • Its effectiveness is lost after exposure of the cultures to NO-releasing chemicals. [ABSTRACT FROM AUTHOR]

Equine multinodular pulmonary fibrosis (EMPF) is a recently described form of interstitial pneumonia associated with equine herpesvirus type 5 (EHV-5). This disease has been reported in North and South America, Europe and Oceania but not, to our knowledge, in horses in Japan. We diagnosed EMPF in two Thoroughbred horses in Japan on the basis of gross and histopathological findings. In both cases, significant gross lesions, restricted to the lungs, consisted of numerous firm and coalescing nodules widely distributed throughout the lung. The nodules were <3 cm in diameter and pale white to tan in colour. Microscopically, they showed severe interstitial fibrosis and infiltration of macrophages, neutrophils, lymphocytes and a few eosinophils. The residual alveoli were lined by cuboidal epithelial cells (type II pneumocytes) and filled with many macrophages, which rarely displayed oval eosinophilic to amphophilic intranuclear inclusion bodies. Polymerase chain reaction and sequence analyses identified the glycoprotein H gene of EHV-5, and in-situ hybridization detected EHV-5 in the alveolar macrophages in the lesions. In one case, electron microscopy revealed herpesvirus-like particles and EHV-5 was isolated from pulmonary lesions. [ABSTRACT FROM AUTHOR]

Spinal eosinophilic granulomas (EGs) are uncommon tumors, constituting <1% of all bone tumors. They are mostly seen in the pediatric age group, whereas adult onset is rare. The cervical spine is an infrequent location for EG. The literature is sparse regarding the clinical and management aspects of these lesions, especially in adults. A literature review was performed by searching online databases to analyze all the reported cases of adult-onset cervical EG. Sixty-two cases were identified and analyzed. The male/female ratio was 3.1:1. The mean age at presentation was 32.8 years (range, 18–71 years). Neck pain, limb weakness, and restriction of movement were the most frequent symptoms. The C2 vertebra was the most frequently involved. The vertebral body was involved in >80% of patients and only 2 had vertebra plana morphology. The cervical lesions involved single vertebra in 42 patients (82%), whereas 9 patients (18%) had multiple vertebral involvement. Fifteen patients were managed conservatively, of whom 7 failed and required surgery later; 40 patients underwent definitive surgery and 17 received adjuvant treatment. Posterior-only surgeries were performed in 5 patients, anterior-only in 20, and circumferential in 14. The mean follow-up duration was 68 months (range, 12–268 months). The outcome was good to excellent in all, except 1, irrespective of type of treatment received. Cervical spine EG is rare in adults and usually presents as an osteolytic lesion involving the vertebral body; vertebra plana is very rare. Surgery is typically reserved for patients with severe neurologic deficits and bony instability. The outcome seems good in most cases. [ABSTRACT FROM AUTHOR]

Abstract Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg–Strauss syndrome (CSS), is a rare autoimmune disease with an estimated incidence of approximately 0.11 to 2.66 new cases per 1 million people per year and an overall prevalence of 10.7 to 14 per 1 million adults [1]. No gender predominance or ethnic predisposition has clearly been demonstrated in CSS [1]. Most of the patients are misdiagnosed over a period of time prior to being correctly classified with the disease. Here, we report the complex case of a 64-year-old African American man with advanced heart failure who received a left ventricular assist device (LVAD) and was subsequently diagnosed with EGPA. EGPA is a clinical syndrome that is associated with sequelae that can negatively add to the morbidity associated with LVAD placement. Highlights • The specific management of comorbidities in LVAD patients is an area where data are still emerging, and clinicians, albeit rarely, may encounter patients with EGPA. • EGPA is a clinical syndrome that is associated with sequelae that can negatively add to the morbidity associated with LVAD placement. • Pauci-immune glomerulonephritis is known to rapidly progress to ESRD and is associated with anti-MPO antibodies. • Thrombosis is a complication of eosinophilia. • There are three phases of EGPA: prodromal, eosinophilic, and the final vasculitic phase which usually arises 3 years post disease onset. [ABSTRACT FROM AUTHOR]

Abstract Objective To compare the serum glycerophospholipid levels in the inflammatory subtypes of asthma by using targeted metabolomic analysis. Methods Demographic and clinical data were collected from 51 patients with asthma between January 2015 and December 2015. Routine blood and sputum induction tests were performed. Eosinophilic asthma was defined as induced sputum containing ≥ 3% eosinophils, and neutrophilic asthma, as induced sputum containing ≥ 71% neutrophils. Serum metabolic glycerophospholipid profile was determined by liquid chromatography-mass spectrometry. Differences in glycerophospholipid levels between eosinophilic and non-eosinophilic asthma and between neutrophilic and non-neutrophilic asthma were analyzed using partial least squares discriminant analysis. Results The serum lysophosphatidylglycerol level was significantly higher in the group with ≥ 3% eosinophils in sputum than in the group with < 3% eosinophils in sputum. The area under the receiver-operating characteristic curve was ≥ 70%. There was no significant difference in the serum metabolic glycerophospholipid profile between the group with sputum neutrophils ≥ 71% and the group with sputum neutrophils < 71%. Conclusion Serum lysophosphatidylglycerol is produced abundantly in eosinophilic asthma and may be a biomarker of eosinophilic asthma. This information is helpful for identifying and tailoring treatment for the common asthma subtypes. [ABSTRACT FROM AUTHOR]

Parsonage-Turner Syndrome (PTS), also known as idiopathic brachial plexopathy or neuralgic amyotrophy, is an uncommon condition characterized by acute onset of shoulder pain, most commonly unilateral, which may progress to neurologic deficits such as weakness and paresthesias (Feinberg and Radecki, 2010 [1]). Although the etiology and pathophysiology of PTS remains unclear, the syndrome has been reported in the postoperative, postinfectious, and post-vaccination settings, with recent viral illness reported as the most common associated risk factor (Beghi et al., 1985 [2]). Various viral, bacterial, and fungal infections have been reported to precede PTS, however, currently there are no reported cases of PTS in the setting of recent infection with SARS-CoV2 (COVID-19). We present a case of a 17 year old female patient with no significant past medical or surgical history who presented with several weeks of severe joint pain in the setting of a recent viral illness (SARS-CoV2, COVID-19). MRI of the left shoulder showed uniform increased T2 signal of the supraspinatus, infraspinatus, teres minor, teres major, and trapezius muscles, consistent with PTS. Bone marrow biopsy results excluded malignancy and hypereosinophilic syndrome as other possible etiologies. Additional rheumatologic work-up was also negative, suggesting the etiology of PTS in this patient to be related to recent infection with SARS-CoV2 (COVID-19). Radiologists should be aware of this possible etiology of shoulder pain as the number of cases of SARS-CoV2 (COVID-19) continues to rise worldwide. • Parsonage-Turner syndrome (PTS) is a rare cause of acute unilateral shoulder pain. • Recent viral illness is the most common associated risk factor for PTS. • We report a novel case of PTS in the setting of SARS-CoV2 (COVID-19) infection. [ABSTRACT FROM AUTHOR]

Le syndrome de Churg et Strauss (SCS) ou granulomatose éosinophilique avec polyangéite est une vascularite systémique caractérisée par une artérite nécrosante, une infiltration éosinophilique et un granulome extravasculaire pouvant intéresser plusieurs organes. L'atteinte cardiaque représente la première cause de mortalité au cours de cette vascularite. Il s'agit de myocardite, de péricardite, de coronarite ou de dysrythmie et rarement d'atteinte valvulaire. Nous rapportons l'observation d'un SCS révélé par une périmyocardite aiguë. Churg-Strauss syndrome (CSS) or eosinophilic granulomatosis with polyangiitis is a systemic vasculitis characterized by necrotizing arteritis, eosinophilic infiltration and extravascular granuloma; that may involve several organs. Cardiac involvement is the first cause of death in this vasculitis. These include myocarditis, pericarditis, coronary heart disease, dysrhythmias, and rarely valvular involvement. We report the observation of CSS revealed by acute perimyocarditis. [ABSTRACT FROM AUTHOR]

Objective To investigate differences between childhood-onset ANCA-associated vasculitides (cAAVs) and matched adult-onset controls (aAAVs). Methods cAAV clinical pictures at onset and outcomes were compared to a randomly selected sample of aAAV patients from the French Vasculitis Study Group Registry. Cases and controls were matched for AAV (granulomatosis with polyangiitis [GPA], microscopic polyangiitis [MPA] or eosinophilic granulomatosis with polyangiitis [EGPA]), sex and year of enrollment. Medications, disease activity and damage were prospectively recorded. Kaplan–Meier curves and the log-rank test were used to analyze case-vs.-control differences for predefined outcomes. Results Comparing 35 cAAVs (25 GPA, 4 MPA, 6 EGPA) to 151 aAAVs (106 GPA, 17 MPA, 28 EGPA), their respective median follow-up durations were 71 and 64 months ( P = 0.49), and, at baseline, children had less frequent myalgias ( P = 0.005) and peripheral neuropathy ( P < 0.001) but were more frequently febrile ( P < 0.05). Rates of renal involvement were comparable (13 [37%] cAAVs vs. 73 [48%] aAAVs; P = 0.31). Initial GPA-associated ischemic abdominal pain and nasal cartilage damage were more common in cAAVs than aAAVs ( P < 0.05). During follow-up, the cAAV relapse rate was higher (24.5 vs. 18.7 flares per 100 patient-years; P < 0.05) and, at last visit, cases had accumulated more damage, mostly ear, nose & throat sequelae ( P = 0.001), associated with longer maintenance therapy ( P = 0.03), than aAAV controls. Four (11.4%) cAAV and 13 (8.6%) aAAV patients died ( P = 0.53). Conclusion cAAVs are severe diseases, characterized by a higher relapse rate, more accrued damage and longer maintenance therapy than for aAAVs. [ABSTRACT FROM AUTHOR]

Introduction Langerhans cell histiocytosis (LCH) is a rare uni or multisystem disorder associated with extreme production of immunoreactive Langerhans cells. Although this disorder has been reported in all age groups, spinal involvement especially thoracolumbar spine is seldom reported in toddlers. Case presentation We present a one (1) year, four (4) months female child with a history of recurrent fever, irritability and severe anemia of one (1) month duration. CT-scan and MRI revealed a collapsed twelfth thoracic vertebra (T12) body height greater than 95% with a huge intradural soft tissue mass. T12 cortectomy via posterior thoracolumbar approach was used to decompress the soft tissue part followed by interbody fusion with titanium cage filled with autologous iliac crest bone graft, fixation using plates and screws. Conclusion We are of the view that surgical decompression of spinal LHC lesions with interbody fusion with titanium cage filled with autologous iliac crest bone graft and fixation using plates and screws is very crucial in relieving neurological deficits. However, the patient will need repeated surgeries as she advances with age in a timely manner to avert any neurological deficit that may occur. [ABSTRACT FROM AUTHOR]

Background: Eosinophilic myocarditis (EM) is an acute life-threatening inflammatory disease of the heart. Neither large case series nor clinical trials on this specific myocarditis have been reported.Objectives: Based on a systematic revision of all published histologically proven cases, this study aimed to describe the clinical presentation, treatment, and outcome of EM.Methods: The study screened 443 manuscripts in MEDLINE and EMBASE on cases of EM published until June 2017. The authors identified 264 patients and included in the main analysis 179 patients admitted to hospital with histologically proven EM.Results: Median age was 41 years (interquartile range: 27 to 53 years) with similar prevalence in both sexes; pediatric cases (≤16 years of age) accounted for 10.1%. The main symptom at presentation was dyspnea (59.4%), with peripheral eosinophilia observed in 75.9%. Median left ventricular ejection fraction at presentation was 35% (interquartile range: 25% to 50%). The disorders most frequently associated with EM were hypersensitivity and eosinophilic granulomatosis with polyangiitis, which accounted for 34.1% and 12.8% of cases, respectively, whereas idiopathic or undefined forms accounted for 35.7% of cases. Steroids were administered in 77.7% of patients. A temporary mechanical circulatory support (n = 30) was instituted in 16.8% of patients. In-hospital death was 22.3% (n = 40), with the highest occurrence in the hypersensitivity form (36.1%; p = 0.026).Conclusions: EM has a poor prognosis during the acute phase, despite a publication bias that could have led to an overestimation of mortality. Associated conditions are identified in approximately 65% of cases. Specific trials and multicenter registries are needed to provide evidence-based treatments to improve in-hospital outcome. [ABSTRACT FROM AUTHOR]

Objective Atypical manifestations have been described in patients with ANCA-associated vasculitides (AAV), such as pachymeningitis, orbital mass or chronic periaortitis. Because these manifestations have been associated to the spectrum of IgG4-related disease (IgG4-RD), we hypothesized that both diseases could overlap. Methods We conducted a European retrospective multicenter observational study including patients fulfilling ACR and Chapel Hill criteria for AAV and IgG4-RD Comprehensive Diagnostic Criteria. Results Eighteen patients were included (median age 55.5 years, 13 men). AAV and IgG4-RD were diagnosed concomitantly in 13/18 (72%) patients; AAV preceded IgG4-RD in 3/18 (17%) while IgG4-RD preceded AAV in 2/18 (11%). AAV diagnoses included granulomatosis with polyangiitis in 14 (78%), microscopic polyangiitis in 3 (17%), and eosinophilic granulomatosis with polyangiitis in one case. IgG4-RD diagnosis included definite IgG4-RD in 5 (28%) cases, probable IgG4-RD in 5 (28%) and possible IgG4-RD in 8 (44%). IgG4-RD manifestations were chronic periaortitis in 9/18 (50%) patients, orbital mass and tubulointerstitial nephritis in 4 (22%) cases, prevertebral fibrosis in 3 (17%), pachymeningitis and autoimmune pancreatitis in 2 (11%) cases. Patients required median number of 2 (range 0–4) lines of immunosuppressants in combination with glucocorticoids. During the follow-up (median 49,8 months, range 17,25–108 months), AAV manifestations relapsed in 10/18 (56%) cases and IgG4-RD lesions in 5/18 (28%). When used, mainly for relapses, rituximab showed response in all cases. Conclusion AAV and IgG4-RD may overlap. Clinicians should consider that atypical manifestations during AAV could be related to IgG4-RD rather than to refractory granulomatous or vasculitic lesions. [ABSTRACT FROM AUTHOR]

Objective Describe the presentation, imaging characteristics, management, and outcomes of pediatric patients with Langerhans cell histiocytosis (LCH) of the temporal bone. Methods A retrospective chart review was performed between 2000 and 2014 at a single tertiary care children's hospital. Fourteen patients were identified with a diagnosis of LCH and involvement of the temporal bone. Results Ten patients were female and ten were Caucasian. Mean age at diagnosis was 3 years (range 0.3–9.6 years). The most common presenting symptoms were scalp lesions, postauricular lesions, otalgia, and persistent ear infections. Three patients had documented hearing loss. Four cases had otic capsule invasion. Computed tomography demonstrated a lytic temporal bone lesion within the following subsites: mastoid, squamous temporal bone, external auditory canal, middle ear, and petrous apex. Four patients had intracranial disease. Extent and location of disease prompted all patients to be initially managed with chemotherapy. Surgical excision was limited to one case of localized recurrence. After a mean follow-up of 85.2 months (SD 42.4 months) there were no deaths but eight patients had a recurrence. Two patients developed long-term otologic sequelae requiring surgery. One patient developed labyrinthitis ossificans. Conclusion LCH has a varied presentation, age distribution, and treatment algorithm. This series of 14 pediatric patients with temporal bone involvement supports a limited role for surgical resection. Long-term follow-up is critical for detection of local and disseminated disease, and for monitoring of otologic complications. [ABSTRACT FROM AUTHOR]

Background Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis characterized by hypereosinophilia. EGPA typically develops in three clinical phases, beginning with asthma, followed by tissue eosinophilia and finally systemic vasculitis. Cardiac involvement is the most important predictor of mortality; it occurs in approximately 15–60% of EGPA patients, a significant proportion of whom are asymptomatic and have normal electrocardiogram (ECG) and echocardiogram. Early detection and management of cardiac disease could positevely affect prognosis. Cardiovascular magnetic resonance (CMR) has emerged as the gold standard cardiac imaging technique in the evaluation of cardiomyopathies, due to its ability to reliably assess anatomy, function, and tissue characterization. Aim Purpose of this study was to assess the role of CMR in detecting cardiac disease in patients with EGPA in clinical remission. Methods A dedicated CMR protocol including functional analysis, and pre and post-contrast tissue characterization was performed in 11 patients with EGPA and the results were compared with 11 healthy subjects. Results EGPA patients had lower left ventricular ejection fraction compared to controls (56 ± 19 vs 68.7 ± 5.2, p value 0.02). Late gadolinium enhancement (LGE), representing replacement fibrosis, was positive in 9/11 (82%) patients, mainly with a non-ischemic pattern. In 3/11 (27%) patients a left ventricular thrombus was detected; in 3/11 (27%) patients myocardial edema was detected. CMR parameters of interstitial fibrosis were significantly more elevated in EGPA patients compared to controls. Conclusions Patients with EGPA in clinical remission showed a high cardiovascular burden as demonstrated by lower EF, signs of active inflammation, presence of interstitial and replacement fibrosis and intraventricular thrombosis. Further studies on wider populations are warranted to better understand how these findings could impact on prognosis and eventually guide therapy. [ABSTRACT FROM AUTHOR]

• Visceral larva migrans (VLM) is a complication of helminthic infection. • Rarely VLM can involve the heart, causing death. • Toxocara catis can infect a patient who has ingested cat feces. • The characteristic lesion of VLM is an eosinophilic granuloma. We discuss the pathophysiology and epidemiology of Toxocara catis infection complicated by visceral larval migrans. We describe a previously healthy 19-month-old white male child found dead in bed by his mother. He had what was thought to be an upper respiratory infection for several days.The child had eosinophilic hepatitis and myocarditis complicating visceral larval migrans due to Toxocara catis infection from the family cat. We discuss the pathologic and clinical aspects of visceral larval migrans due to of Toxocara catis infection. [ABSTRACT FROM AUTHOR]

Eosinophilic granulomatosis with polyangitis (EGPA) is a rare small- and medium-sized vessel vasculitis belonging to the group of anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV). It is commonly divided into two phenotypes depending on the presence of ANCAs targeting myeloperoxidase (MPO). MPO-ANCAs are present in 31% to 38% of patients and are associated with a vasculitis phenotype of the disease, whereas patients without MPO-ANCA are at risk of cardiac involvement. Despite significant advances in understanding the overall pathogenesis of the disease, the explanation for this dichotomy is still unclear. In this review, we synthesize our knowledge of the pathogenesis of EGPA and attempt to i) distinguish EGPA from other diseases including other AAVs, asthma, allergy and hypereosinophilic-associated conditions and ii) speculate about the preponderant mechanisms, which could explain the two disease phenotypes. [ABSTRACT FROM AUTHOR]

Background To investigate the prevalence and prognostic relevance of cardiac involvement in an ANCA-associated vasculitis (AAV) population of eosinophilic granulomatosis with polyangiitis (EGPA) and granulomatosis with polyangiitis (GPA) patients. Methods Prospective cohort study of fifty EGPA and forty-one GPA patients in sustained remission without previous in-depth cardiac screening attending our clinical immunology outpatient department. Cardiac screening included clinical evaluation, ECG, 24-hour Holter registration, echocardiography and cardiac magnetic resonance imaging (CMR) with coronary angiography and endomyocardial biopsy upon indication. Fifty age-, sex- and cardiovascular risk factor-matched control subjects were randomly selected from a population study. Long-term outcome was assessed using all-cause and cardiovascular mortality. Results A total of 91 AAV-patients (age 60 ± 11, range 63–87 years) were compared to 50-matched control subjects (age 60 ± 9 years, range 46–78 years). ECG and echocardiography demonstrated cardiac abnormalities in 62% EGPA and 46% GPA patients vs 20% controls ( P < 0.001 and P = 0.014, respectively). A total of 69 AAV-patients underwent additional CMR, slightly increasing the prevalence of cardiac involvement to 66% in EGPA and 61% in GPA patients. After a mean follow-up of 53 ± 18 months, presence of cardiac involvement using ECG and echocardiography in AAV-patients showed increased all-cause and cardiovascular mortality (Log-rank P = 0.015 and Log-rank P = 0.021, respectively). Conclusion Cardiac involvement in EGPA and GPA patients with sustained remission is high, even if symptoms are absent and ECG is normal. Moreover, cardiac involvement is a strong predictor of (cardiovascular) mortality. Therefore, risk stratification using cardiac imaging is recommended in all AAV-patients, irrespective of symptoms or ECG abnormalities. [ABSTRACT FROM AUTHOR]

Background Context: Benign tumors of the vertebrae are generally an uncommon cause for surgery. Complete removal of these tumors requires in most cases extensive surgical technique that consists of generous surgical exposure followed by laminectomy, facetectomy, and sometimes even an instrumented fusion.Purpose: The aim was to describe our experience in performing resection of benign vertebral tumors, using a minimally invasive surgical (MIS) approach.Study Design: This was a retrospective review of case records.Patient Sample: Patients who underwent MIS, resection of benign vertebral tumors.Outcome Measures: Complete neurologic examination and pain evaluation, as measured by the visual analog scale (VAS). Secondary outcomes included postoperative spinal instability assessment and surgical margins examinations.Methods: Patients were evaluated preoperatively and postoperatively at 1, 3, and 6 months intervals clinically and radiographically using plain radiographs and postoperative computed tomography (CT) scans. Final pathologic report, operative time, blood loss, complications, and hospital length of stay were also recorded.Results: Between 2009 and 2013, 14 patients underwent MIS, resection of benign vertebral tumors at our institution. Mean follow-up time was 4 years. There were eight men and six women with a mean age of 27 years (range 16-68 years). For tumors located in the posterior elements, a direct posterior approach was used. Tumors located at the pedicle of the vertebra were excised using a transpedicular approach, and tumors protruding into the foramen were excised using the transforaminal approach. The transcanal approach was used when decompression of the thecal sac or nerve root was required, and the retroperitoneal transpsoas approach was used for tumors located in the vertebral body. Complete removal of these tumors was achieved in all cases, and was verified by a follow-up CT scan. Pathology revealed osteoid osteoma in five patients, osteoblastoma in three patients. Eosinophilic granuloma, fibrous dysplasia, and fibroid adenoma were found in one case each. Average VAS pain score improved from 7.7 (7-9) to 2.8 (0-7) after surgery.Conclusions: Minimally invasive techniques are a valuable choice for the treatment of benign osseous tumors of the spine. A larger, long-term study is in progress. In the meantime, we suggest surgeons experienced with both open and MIS surgery should consider these techniques. [ABSTRACT FROM AUTHOR]

Objective To determine the diagnostic and prognostic significance of cardiac magnetic resonance imaging (CMRI) in a cohort of patients with eosinophilic granulomatosis with polyangiitis (EGPA). Methods We conducted a monocentric retrospective study including 42 EGPA patients who had consecutively undergone CMRI at diagnosis or during follow-up, independently of signs of cardiac involvement. Results Forty-two patients (male 59.5%, mean age at diagnosis 46.5 years) were included. ANCA was positive in 26.2%, and median EGPA duration before the 1st CMRI screening was 5 months. Seventeen (40.5%) were diagnosed with cardiomyopathy, independently of CMRI findings. CMRI showed myocardial late gadolinium enhancement (LGE) in 82.4% patients with cardiomyopathy vs. 44% without cardiomyopathy (P = 0.024). Using LGE as the sole criterion, CMRI sensitivity and specificity for diagnosing cardiomyopathy were 82.4% and 56%, respectively. Among the 15 patients with cardiomyopathy who underwent additional CMRI during follow-up, CMRI-detected cardiac lesions had improved in 7 patients, while those of 8 patients worsened or stabilized despite treatment. These latter patients presented with significantly more cardiac events during follow-up (P = 0.026). No differences were found between non-cardiomyopathic patients with or without CMRI anomalies concerning EGPA cardiac manifestations and outcomes. Conclusion The diagnostic significance of myocardial LGE in EGPA patients remains uncertain and should not be the only criterion for cardiomyopathy diagnosis. For patients with no other signs of cardiomyopathy, CMRI-detected anomalies do not seem to adversely affect prognosis or outcome. For patients with cardiomyopathy, CMRI reassessment seems promising in detecting patients with a less favorable cardiac outcome. [ABSTRACT FROM AUTHOR]

The anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitides granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA) and microscopic polyangiitis (MPA) constitute a wide spectrum of clinical manifestations of systemic vasculitis which may range from limited disease to organ or life-threatening disease. The introduction of biologics for the management of severe ANCA-associated vasculitis and severe, relapsing disease refractory to conventional immunosuppressants, has significantly improved the clinical prognosis of these autoimmune disorders. Rituximab, an anti-CD20 monoclonal antibody is licenced for remission induction in severe GPA and MPA and the management of severe relapsing, refractory GPA and MPA. Belimumab, an anti-B lymphocyte stimulatory monoclonal antibody is in clinical trials for the management of the ANCA-associated vasculitis GPA. Mepolizumab and Omalizumab are biologics which have been reported to be efficacious in refractory asthma associated with EGPA. The role of anti-TNF therapy and T cell targeting drugs in ANCA-associated vasculitis is less clear due to limited study data. This review will summarise the clinical trials and clinical practice use of biologic treatment strategies for the management of ANCA-associated vasculitis. [ABSTRACT FROM AUTHOR]

Objectives To investigate the accuracy of quantitative diffusion-weighted imaging with apparent diffusion coefficient (ADC) mapping for characterizing bone lesions as benign or malignant. Methods At 3 T, 31 subjects with intramedullary lesions imaged by DWI ( b -values 50, 400, 800 s/mm 2 ) were included. ADC values (minimum, mean, maximum) were recorded by three observers independently. Interobserver variability and differences between ADC values in benign and malignant lesions were assessed (unpaired t -test, receiver operating characteristic (ROC) analysis). Results Of 31 lesions, 18 were benign (osteoblastic ( n = 1), chondroid ( n = 6), cysts ( n = 4), hemangiomatosis ( n = 1), fibrous ( n = 3), eosinophilic granuloma ( n = 1), giant cell tumor ( n = 1), osteomyelitis ( n = 1)) and 13 were malignant (primary ( n = 5), metastases ( n = 8)). Overall, there were higher minimum (1.27 × 10 −3 mm 2 /s vs 0.68 × 10 −3 mm 2 /s, p < 0.001), mean (1.68 × 10 −3 mm 2 /s vs 1.13 × 10 −3 mm 2 /s, p < 0.001), and maximum (2.09 × 10 −3 mm 2 /s vs 1. 7 × 10 −3 mm 2 /s, p = 0.03). ADC values in benign lesions compared with those in malignancies. ROC analysis revealed areas under the curve for minimum, mean, and maximum ADC values of 0.91, 0.85, and 0.71, respectively. ADC measurements were made with high inter-observer concordance ( ρ = 0.83–0.96). Conclusion Quantitative ADC maps may have predictive value for the characterization of bone lesions. Benign lesions generally have higher minimum, mean, and maximum ADC values than malignancies, with the minimum value offering the highest accuracy for characterization. [ABSTRACT FROM AUTHOR]

The rare form and mildest variant of Langerhans cell histiocytosis is eosinophilic granuloma (EG). In the clinical presentation, EG can be monostotic, polyostotic, or can encompass many organs. The parietal bone is the most common location of the skull bones that are affected by EG. So far, there have been no reported cases of EG with skull odor as an unexplained presentation. An 8-year-old girl presented with a 4 months history of a right parietal bone swelling of the skull with an offensive odor. There was no discharge and no history of vomiting or trauma. An MRI scan of the brain showed swelling with a bone lesion of the right parietal bone. Infection was the source of the swelling and the bad odor. Treatment was done by surgical excision of the lesion. EG has a variety of presentations and should be suspected when tenderness and local swelling are present. Radiography was found to be helpful in the diagnosis and surgical treatment was done to manage the case. • Most common site to be affected by EG in the Skull is the parietal bone. • EG may be infected and present with an offensive odor. • EG cells Stains positive for S-100 and CD1a. • Most of the Cases are reported among children and most of them present with Headache. [ABSTRACT FROM AUTHOR]