Recurrent CLTC::SYK fusions and CSF1R mutations in juvenile xanthogranuloma of soft tissue.

Juvenile xanthogranuloma (JXG) is a histiocytic neoplasm that usually presents in the skin. Rarely, extracutaneous localizations occur; the genetic drivers of this clinical variant of JXG remain incompletely characterized. We present detailed clinicopathologic and molecular data of 16 children with extracutaneous JXG and 5 adults with xanthogranulomas confined to the central nervous system (CNS) or soft tissue. Tissue samples were obtained through the Dutch Nationwide Pathology Databank and analyzed with an innovative sequencing technique capable of detecting both small genomic variants and gene rearrangements. Targetable kinase alterations were detected in 16/16 children and 1/5 adults. Alterations included CLTC::SYK fusions in 6 children and CSF1R mutations in 7 others - all below 2 years old with soft tissue tumors. One child had a CSF1R mutation and MRC1::PDGFRB fusion. Most were treated surgically, although spontaneous regression occurred in 1/6 with CLTC::SYK and 2/7 with CSF1R mutations - underscoring that treatment is not always necessary. Tumors with CLTC::SYK fusions generally lacked Touton giant cells, but exhibited many other histologic features of JXG and concordant methylation profiles. Using multispectral immunofluorescence, phosphorylated-SYK expression was localized to CD163+ histiocytes; tumors with CLTC::SYK fusions also demonstrated mTOR activation, Cyclin D1 expression, and variable phosphorylated-ERK expression. BRAFV600E was detected in 1 child and 1 adult with CNS xanthogranulomas; both responded to BRAF inhibition. Finally, a TPM3::NTRK1 fusion or MAP2K1 deletion were detected in 2 children with systemic JXG who experienced spontaneous disease regression. This study advances the molecular understanding of histiocytic neoplasms and may guide diagnostics and clinical management.

Assessment of psycho-oncology in the Middle East and North Africa region: a systematic review and meta-analysis.

BACKGROUND: The Middle East and North Africa (MENA) region is expected to witness a significant increase in the burden of cancer. Contrary to Western literature, the burden of psycho-oncology is yet to be established within the MENA region. This study reviews all available evidence characterizing the psychological burden among patients with cancer across the MENA region. METHODS: We systematically explored the PubMed/MEDLINE, Cochrane/CENTRAL, and Web of Science (WoS) databases for reports on the psychiatric burden among patients with cancer residing within the MENA region from January 2000 until January 2023. Raw proportion were extracted and analyzed using a random-effects model. FINDINGS: Eighty-three studies comprised of 16 810 participants, representing 14 countries, met our inclusion criteria. Across the MENA region, the prevalence of depression, anxiety, and distress were 44% (95% CI, 39%-50%), 47% (95% CI, 40%-54%), and 43% (95% CI, 30%-56%), respectively. Prevalence of depression was significantly different across countries, with Palestine (73%; 95% CI, 42%-91%) reporting the highest rate while Morocco (23%; 95% CI, 7%-56%) reported the lowest. Similarly, anxiety significantly differed across MENA nations ranging from 64% (95% CI, 3%-99%) in Morocco to 28% (95% CI, 18%-42%) in Tunisia. Rates of depression and anxiety were significantly different across measurement tools but not between Arabic-speaking versus Persian/Farsi-speaking countries. Meta-regression models showed that neither publication year nor age affected the prevalence of both anxiety and depression (P = .374 and .091 for depression and P = .627, and .546 for anxiety, respectively). INTERPRETATION: We report an abnormally high rate of psychiatric burden among patients with cancer in the MENA region. Thus, establishing appropriate psycho-oncologic interventions within the MENA region is of utmost importance.

Factors affecting cervical screening using the health belief model during the last decade: A systematic review and meta-analysis.

AIMS: To examine the utility of the health belief model (HBM) and other socioeconomic factors in shaping cervical screening behaviors. Also, to provide recommendations on improving screening uptake. METHODOLOGY: A systematic literature search was conducted using the PubMed/MEDLINE, Cochrane/CENTRAL, and Web of Science databases for articles reporting on the factors associated with cervical screening using the HBM within the period from January of 2002 to January of 2023. Effect sizes for the various HBM constructs were pre-determined using the log odds ratio (logOR) and expressed with their confidence intervals. All reporting was in line with the PRISMA guidelines. RESULTS: A total of 21 studies were included in the final analysis comprised of 15,365 participants. Our pooled analysis demonstrated that perceived susceptibility (OR: 1.40, 95% CI, 1.03-1.89), perceived benefits (OR: 1.30; 95% CI, 1.13-1.50), and self-efficacy (OR: 1.11; 95% CI, 1.05-1.17) were significantly associated with both the uptake of and intention to adopt preventive measures against cervical cancer. Conversely, women with higher perceptions of barriers were less likely to adopt any measure for cervical cancer screening or prevention (OR: 0.72; 95% CI, 0.57-0.91). In terms of sociodemographic effectors, older age (OR: 1.09; 95% CI, 1.01-1.19), graduate/post-graduate education (OR: 2.80; 95% CI, 1.46-5.37), higher knowledge of cervical cancer (OR: 2.21; 95% CI, 1.27-3.84), and being married (OR: 3.89; 95% CI, 1.38-10.92) were all associated with altering preventive behaviors and intentions toward cervical cancer. CONCLUSION: This review delineates the most important and effective cognitive components that should be targeted within interventions aiming to promote cervical cancer prevention.

Napsin A Immunoreactivity in "Hobnail" Epithelium in Benign Endometrial and Endocervical Polyps.

Endometrial and endocervical polyps not uncommonly exhibit focal benign "hobnail" change/metaplasia within the glandular epithelium, sometimes in association with inflammation or infarction. In most cases, this is readily recognized as benign but occasionally, especially in endometrial polyps, this change prompts consideration of a premalignant or malignant lesion, including early serous or clear cell carcinoma. Herein we highlight the previously unreported phenomenon of positive staining of this hobnail epithelium with Napsin A which has the potential to exacerbate concern for clear cell carcinoma. Endometrial (n = 22) and endocervical (n = 17) polyps showing hobnail change were stained with Napsin A. Six cases were positive (4 of 22 endometrial and 2 of 17 endocervical polyps). In all cases, Napsin A positivity was confined to the hobnail epithelium. The hobnail epithelium was positive with estrogen receptor and hepatocyte nuclear factor 1- beta and exhibited wild-type immunoreactivity with p53 in all cases where these markers were performed. In addition, in 2 of 3 uterine adenosarcomas with focal hobnail change the epithelium was Napsin A positive. Pathologists should be aware that Napsin A may be expressed in benign/reactive hobnail epithelium in endometrial and endocervical polyps and should not consider positivity with this marker as a diagnostic of clear cell carcinoma.

Modifiable risk factors for cancer in the middle East and North Africa: a scoping review.

PURPOSE: This scoping review examines controllable predisposing factors attributable to cancer in the Middle East and North Africa (MENA) region's adult population, highlighting opportunities to enhance cancer prevention programs. DESIGN: We systematically searched the PubMed, Science Direct, and CINAHL, EMBASE, and Cochrane Library databases from 1997 to 2022 for articles reporting on the impact of modifiable risk factors on adult patients with cancer in the MENA region. RESULTS: The review identified 42 relevant articles, revealing that tobacco consumption, obesity, physical inactivity, and diet are significant modifiable risk factors for cancer in the region. Tobacco smoking is a leading cause of lung, bladder, squamous cell carcinoma, and colorectal cancer. A shift towards a westernized, calorie-dense diet has been observed, with some evidence suggesting that a Mediterranean diet may be protective against cancer. Obesity is a known risk factor for cancer, particularly breast malignancy, but further research is needed to determine its impact in the MENA region. Physical inactivity has been linked to colorectal cancer, but more studies are required to establish this relationship conclusively. Alcohol consumption, infections, and exposure to environmental carcinogens are additional risk factors, although the literature on these topics is limited. CONCLUSION: The review emphasizes the need for further research and the development of targeted cancer prevention strategies in the MENA region.

Evaluating the understanding of the ethical and moral challenges of Big Data and AI among Jordanian medical students, physicians in training, and senior practitioners: a cross-sectional study.

AIMS: To examine the understanding of the ethical dilemmas associated with Big Data and artificial intelligence (AI) among Jordanian medical students, physicians in training, and senior practitioners. METHODS: We implemented a literature-validated questionnaire to examine the knowledge, attitudes, and practices of the target population during the period between April and August 2023. Themes of ethical debate included privacy breaches, consent, ownership, augmented biases, epistemology, and accountability. Participants' responses were showcased using descriptive statistics and compared between groups using t-test or ANOVA. RESULTS: We included 466 participants. The greater majority of respondents were interns and residents (50.2%), followed by medical students (38.0%). Most participants were affiliated with university institutions (62.4%). In terms of privacy, participants acknowledged that Big Data and AI were susceptible to privacy breaches (39.3%); however, 59.0% found such breaches justifiable under certain conditions. For ethical debacles involving informed consent, 41.6% and 44.6% were aware that obtaining informed consent posed an ethical limitation in Big Data and AI applications and denounced the concept of "broad consent", respectively. In terms of ownership, 49.6% acknowledged that data cannot be owned yet accepted that institutions could hold a quasi-control of such data (59.0%). Less than 50% of participants were aware of Big Data and AI's abilities to augment or create new biases in healthcare. Furthermore, participants agreed that researchers, institutions, and legislative bodies were responsible for ensuring the ethical implementation of Big Data and AI. Finally, while demonstrating limited experience with using such technology, participants generally had positive views of the role of Big Data and AI in complementing healthcare. CONCLUSION: Jordanian medical students, physicians in training and senior practitioners have limited awareness of the ethical risks associated with Big Data and AI. Institutions are responsible for raising awareness, especially with the upsurge of such technology.

Mesonephric-like adenocarcinoma of the female genital tract: novel observations and detailed molecular characterisation of mixed tumours and mesonephric-like carcinosarcomas.

AIMS: To report novel observations in five mesonephric-like adenocarcinomas (MLAs) of the female genital tract. METHODS AND RESULTS: We report two endometrial MLAs in association with endometrioid carcinoma and atypical hyperplasia and three (one endometrial, two ovarian) cases with a sarcomatoid component (mesonephric-like carcinosarcoma). Pathogenic KRAS mutations, which are characteristic of MLA, were identified in all cases although interestingly, in one of the mixed carcinomas, this was confined to the endometrioid component. The concurrent MLA, endometrioid carcinoma and atypical hyperplasia components in one case harboured identical EGFR, PTEN and CCNE1 mutations, suggesting that the atypical hyperplasia gave rise to a Müllerian carcinoma with both endometrioid and mesonephric-like components. The carcinosarcomas all contained a component of MLA and a sarcomatous component with chondroid elements. In the ovarian carcinosarcomas, the coexisting epithelial and sarcomatous components shared some mutations including KRAS and CREBBP, suggesting that they are clonally related. Furthermore, in one case CREBBP and KRAS mutations detected in the MLA and sarcomatous components were also detected in an associated undifferentiated carcinoma component, suggesting that it was clonally related to the MLA and sarcomatous components. CONCLUSIONS: Our observations provide additional evidence that MLAs have a Müllerian origin and characterise mesonephric-like carcinosarcomas in which chondroid elements appear to be characteristic. In reporting these findings, we provide recommendations for distinction between a mesonephric-like carcinosarcoma and a MLA with a spindle cell component.

SATB2 Cytoplasmic Expression is Characteristic of a Subset of Ovarian Stromal Cells and Sex Cord Stromal Tumors.

Special AT-rich sequence-binding protein 2 (SATB2) is a nuclear transcription factor that shows consistent nuclear staining in colorectal adenocarcinoma and osteosarcoma. Following the observation of cytoplasmic staining with this marker in luteinized ovarian stromal cells, we studied the expression of SATB2 in ovarian stromal cells, various types of follicular cysts, and sex cord-stromal tumors. Eighty-five cases were stained for SATB2. Ovarian hilar Leydig cells (n = 12), luteinized stromal cells (n = 10), corpora lutea (n = 4), luteinized follicular cysts (n = 4), and stromal hyperthecosis (n = 6) exhibited consistent, usually diffuse, granular cytoplasmic staining. In addition, Leydig cell tumors (n = 1) and steroid cell tumors (n = 4) showed diffuse cytoplasmic staining. SATB2 also exhibited cytoplasmic staining in most Sertoli-Leydig cell tumors (n = 16) and gynandroblastomas (n = 3) confined to the Leydig cell component. Adult granulosa cell tumors (n = 14), juvenile granulosa cell tumors (n = 3), sex cord tumors with annular tubules (n = 3), cellular fibromas (n = 3), sclerosing stromal tumors (n = 1), and thecomas (n = 1) were negative apart from cytoplasmic staining in associated luteinized stromal cells. SATB2 cytoplasmic staining has not been previously described in these lesions but is characteristic of a variety of ovarian stromal cells and sex cord-stromal tumors, in particular, those exhibiting luteinization or a Leydig or steroid cell component. SATB2 staining may be of value in identifying luteinized or Leydig cells when these are morphologically inconspicuous.

Ovarian Immature Teratoma With Nodal Gliomatosis: A Case Report and Literature Review.

Gliomatosis involving lymph nodes (nodal gliomatosis) is rarely encountered in association with an ovarian teratoma, with 12 cases previously reported. We report this rare occurrence in a 23-yr-old female with an ovarian immature teratoma. The ovary contained a grade 3 immature teratoma, with immature neuroepithelium. A subcapsular liver mass contained metastatic immature teratoma with neuroepithelium. The omentum and peritoneum contained mature glial tissue, consistent with gliomatosis peritonei with no evidence of immature elements. One pelvic lymph node contained multiple nodules of mature glial tissue, diffusely positive for glial fibrillary acidic protein, in keeping with nodal gliomatosis. In reporting this case, we review prior reports of nodal gliomatosis.

Effect of an educational intervention on the knowledge, attitudes, and practices of healthcare workers at King Hussein Cancer Center towards predatory publishers.

AIMS: We explored the effect of an educational intervention on the knowledge, attitudes, and practices of healthcare workers (HCWs) towards predatory publishing. METHODS: A retrospective pre-post quasi experimental design was implemented on HCWs within King Hussein Cancer Center (KHCC). Following a 60-min educational lecture, a self-administered questionnaire was completed by participants. Pre- and post-intervention scores for familiarity, knowledge, practices, and attitudes were compared using the paired sample t-test. Multivariate linear regression was used to identify predictors of mean differences (MD) of knowledge scores. RESULTS: A total of 121 respondents completed the questionnaire. The majority of participants demonstrated underwhelming awareness of predatory publishing and average levels of knowledge of their characteristics. Furthermore, respondents did not take the necessary precautions to avoid predatory publishers. The intervention (i.e. the educational lecture) improved familiarity (MD: 13.4; 95%CI: 12.4 - 14.4; p-value < .001), knowledge of predatory journal's characteristics (MD: 12.9; 95%CI: 11.1 - 14.8; p-value < .001), awareness and perceived compliance to preventive measures (MD: 7.7; 95%CI: 6.7 - 8.6; p-value < .001), and positively influenced attitudes towards open access and safe publishing (MD: 0.8; 95%CI: 0.2 - 1.5; p-value = 0.012). Females had significantly lower familiarity scores (p-value = 0.002). Moreover, those who had published in open access journals, received at least one predatory e-mail, or had more than 5 published original articles had significantly higher familiarity and knowledge scores (all p-value < 0.001). CONCLUSIONS: An educational lecture proved effective in improving awareness of KHCC's HCW's to predatory publishers. Nonetheless, the mediocrity of pre-intervention scores raises concerns on effectiveness of the predatory covert practices.

Sex differences in cancer incidence and mortality among patients managed at King Hussein Cancer Center.

We investigated if female survival advantage trends in cancer are consistent within Jordanian patients with cancer across different age groups and nonsex specific cancer types. We explored the King Hussein Cancer Center registry for primary malignant tumors from 2006 to 2019. The registry (n = 16 454) was stratified into three groups based on age: children (<15 years), adolescents and young adults (AYA) (modified; 15-49 years), and older adults (≥50 years). Kaplan-Meier analysis was used to estimate the 5-year all-site cancer-specific survival probabilities, which were compared using the log-rank test by sex and age group. Out of 16 454 eligible records, 2286 (13.9%) were children, 5975 (36.3%) were AYAs, and 8193 (49.8%) were older adults. Males outnumbered females 10 339 (62.8%) to 6115 (37.2%). The 5-year OS rates were 74.0% (71.6%-76.4%) and 72.7% (69.9%-75.5%) for pediatric males and females, respectively, 57.3% (55.6%-59.0%) and 64.5% (62.6%-66.4%) for male and female AYAs, respectively, and 37.5% (36.2%-38.9%) and 44.2% (42.3%-46.2%) for older adult males and females, respectively. Females demonstrated significantly better overall survival in the AYA and older adults' groups. In conclusion, females exhibit a survival advantage in terms of nonsex specific cancers. This advantage peaks at the AYA age stratum and mitigates thereafter. Further studies are warranted to examine the etiological factors behind such discrepancy on a site-by-site basis so that sex-specific interventions can be designed and validated.

Feasibility of treating pediatric intracranial germ cell tumors in a middle-income country: The Jordanian experience.

BACKGROUND: Pediatric intracranial germ cell tumors (iGCT) are rare, with limited data available from Arabic countries. METHODS: We retrospectively reviewed the medical charts of children <18 years diagnosed with iGCT at King Hussein Cancer Center/Jordan (January 2003 to December 2020) for clinical characteristics, treatment, and morbidities. RESULTS: Sixteen patients had germinoma; median age was 6.9 years and median symptoms duration 8 months. Nine tumors were suprasellar, five pineal, and two bifocal. Four were metastatic. Eight patients had slightly elevated beta subunit human chorionic gonadotropin and 11 patients had resection/biopsy. Fifteen patients received chemotherapy; mostly carboplatin (450 mg/m2 )/etoposide, which had low toxicity. All patients received radiotherapy (different doses and fields). At median follow-up of 7.7 years, one tumor recurred (progression-free survival: 91% ± 8%). Twelve patients who continued follow-up had stable visual and endocrine deficits to their initial presentation. Five finished or are finishing diploma and seven had poor school performance (four left school). Six patients were diagnosed with nongerminomatous germ cell tumor; median symptom duration was 1 month. Three tumors were pineal, two suprasellar, and one at quadrigeminal plate. Three were metastatic. Five tested patients had high tumor markers and four had resection/biopsy. All patients received chemotherapy, and then five received craniospinal radiation. Two patients are alive, two died with tumor progression, one died in remission with electrolyte imbalance, and one developed leukemia and died with septic shock. CONCLUSIONS: We achieved excellent survival in treating germinoma using a feasible protocol for low middle-income countries. However, patients encountered significant morbidities exacerbated by delayed diagnosis and unnecessary surgical interventions despite abnormal tumor markers. Raising awareness on iGCT symptomatology and diagnosis may help limit these morbidities.

H3 K27M-mutant diffuse midline glioma with osseous metastases: A case report and a literature review.

INTRODUCTION: Diffuse midline glioma (DMG) is a primary tumor of the central nervous system (CNS) with aggressive nature. It arises from midline structures in the brain and spinal cord. Recently, the presence of H3 K27M mutation is described in most cases. Extra-cranial osseous metastasis is rarely encountered. CASE PRESENTATION: We present an interesting case of DMG with bone metastasis at presentation in a 19-year-old male. In addition, a literature review on similar cases is presented. DISCUSSION: DMG mostly affects children and young adults. It has a broad spectrum of phenotypes, shows diffuse growth pattern, midline location, and is frequently H3 K27M-mutant. Radiotherapy remains the mainstay of treatment that might improve overall survival. Metastasis outside the CNS remains a rare occurrence, especially at presentation, and constitute a diagnostic challenge. CONCLUSION: Bone is one of the most common sites for metastasis of primary CNS tumors, which would severely impact prognosis. Oncologists, radiologists, and pathologists should keep an index of suspicion when encountering bone metastasis in the presence of a CNS midline tumor, so that timely diagnosis and management can be rendered.

Religion, Islam, and Compliance with COVID-19 Best Practices.

While many have implemented best practices intended to help stem the spread of COVID-19, there are also a substantial number of citizens, both domestically and abroad, who have resisted these practices. We argue that public health authorities, as well as scientific researchers and funders, should help address this resistance by putting greater effort into ascertaining how existing religious practices and beliefs align with COVID-19 guidelines. In particular, we contend that Euro-American scholars-who have often tended to implicitly favor secular and Christian worldviews-should put added focus on how Islamic commitments may (or may not) support COVID-19 best practices, including practices that extend beyond the domain of support for mental health.

How Telemedicine and Centralized Care Changed the Natural History of Retinoblastoma in a Developing Country: Analysis of 478 Patients.

PURPOSE: To evaluate the efficacy of integrating a telemedicine-based twinning partnership and centralized care for retinoblastoma on survival and eye salvage. DESIGN: Four hundred seventy-eight retinoblastoma patients treated at a tertiary referral cancer center (King Hussein Cancer Centre [KHCC]) from 2003 through 2019. PARTICIPANTS: Four hundred seventy-eight retinoblastoma patients treated at KHCC after implementing a telemedicine-based program with St. Jude Children's Research Hospital. METHODS: We reviewed the outcomes of retinoblastoma patients who were treated at KHCC after implementing a telemedicine-based eye salvage program with St. Jude Children's Research Hospital, and we compared that with outcomes for retinoblastoma patients who were treated before implementing a telemedicine-based retinoblastoma service at KHCC. MAIN OUTCOME MEASURES: We analyzed patient demographics, clinical characteristics, treatments received, consultation type and duration, and long-term patient outcomes before and after implementing the twinning program. RESULTS: Over 17 years, 813 eyes from 478 children with retinoblastoma were treated at KHCC. Three hundred thirty-five patients (70%) had bilateral disease. Six patients (4%) with unilateral disease and 66 patients (20%) with bilateral disease had a family history of retinoblastoma. After the twinning program was established in 2003, the mortality rate decreased from 38% to 5% (P < 0.0001), and the overall eye salvage rate increased from 4% to 61% (98% for group A, 93% for group B, 81% for group C, and 48% for group D; P < 0.0001). Initially, all cases were discussed via telemedicine, but as knowledge transfer increased, the proportion of cases that required discussion decreased to less than 3% 10 years later. Similarly, treatment changes based on consultations decreased from 70% to 7% after 10 years. Both survival and eye-salvage rates were comparable at the early and later stages of implementing the twinning program. At a median follow-up of 120 months, 5% of patients had died of metastases or secondary neoplasms, 81% were alive, and 14% were lost to follow-up. CONCLUSIONS: Centralization of care at a single center in developing countries can achieve patient outcomes comparable with those of developed countries via twinning and telemedicine. This benefit can extend to a large region because two thirds of patients treated at KHCC were non-Jordanians.

The Predictive Value of the Eighth Edition of the Clinical TNM Staging System for the Likelihood of Eye Salvage for Intraocular Retinoblastoma by Systemic Chemotherapy and Focal Therapy.

BACKGROUND: The American Joint Committee on Cancer/Union for International Cancer Control (AJCC/UICC) cTNM staging is emerging as a universal staging for all cancers, including retinoblastoma. METHODS: Here we evaluated the predictive value of the eighth edition AJCC/UICC cTNM staging in comparison with the International Intraocular Retinoblastoma Classification for eye globe salvage by primary systemic chemotherapy and focal therapy (CRD) using logistic regression model for the probability of treatment failure. RESULTS: The eye salvage rate for 565 treated eyes was 95% (n=139/147) for T1 tumors (98% for T1a and 93% for T1b), 56% (n=230/410) for T2 (81% for T2a and 53% for T2b), and 0% for T3 tumors, and was 98%, 93%, 76%, and 44% for group A, B, C, and D tumors, respectively. As estimated by odds ratios, T2 were 13.6-fold more likely to fail treatment than T1, and T1b, T2a, and T2b were 2.8-, 9.4-, and 35.1-fold more likely to fail treatment than T1a, respectively. Group B, C, and D tumors were 2.8-, 12.7-, and 50.1-fold more likely to fail treatment than group A tumors, respectively. Eye salvage rate was 62% for eyes with focal seeds (3 mm close to the tumor), and 42% for eyes with diffuse seeds (clouds more than 3 mm from tumor edge) (P<0.0001). CONCLUSION: Both, the eighth edition cTNM classification and the International Intraocular Retinoblastoma Classification systems, can effectively predict eye salvage rates for retinoblastoma by CRD. Eyes with higher cT stages are more likely to experience treatment failure. Because the cT2b group is very heterogeneous, our findings suggest further division of this group based on the severity of vitreous/subretinal seeds, this should be revised in the next edition of cTNM system.

Diffuse midline glioma H3 K27M-mutant in adults: A report of six cases and literature review.

AIM: Diffuse midline glioma (DMG) H3 K27M-mutant is a specific entity that, as the name indicates, tends to occur in midline structures including the thalamus, brainstem, and spinal cord. DMG predominates in children, is an aggressive tumor with poor prognosis, and is considered a WHO grade IV tumor regardless of histological features. The exact frequency of these mutations in adults diagnosed with glioma in the midline is unknown. MATERIALS AND METHODS: We report a series of 6 more adult cases, and we critically review the current literature on adults with DMG H3 K27M-mutant. RESULTS: There were 5 males and 1 female. The age ranged from 26 to 52 years (median 39 years). All cases showed astrocytic differentiation, with positive staining for H3 K27M protein, and loss of H3 K27me in the tumor cells confirming the diagnosis. CONCLUSION: H3 K27M-mutant midline glioma can occur in adults, affecting midline structures. Increasing awareness of the reporting pathologists of this entity might help in a better determination of the frequency of mutant DMG in adults as well as better diagnosis and patient counseling of the outcome.

Central Nervous System Tumors in Adolescents and Young Adults: An Epidemiological Study from Jordan.

BACKGROUND: The adolescent and young adult (AYA) age group lacks targeted epidemiologic studies that assess the prevalence and outcome of tumors. We aim to provide deep analysis of the epidemiology of central nervous system (CNS) tumors in AYA in Jordan. METHODOLOGY: This is a retrospective study for all CNS tumors in the AYA group patients diagnosed and managed at King Hussein Cancer Center in 2007-2016. A patient list was retrieved from the Center's Cancer Registry, and clinicopathologic data were reviewed individually from the patients' records. RESULTS: A total of 370 cases of primary CNS tumors were retrieved, with a median age of 28.5 years. Males outnumbered females; 57.6 and 42.4%, respectively. Most tumors occurred in the cerebrum (62.2%, n = 230), the frontal lobe was the most commonly affected (29%). Glioma was the most common histologic category (58.9%, n = 218), with high-grade tumors, including glioblastoma and anaplastic astrocytoma, prevailing. Embryonal tumors comprised the second most common group (16.8%, n = 62). Medulloblastoma was the prototype of embryonal tumors (91.9%; n = 57). Glioma tended to affect the older age group than embryonal tumors (p value = 0.002). On last available follow-up, 29.5% were lost to follow-up, 36% were alive, and 34.6% were deceased. The median overall survival (OS) for all tumors was 47.6 months. Embryonal tumors had a better outcome than glioma (median OS 76.3 vs. 30.3 months, respectively; p value = 0.001). CONCLUSIONS: High-grade glioma affecting the cerebrum was the most common tumor among AYA age group and was associated with a less favorable outcome compared to embryonal tumors. More research is needed to address this special age group.

Disclosure of medical errors: physicians' knowledge, attitudes and practices (KAP) in an oncology center.

BACKGROUND: Between the need for transparency in healthcare, widely promoted by patient's safety campaigns, and the fear of negative consequences and malpractice threats, physicians face challenging decisions on whether or not disclosing medical errors to patients and families is a valid option. We aim to assess the knowledge, attitudes and practices (KAP) of physicians in our center regarding medical error disclosure. METHODS: This is a cross-sectional self-administered questionnaire study. The questionnaire was piloted and no major modifications were made. A day-long training workshop consisting of didactic lectures, short and long case scenarios with role playing and feedback from the instructors, were conducted. Physicians who attended these training workshops were invited to complete the questionnaire at the end of the training, and physicians who did not attend any training were sent a copy of the questionnaire to their offices to complete. To assure anonymity and transparency of responses, we did not query names or departments. Descriptive statistics were used to present demographics and KAP. The differences between response\s of physicians who received the training and those who did not were analyzed with t-test and descriptive statistics. The 0.05 level of significance was used as a cutoff measure for statistical significance. RESULTS: Eighty-eight physicians completed the questionnaire (55 attended training (62.50%), and 33 did not (37.50%)). Sixty Five percent of physicians were males and the mean number of years of experience was 16.5 years. Eighty-Seven percent (n = 73) of physicians were more likely to report major harm, compared to minor harm or no harm. Physicians who attended the workshop were more knowledgeable of articles of Jordan's Law on Medical and Health Liability (66.7% vs 45.5%, p-value = 0.017) and the Law was more likely to affect their decision on error disclosure (61.8% vs 36.4%, p-value = 0.024). CONCLUSION: Formal training workshops on disclosing medical errors have the power to positively influence physicians' KAP toward disclosing medical errors to patients and possibly promoting a culture of transparency in the health care system.

Innovative Surgical Planning in Resecting Soft Tissue Sarcoma of the Foot Using Augmented Reality With a Smartphone.

Augmented or hybrid reality is a display technology that combines the real world with the virtual world; it permits digital images of preoperative planning information to be combined with the surgeon's view of the real world. Augmented reality (AR) can increase the surgeon's intraoperative vision by providing virtual transparency of the real patient and has been applied to a wide spectrum of orthopedic procedures, such as tumor resection, fracture fixation, arthroscopy, and component's alignment in total joint arthroplasty. We present a case of a male patient who presented with pain in the medial aspect of his left foot after he underwent an incomplete mass excision elsewhere where it turned out to be synovial sarcoma. Because the mass was small, impalpable, and deeply positioned beneath both the plantar and the medial plantar aponeuroses, it was impossible to preoperatively decide a plan for resection. We opted to use the aid of AR in the form of an application using the camera of a smartphone. We were able to excise the tumor with negative surgical margins. On 12-month follow-up, the patient is in complete remission and has optimal mobility and functionality of his foot. In conclusion, AR holds great potential for use in the future of orthopedic surgical oncology. We emphasize using it via a handheld device that we found to be optimal for planning resection of the small and relatively fixed tumor. Based on our literature review, this is the first case describing the surgical planning in resecting an impalpable synovial sarcoma of the foot using AR technology.