World Health Organization Classification System Research Articles

Patterns and characteristics of traumatic dental injuries in children – a retrospective study in a dental hospital in Kolkata, India

Introduction and aim. Traumatic dental injuries (TDIs) in children can have aesthetic, functional, and psychological conse quences. This retrospective study aimed to investigate the epidemiology and patterns of TDIs in a hospital-based population. Material and methods. Patient records from the Department of pediatric and Preventive Dentistry in a dental college and hospital in Kolkata, India, were reviewed. A total of 381 patients, aged 1 to 12 years, who presented one or more traumatized an terior teeth between September 2018 and August 2022, were included. Data on age, sex, mechanism of injury, type of trauma, dentition type, and number of affected teeth were collected. The World Health Organization classification system was used to classify the type of dental trauma. Results. Among the study population, there were 235 males (61.7%) and 146 females (38.3%), with a mean age of 7.67±3.87 years. Falls were the most common cause of trauma (52.2%). The maxillary central incisors were the most frequently affected teeth in both primary and permanent dentitions. Conclusion. Falls were the most common cause of trauma and the maxillary central incisors were the most commonly affected teeth in children.

L1 Cell Adhesion Molecule (L1CAM) Expression and Molecular Alterations Distinguish Low-Grade Oncocytic Tumor From Eosinophilic Chromophobe Renal Cell Carcinoma

Renal low-grade oncocytic tumor (LOT) is a recently recognized renal cell neoplasm designated within the “other oncocytic tumors” category in the 2022 World Health Organization classification system. Although the clinicopathologic, immunohistochemical, and molecular features reported for LOT have been largely consistent, the data are relatively limited. The morphologic overlap between LOT and other low-grade oncocytic neoplasms, particularly eosinophilic chromophobe renal cell carcinoma (E-chRCC), remains a controversial area in renal tumor classification. To address this uncertainty, we characterized and compared large cohorts of LOT (n = 67) and E-chRCC (n = 69) and revealed notable differences between the 2 entities. Clinically, LOT predominantly affected women, whereas E-chRCC showed a male predilection. Histologically, although almost all LOTs were dominated by a small-nested pattern, E-chRCC mainly showed solid and tubular architectures. Molecular analysis revealed that 87% of LOT cases harbored mutations in the tuberous sclerosis complex (TSC)-mTOR complex 1 (mTORC1) pathway, most frequently in MTOR and RHEB genes; a subset of LOT cases had chromosomal 7 and 19q gains. In contrast, E-chRCC lacked mTORC1 mutations, and 60% of cases displayed chromosomal losses characteristic of chRCC. We also explored the cell of origin for LOT and identified L1 cell adhesion molecule (L1CAM), a collecting duct and connecting tubule principal cell marker, as a highly sensitive and specific ancillary test for differentiating LOT from E-chRCC. This distinctive L1CAM immunohistochemical labeling suggests the principal cells as the cell of origin for LOT, unlike the intercalated cell origin of E-chRCC and oncocytoma. The ultrastructural analysis of LOT showed normal-appearing mitochondria and intracytoplasmic lumina with microvilli, different from what has been described for chRCC. Our study further supports LOT as a unique entity with a benign clinical course. Based on the likely cell of origin and its clinicopathologic characteristics, we propose that changing the nomenclature of LOT to “Oncocytic Principal Cell Adenoma of the Kidney” may be a better way to define and describe this entity.

Obese patients with CEAP (clinical, etiology, anatomy, pathophysiology) C2 and C3 disease show enhanced symptom improvement after endovenous thermal ablation

ObjectiveEndovenous thermal ablation (EVTA) is a prevalent treatment option for patients with severe venous disease. However, the decision to intervene for patients with less severe disease (CEAP [clinical, etiology, anatomy, pathophysiology] C2 and C3) is less clear and becomes further complicated for patients with obesity, a pathology known to increase venous disease symptom severity. Therefore, the objective of this study was to use the Society for Vascular Surgery Vascular Quality Initiative database to evaluate outcomes after EVTA in obese patients with CEAP C2 and C3 venous insufficiency. MethodsUsing the Society for Vascular Surgery Vascular Quality Initiative database, we retrospectively analyzed the initial procedure of all patients with a CEAP clinical class of C2 or C3 who underwent EVTA from January 2015 to December 2021. Patients were grouped by obesity, defined as a body mass index of ≥30 kg/m2. The primary outcome was the change in venous clinical severity score (VCSS) from the procedure to the patient's initial follow-up. The secondary outcomes included the change in patient-reported outcomes at follow-up via the HASTI (heaviness, achiness, swelling, throbbing, itching) score, incidence of follow-up complications, and recanalization of treated veins. The change in the VCSS and HASTI score were analyzed using Student t tests, and complications and recanalization were assessed using the Fisher exact test. Significant outcomes were confirmed by multiple variable logistic regression. The remaining significant variables were then analyzed, with obesity categorized using the World Health Organization classification system to analyze how increasing obesity levels affect outcomes. ResultsThere were 8146 limbs that met the inclusion criteria, of which 5183 (63.6%) were classified as nonobese and 2963 (36.4%) as obese. Obesity showed no impact on improvement in the VCSS (−3.29 vs −3.35; P = .408). Obesity was found to be associated with a larger improvement in overall symptoms, as evidence by a greater improvement in the HASTI score (−7.24 vs −6.62; P < .001). Obese limbs showed a higher incidence of superficial phlebitis (1.5% vs 0.7%; P = .001), but no difference was found in recanalization or any other complication. ConclusionsThese data suggest that obese patients with CEAP clinical class C2 or C3 experience greater improvement in their perceived symptoms after EVTA with little difference in clinical improvement and complications compared with nonobese patients. Although obesity has been associated with increased severity of venous disease symptoms, obese patients are able to derive significant relief after treatment during the short term and may experience greater relief of symptoms than nonobese patients when treated at more mild disease presentations.

2021 WHO Classification of Lung Cancer: Molecular Biology Research and Radiologic-Pathologic Correlation.

The 2021 World Health Organization (WHO) classification system for thoracic tumors (including lung cancer) contains several updates to the 2015 edition. Revisions for lung cancer include a new grading system for invasive nonmucinous adenocarcinoma that better reflects prognosis, reorganization of squamous cell carcinomas and neuroendocrine neoplasms, and description of some new entities. Moreover, remarkable advancements in our knowledge of genetic mutations and targeted therapies have led to a much greater emphasis on genetic testing than that in 2015. In 2015, guidelines recommended evaluation of only two driver mutations, ie, epidermal growth factor receptor (EGFR) mutations and anaplastic lymphoma kinase (ALK) fusions, in patients with nonsquamous non-small cell lung cancer. The 2021 guidelines recommend testing for numerous additional gene mutations for which targeted therapies are now available including ROS1, RET, NTRK1-3, KRAS, BRAF, and MET. The correlation of imaging features and genetic mutations is being studied. Testing for the immune biomarker programmed death ligand 1 is now recommended before starting first-line therapy in patients with metastatic non-small cell lung cancer. Because 70% of lung cancers are unresectable at patient presentation, diagnosis of lung cancer is usually based on small diagnostic samples (ie, biopsy specimens) rather than surgical resection specimens. The 2021 version emphasizes differences in the histopathologic interpretation of small diagnostic samples and resection specimens. Radiologists play a key role not only in evaluation of tumor and metastatic disease but also in identification of optimal biopsy targets. ©RSNA, 2024 Test Your Knowledge questions in the supplemental material and the slide presentation from the RSNA Annual Meeting are available for this article.

Pediatric spinal ependymoma with chromothripsis of chromosome 6: a case report and review of the literature

BackgroundEpendymomas are the third most common central nervous system tumor in the pediatric population; however, spinal ependymomas in children are rare. Ependymomas affecting the spinal cord most frequently occur in adults of 20–40 years of age. The current World Health Organization classification system for ependymomas is now composed of ten different entities based on histopathology, location, and molecular studies, with evidence that the new classification system more accurately predicts clinical outcomes.Case presentationWe present the case of a 16-year-old Caucasian female patient with a history of type 2 neurofibromatosis with multiple schwannomas, meningioma, and spinal ependymoma. Chromosome analysis of the harvested spinal ependymoma tumor sample revealed a 46,XX,−6,+7,−22,+mar[16]/46,XX[4] karyotype. Subsequent OncoScan microarray analysis of the formalin-fixed paraffin-embedded tumor sample confirmed + 7, −22 and clarified that the marker chromosome represents chromothripsis of the entire chromosome 6 with more than 100 breakpoints. Fluorescent in situ hybridization and microarray analysis showed no evidence of MYCN amplification. The final integrated pathology diagnosis was spinal ependymoma (central nervous system World Health Organization grade 2 with no MYCN amplification.ConclusionThis case adds to the existing literature of pediatric patients with spinal ependymomas and expands the cytogenetic findings that may be seen in patients with this tumor type. This case also highlights the value of cytogenetics and microarray analysis in solid tumors to provide a more accurate molecular diagnosis.

The clinical, molecular, and prognostic features of the 2022 WHO and ICC classification systems for myelodysplastic neoplasms

Myelodysplastic neoplasms (MDS) are clonal disorders of bone marrow failure exhibiting a variable risk of progression to acute myeloid leukemia. MDS exhibit certain prognostic genetic or cytogenetic abnormalities, an observation that has led to both the pathologic reclassification of MDS in the 2022 World Health Organization (WHO) and International Consensus Classification (ICC) systems, as well as to an updated prognostic schema, the Molecular International Prognostic Scoring System (IPSS-M). This single-institution study characterized the molecular patterns and clinical outcomes associated with the 2022 WHO and ICC classification schemas to assess their clinical utility. Strikingly, with the exception of one individual, all 210 patients in our cohort were classified into analogous categories by the two pathologic/diagnostic schemas. Most patients (70%) were classified morphologically while the remaining 30% had genetically classified disease by both criteria. Prognostic risk, as assessed by the IPSS-M score was highest in patients with MDS with biallelic/multi-hit TP53 mutations and lowest in pts with MDS-SF3B1. Median leukemia-free survival (LFS) was shortest for those with MDS with biallelic/multi-hit TP53 (0.7 years) and longest for those with MDS with low blasts (LFS not reached). These data demonstrate the clear ability of the 2022 WHO and ICC classifications to organize MDS patients into distinct prognostic risk groups and further show that both classification systems share more similarities than differences. Incorporation of the IPSS-M and IPSS-R features provide additive prognostic and survival components to both the WHO and ICC classifications, which together enhance their utility for evaluating and treating MDS patients.

Investigation of Age-Specific Differences in Survival and Cause of Death Among Diffuse Large B-Cell Lymphoma Patients with and without HIV in the Modern Era of Antiretroviral Therapy: A Population-Based Analysis from 2010 to 2017

Introduction Despite improved prognosis with combined antiretroviral therapy (cART) among patients with human immunodeficiency virus (PWHIV) diagnosed with diffuse large B-cell lymphoma (DLBCL), population-based studies suggest inferior survival rates compared to patients without HIV. Uncertainty remains about the persistence of this survival difference across age groups. Examining outcomes across ages may help identify higher-risk groups in the current era of cART, providing insights for designing and conducting age-specific interventions. Methods A retrospective population-based cohort was conducted using US population-based cancer registry data among patients aged 15-79 years with newly diagnosed DLBCL from 2010 to 2017, with follow-up through 2019. The 2010-2017 period reflects the availability of integrase inhibitors and the introduction of distinct lymphoma subtypes to the World Health Organization classification system in cancer registries. HIV status was reported at lymphoma diagnosis. Patients were classified into three age groups: adolescents and young adults (AYAs, 15-39 years), adults (40-59 years), and older adults (60-79 years). Overall survival (OS) was defined from diagnosis to death of any cause. Probabilities were estimated using the Kaplan-Meier method and compared with the log-rank test. Multivariable Cox regression models were fitted to assess all-cause mortality, adjusting for cancer stage, race/ethnicity, sex, year of diagnosis, and age. We estimated the cumulative incidence of infections and HIV-related deaths using the competing risk method. Results Patient characteristics: A total of 26,905 DLBCL patients were identified (1,030 PWHIV and 25,758 patients without HIV). Compared to patients without HIV, PWHIV were younger (median age at diagnosis 48 years vs. 62 years), predominantly male (85% vs. 56%), more likely to be non-Hispanic Black (38% vs. 8%) and Hispanic (24% vs. 18%), and more likely to have advanced-stage disease at diagnosis (71% vs. 56%) (Table 1). OS in DLBCL PWHIV vs. patients without HIV:Within each age group, OS was significantly lower among PWHIV with DLBCL compared to patients without HIV(Table 1 and Figure 1). With a median follow-up of 67 months, 5-year OS was particularly low among AYAs (53% vs. 87%, p&amp;lt;0.0001) and adults (48% vs. 74%, p&amp;lt;0.0001). Similarly, older adults experienced lower 5-year OS rates (45% vs. 57%, p&amp;lt;0.0001). Multivariable Cox models suggest that PWHIV has an increased risk of all-cause mortality within each age group (Figure 1). OS in different age groups by HIV status: For PWHIV, age was not a significant factor for increased all-cause mortality, with 5-year OS rates ranging from 45% in adults to 53% in AYAs (p=0.170). Conversely, in DLBCL patients without HIV, AYAs had superior 5-year OS rates (87%, p&amp;lt;0.001) compared to adults (74%) and older adults (57%). Cause of death by HIV status: Among both PWHIV and without HIV, lymphoma/leukemia-related mortality was the most common cause of death reported in death certificates (47% and 73% respectively). However, the 5-year cumulative incidence of infection mortality was higher among PWHIV (range 3-4% across age groups) compared to patients without HIV (1% across age groups). For PWHIV, other HIV-related mortality had a 5-year cumulative incidence ranging from 9% to 14% across age groups. Results stratified by cancer stage (early- or advanced-stage disease) were consistent with those presented above. Conclusion This study highlights worse outcomes among PWHIV diagnosed with DLBCL compared to patients without HIV within each age group at the population level in the modern era of cART, notably among AYAs. Unlike the findings in DLBCL patients without HIV, age was not significantly associated with mortality among PWHIV with DLBCL. Future studies should explore age-specific demographic, biological, or healthcare system factors mediating these outcome disparities. The considerable HIV-related mortality suggests refining HIV surveillance approaches among PWHIV with DLBCL in prospective studies or clinical trials. Identifying the optimal integration of chemotherapy and cART may improve outcomes in this vulnerable population.

Granularity in disease classification impacts survival prediction in advanced systemic mastocytosis: A single institution study of 329 informative cases.

The World Health Organization (WHO) classification system categorizes advanced systemic mastocytosis (SM-Adv) into aggressive SM (ASM), mast cell leukemia (MCL), and SM with associated hematological neoplasm (SM-AHN). By contrast, the International Consensus Classification (ICC) requires "immature" MC cytomorphology for the diagnosis of MCL and limits SM-AHN to myeloid neoplasms (SM-AMN). The current study includes 329 patients with SM-Adv (median age 65 years, range 18-88; males 58%): WHO subcategories SM-AHN (N = 212; 64%), ASM (N = 99; 30%), and MCL (N = 18; 6%); ICC subcategories SM-AMN (N = 190; 64%), ASM (N = 99; 33%), and MCL (N = 9; 3%); WHO-defined MCL with "mature" MC cytomorphology and SM-AHN associated with lymphoid neoplasms were operationally labeled as "MCL-mature" (N = 9) and SM-ALN (N = 22), respectively, and distinguished from ICC-defined MCL and SM-AMN. Multivariable analysis that included the Mayo alliance risk factors for survival in SM (age >60 years, anemia, thrombocytopenia, increased alkaline phosphatase) revealed more accurate survival prediction with the ICC versus WHO classification order: (i) survival was significantly worse with MCL-immature versus MCL-mature (hazard ratio [HR] 15; p < .01), (ii) prognostic distinction between MCL and SM-AHN/AMN was confirmed in the context of ICC (HR 9.3; p < .01) but not WHO classification order (p = .99), (iii) survival was similar between MCL-mature and SM-AMN (p = .18), and (iv) SM-AMN (HR 1.7; p < .01) but not SM-ALN (p = .37) was prognostically distinct from ASM. The current study provides evidence for the independent prognostic contribution of both the ICC system for SM-Adv and the Mayo alliance risk factors for survival in SM.

Bladder Neoplasia in Pediatric Patients-A Single-Center Experience Including a Case Series.

Objective: Bladder lesions like urothelial carcinoma are rare in the first two decades of life. A biopsy of the bladder or urinary cytological examination is seldom required. Gross painless hematuria is the most relevant clinical syndrome. Methods: A retrospective analysis of surgical pathology records collected between 1984 and 2014 at our institution was performed in a search for cases of urothelial neoplasms originating within the urinary bladder in pediatric patients. Diagnoses were confirmed based on pathologic examination using the 2004 World Health Organization (WHO) classification system. We selected keywords such as bladder neoplasia, bladder lesion, urothelial neoplasia, rhabdomyosarcoma, and children. In addition, we describe clinical presentation and diagnostic procedures as well as treatment and follow-up of two patients. A review of the literature was performed to analyze recommendations concerning diagnostic staging, treatment, and follow-up examinations as well as surveillance of urothelial tumors in the pediatric population. Results: Screening the pathology database of the Institute of Medical Genetics and Pathology of the University Hospital Basel between 1988 and 2014 yielded 287 samples involving the urinary bladder, 110 autopsies, 135 biopsies, and 42 cytology specimens. Of these, most samples originated from malformations and inflammation. Only five were tumors: two were urothelial tumors and three were rhabdomyosarcomas. The majority of specimens comprised resections of the diverticula or distal ureter. Our case reports include two patients with a urothelial tumor. Among the urothelial tumors, one was a papillary urothelial neoplasm of low malignant potential (PUNLMP). Painless hematuria was the directing clinical symptom. The tumor was investigated by FISH, and a 9p21 deletion was found. The second tumor-like lesion was a fibroepithelial polyp arising from the bladder neck. Conclusions: Bladder tumors in children are rare and mostly consist of urothelial and mesenchymal neoplasms. Rhabdomyosarcoma is the most common malignant bladder tumor in childhood. Similar to adult urothelial neoplasms, the loss of 9p21 is also implicated in urothelial neoplasms in childhood. Despite an increasing number of case reports and small series published within the last 2 decades, general treatment protocols including recommendations for staging, tumor markers, and follow-up examinations are still not yet available for this tumor entity in the pediatric population.

Assessment of the nutritional status of school children in Galle Municipality area: A comparison between different body mass index classification systems

Introduction: Asian populations are at an increased risk of cardiometabolic disorders due to higher fat mass at a relatively lower body mass index (BMI). Several discrepancies have been reported in the BMI classification systems currently in practice to assess the nutritional status among children. Aim of this study was to compare the different international BMI based classification systems to identify the nutritional status in a cohort of Sri Lankan children.Methods: A cross sectional study was conducted on 833 (48.37% boys) school children aged 5-15 years in Galle Municipality area, Sri Lanka. The nutritional status of each child was defined according to five BMI classification systems: World Health Organization (WHO), Centers for Disease Control and Prevention(CDC), and International Obesity Task Force (IOTF), Indian and Sri Lankan systems. The agreement between them was tested using Cohen’s kappa coefficient.Results: Prevalence of under-weight, overweight and obesity of children, as determined by the WHO, CDC, IOTF, Indian and the Sri Lankan classification systems showed significant differences. The WHO classification system had the best agreement (k=0.649) with the Indian classification system. A wide difference between the Sri Lankan classification system (24.37%) and the Indian (9.60%) and WHO (5.04%) classification systems in defining obesity were observed.Conclusion &amp; recommendations: A wide difference between different BMI classification systems when determining the nutritional status in children emphasise the importance of developing a country-specific classification system to identify the nutritional status among Sri Lankan children.

Low-Grade Uterine Leiomyosarcoma Is Highly Sensitive to Hormonal Treatment.

According to the World Health Organization classification system, uterine leiomyosarcomas (ULMS) are high-grade. A diagnosis of smooth-muscle tumors of uncertain malignant potential (STUMP) is made when Stanford Criteria for ULMS are not met. When a STUMP recurs, the tumor is diagnosed as ULMS and medical treatment is the same as for ULMS. In recent years, some sarcoma centers valued the less aggressive clinical behavior of several recurring STUMP and, given their expression of estrogen and progesterone receptors, started to treat them with hormonal therapy. This was a retrospective cohort analysis conducted at three referral centers joining the Leiomyosarcoma Foundation Roundtable. We selected all cases of uterine smooth muscle tumors consistent with STUMP and treated with hormonal therapy. 27 consecutive patients were identified. Median age at diagnosis was 43 years. Stage was IA-IB in more than 70% of patients. In these patients, median time to relapse was 62 months. Sites of first relapses were mostly pelvis and peritoneum (76%). After a median follow-up of 49 months, 14 patients (52%) had a partial response while 10 (37%) had a minor response or stable disease. Median time to progression was not reached. We observed a response or long-term stability rate on hormonal therapy in the 90% range; in all cases the time to relapse was significantly longer than in ULMS and in most cases the relapse was abdominal. On the basis of these findings, we conclude that a proportion of patients with uterine smooth muscle neoplasms actually present with a "low-grade ULMS."

Pregnancy risk and contraception among reproductive-age women with rheumatic heart disease attending care at a tertiary cardiac center in Tanzania: a hospital-based cross-sectional study

BackgroundRheumatic heart disease (RHD) remains prevalent in the developing world and reproductive-age women are disproportionately affected. It is among the common est cardiac diseases during pregnancy and is associated with poor pregnancy outcomes. Despite its importance among reproductive-age women, there are no local studies that characterize the clinical characteristics, risk of poor pregnancy outcomes and contraception which represents one effective way to prevent unplanned pregnancies in this population.MethodsThis was a hospital-based descriptive cross-sectional study. Non-pregnant reproductive-age women with echocardiographically diagnosed RHD were consecutively recruited from in- and out-patients units of the Jakaya Kikwete Cardiac Institute (JKCI). A clinical research form was used to gather socio-demographic, clinical characteristics, contraception status and echocardiographic information. The maternal/pregnancy risk class was determined using the modified World Health Organization (WHO) classification of maternal risk.ResultsTwo hundred thirty-eight women of reproductive age with RHD were recruited. The median age (range) was 36 years (15–49). Two-thirds were dyspneic on moderate exertion and 17.2% had New York Heart Association class IV heart failure. A quarter had atrial fibrillation/flutter. On echocardiography, mitral regurgitation was the most common valvular lesion (68.1%), followed by mitral stenosis (66.8%), and 12.2% of participants had reduced left ventricular ejection fraction. Two-thirds (66%) had a high pregnancy risk (class IV) based on the modified WHO classification system. The proportion of participants using contraception was 7.1% and common methods were: bilateral tubal ligation 5 of 17 (29.4%) and hormonal implant (4 of 17). The most common reason for the choice of a method was safety, 10 out of 17 (58.8%).ConclusionThe majority of women of reproductive age with RHD in our hospital cohort are at the highest pregnancy risk based on the modified WHO classification and a very small proportion of them are on contraception. These results call for action among clinicians to offer counselling to these patients, educating them on their risk and offering appropriate contraception advice while waiting for definitive interventions.

TERT mutations-associated alterations in clinical characteristics, immune environment and therapy response in glioblastomas

Objective TERTis the most frequently mutated gene in adult glioblastomas (GBMs) defined by the 2021 World Health Organization classification system. The present study aims to explore differences in clinical characteristics and immune microenvironment between TERT mutant and wild-type GBM.MethodsThree GBM-related cohorts consisting of 205 GBM patients in our cohort, 463 GBM patients without immune checkpoint inhibitor(ICI) therapy and 1465 tumour patients (including 92 GBM cases) receiving ICI treatment in the MSK cohort were included. Retrospective analysis and immunohistochemistry assay were used for investigating the local (including tumour cells, local immune cells, and seizures) and systemic (including circulating immune cells, coagulation-related functions, and prognosis) effects of TERT mutations. Besides, differences in genetic alterations and immunotherapy responses between TERT mutant and wild-type GBMs were also explored.ResultsWe found that TERT mutant and wild-type GBMs possessed similar initial clinic symptoms, circulating immune microenvironment and immunotherapy response. With respect to that in TERT wild-type GBMs, mutations in TERT resulted in higher levels of tumour-infiltrating neutrophils, prolonged coagulation time, worse chemotherapy response and poorer overall survival.ConclusionMutations in TERT alter the local immune environment and decrease the sensitivity of GBM to chemotherapy.

Phenotypic clues that predict underlying cytogenetic/genetic abnormalities in myeloid malignancies: A contemporary review.

Precise subclassification of myeloid malignancies per the World Health Organization (WHO) classification system and the International Consensus Classification of Myeloid Neoplasms and Acute Leukaemias (ICC) requires investigation and documentation of the presence of cytogenetic and/or molecular genetic changes. These ancillary studies not only help in diagnosis, but also the prognosis of disease; however, they take time to be completed. In contrast, morphological evaluation of material from the blood and bone marrow specimens of cases where myeloid malignancies are suspected is usually completed quickly. Cytomorphological assessment may predict genetic changes and can be helpful in triaging acuity. This is especially true in haematological emergencies such as acute promyelocytic leukaemia (APL), where prompt APL-specific therapy can be life changing. Similarly, some morphological clues may help identify core binding factor leukaemias where a diagnosis of acute myeloid leukaemia (AML) could be rendered without reaching the 20% blast cutoff with immediate treatment-decision implications, or even a subset of cases of AML with FLT3 ITD/NPM1 mutation(s) which show characteristic features. Even though FISH/cytogenetics and/or PCR are still required for establishing the final diagnosis, evaluation for the presence of specific cytomorphological features that help predict genetic changes can be a useful tool to help guide early therapy.

Smooth Muscle Tumors of the Uterus at MRI: Focus on Leiomyomas and FIGO Classification.

Leiomyomas are smooth muscle tumors of the uterus and are the most common uterine neoplasm. Although leiomyomas are usually asymptomatic, they can manifest with symptoms such as pain or uterine bleeding. Leiomyomas are classified on the basis of their anatomic location and morphology. Localization of leiomyomas relative to the endometrium, myometrium, and uterine serosa with use of the International Federation of Gynecology and Obstetrics (FIGO) classification system is helpful for guiding management in symptomatic patients. The FIGO system is a practical and universally accepted approach for classifying leiomyomas to guide radiologists and clinicians in deciding management. The MRI appearance of conventional leiomyomas is related to their tissue contents of smooth muscle and fibrous tissue and is well established. The MRI features of some leiomyoma subtypes and forms of degeneration also have been described. Other smooth muscle tumors of the uterus recognized in the 2020 World Health Organization classification system include intravenous leiomyomatosis, smooth muscle tumors of uncertain malignant potential, and metastasizing leiomyoma. At the far end of the spectrum are leiomyosarcomas, which are frankly malignant and therefore must be managed accordingly. Although MRI features that suggest a diagnosis of leiomyosarcoma have been proposed, these features overlap with those of some leiomyoma subtypes and degeneration. © RSNA, 2023 See the invited commentary by Fennessy and Gargiulo in this issue. Online supplemental material and the slide presentation from the RSNA Annual Meeting are available for this article. Quiz questions for this article are available through the Online Learning Center.

International Opinions on Grading of Urothelial Carcinoma: A Survey Among European Association of Urology and International Society of Urological Pathology Members

BackgroundGrade of non–muscle-invasive bladder cancer (NMIBC) is an important prognostic factor for progression. Currently, two World Health Organization (WHO) classification systems (WHO1973, categories: grade 1–3, and WHO2004 categories: papillary urothelial neoplasm of low malignant potential [PUNLMP], low-grade [LG], high-grade [HG] carcinoma) are used. ObjectiveTo ask the European Association of Urology (EAU) and International Society of Urological Pathology (ISUP) members regarding their current practice and preferences of grading systems. Design, setting, and participantsA web-based, anonymous questionnaire with ten questions on grading of NMIBC was created. The members of EAU and ISUP were invited to complete an online survey by the end of 2021. Thirteen experts had previously answered the same questions. Outcome measurements and statistical analysisThe submitted answers from 214 ISUP members, 191 EAU members, and 13 experts were analyzed. Results and limitationsCurrently, 53% use only the WHO2004 system and 40% use both systems. According to most respondents, PUNLMP is a rare diagnosis with management similar to Ta-LG carcinoma. The majority (72%) would consider reverting back to WHO1973 if grading criteria were more detailed. Separate reporting of WHO1973-G3 within WHO2004-HG would influence clinical decisions for Ta and/or T1 tumors according the majority (55%). Most respondents preferred a two-tier (41%) or a three-tier (41%) grading system. The current WHO2004 grading system is supported by a minority (20%), whereas nearly half (48%) supported a hybrid three- or four-tier grading system composed of both WHO1973 and WHO2004. The survey results of the experts were comparable with ISUP and EAU respondents. ConclusionsBoth the WHO1973 and the WHO2004 grading system are still widely used. Even though opinions on the future of bladder cancer grading were strongly divided, there was limited support for WHO1973 and WHO2004 in their current formats, while the hybrid (three-tier) grading system with LG, HG-G2, and HG-G3 as categories could be considered the most promising alternative. Patient summaryGrading of non–muscle-invasive bladder cancer (NMIBC) is a matter of ongoing debate and lacks international consensus. We surveyed urologists and pathologists of European Association of Urology and International Society of Urological Pathology on their preferences regarding NMIBC grading to generate a multidisciplinary dialogue. Both the “old” World Health Organization (WHO) 1973 and the “new” WHO2004 grading schemes are still used widely. However, continuation of both the WHO1973 and the WHO2004 system showed limited support, while a hybrid grading system composed of both the WHO1973 and the WHO2004 classification system may be considered a promising alternative.

Local and systemic effects of IDH mutations on primary glioma patients.

Adult gliomas are divided into isocitrate dehydrogenase (IDH) wild-type and IDH mutant subtypes according to the new 2021 World Health Organization classification system. However, the local and systemic effects of IDH mutations on primary glioma patients are not well illustrated. Retrospective analysis, immune-cell infiltration analysis, meta-analysis, and immunohistochemistry assay were applied in the present study. The results from our cohort showed that IDH mutant gliomas own a lower proliferating rate compared to that in wild-type gliomas. Patients with mutant IDH exhibited a higher frequency of seizures in both our cohort and the cohort from the meta-analysis. Mutations in IDH result in lower levels of intra-tumour but higher levels of circulating CD4+ and CD8+ T lymphocytes. Levels of neutrophils in both intra-tumour and circulating blood were lower in IDH mutant gliomas. Moreover, IDH mutant glioma patients receiving radiotherapy in combination with chemotherapy exhibited better overall survival with respect to radiotherapy alone. Mutations in IDH alters the local and circulating immune microenvironment, and increases the sensitivity of tumour cell to chemotherapy.

HF01-17 MOBILIZE THE TROOPS! MILITARY INVOLVEMENT IN THE HISTORY OF TESTICULAR CANCER

You have accessJournal of UrologyCME1 Apr 2023HF01-17 MOBILIZE THE TROOPS! MILITARY INVOLVEMENT IN THE HISTORY OF TESTICULAR CANCER Kit Yuen, Alexis Steinmetz, Phillip Rappold, and Ronald Rabinowitz Kit YuenKit Yuen More articles by this author , Alexis SteinmetzAlexis Steinmetz More articles by this author , Phillip RappoldPhillip Rappold More articles by this author , and Ronald RabinowitzRonald Rabinowitz More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000003243.17AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Testicular cancer is a rare malignancy that has become a manageable disease, assisted by the study of the large number of such tumors found in World War II (WWII) soldiers. METHODS: A literature review of textbook chapters, manuscripts, online texts, and peer-reviewed articles was performed for historical descriptions of testis tumors and its treatments. RESULTS: Saint Donat in 1696 described a testis teratoid tumor as a “fetal monstrosity”. Testicular tumor pathology was first documented by British physicians Percival Pott and Astley Cooper in the mid to late 1700s. In 1794, Scottish surgeon Benjamin Bell described the presentation of a testis tumor as a firm painless fleshy enlargement. If treatment with mercury failed, orchiectomy was done. It wasn’t until 1910 that French urologist Maurice Chevassu reported radical orchiectomy. Diagnosis, staging, and evaluation of response to therapy was propelled forward with the discovery of tumor marker human chorionic gonadotropin in 1927. Radiation was introduced to treat testicular cancer in the 1940s. Testis cancer, a tumor of young men, was found in high frequency in the military. Prior to WWII, case series of more than 50 subjects were rare. From examining more than 900 soldiers with testis tumors, the histopathology was established in 1946. In 1950, two large military studies reported that RPLND improved survival compared to orchiectomy alone. In 1952, a modified classification system was published by Frank Dixon and Robert Moore in the first Armed Forces Institute of Pathology (AFIP) tumor atlas. Fathollah Mostofi, along with other military pathologists, contributed greatly to the field; in 1972 he led a group to devise a standardized World Health Organization (WHO) classification system. Until the advent of platinum combination chemotherapy, metastatic disease was bleak and uniformly fatal, with a 90% mortality rate within 1 year. Chemosensitivity was first demonstrated in the 1950s with actinomycin combined with methotrexate. In 1974, a regimen of cisplatin, vinblastine with bleomycin was introduced by Lawrence Einhorn and John Donahue at Indiana University for advanced testicular cancer with greatly improved survival. RPLND templates were further refined with lymph node mapping studies, which decreased morbidity significantly. CONCLUSIONS: World War II brought together young men with testis cancer, a rare disease that ignited fifty years of basic and clinical research resulting in one of the greatest oncological success stories. The military contributed greatly to the diagnosis, histopathology and management of testicular cancer. Source of Funding: N/A © 2023 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 209Issue Supplement 4April 2023Page: e261 Advertisement Copyright & Permissions© 2023 by American Urological Association Education and Research, Inc.MetricsAuthor Information Kit Yuen More articles by this author Alexis Steinmetz More articles by this author Phillip Rappold More articles by this author Ronald Rabinowitz More articles by this author Expand All Advertisement PDF downloadLoading ...

Clinical, Hematological, and Cytogenetic Profile of Myelodysplastic Syndromes in Adults: A Three-year Cross-sectional Study.

Myelodysplastic syndromes (MDS) are a group of clonal hematological disorders with a diverse clinico-hematological profile. Studies in India have shown a different biology from the West. This study aimed to assess the clinicopathologic profile of MDS patients, classify them according to the World Health Organization classification system, categorize them into International Prognostic Scoring System (IPSS) and the revised IPSS prognostic subgroups, and evaluate the treatment outcome. A cross-sectional study was conducted on 48 patients diagnosed with MDS from January 2017 to December 2019 from Rajagiri Hospital, India. Clinical, hematological, and cytogenetic features were analyzed. The patients were stratified according to the IPSS and revised IPSS and were followed-up for a minimum of six months. The patients most affected were those in the seventh decade of life. We found a slight female preponderance and a mean age of 57.5 years in females and 67.7 years in males. Anemia was the most common manifestation of MDS. On the other hand, thrombocytopenia was found to be the least common cytopenia. MDS with multilineage dysplasia was the most common subtype. Cytogenetic abnormalities were seen in 29.5% of cases. Most of the patients were in the low-risk prognostic categories. Our patients were older when compared to those of other Indian studies, with most in the low-risk categories which were like Western data.

Histological subtypes of hepatocellular carcinoma: Their clinical and prognostic significance

Assigning a hepatocellular carcinoma (HCC) to an appropriate subtype is important because this guarantees the diagnosis and treatment and allows decisions regarding the prognosis of the patient. HCC subtyping is usually based on the World Health Organization (WHO) classification and the 2019 fifth edition is the latest version. However, the WHO classification system is still in evolution and has limited clinical relevance. We aimed to evaluate the clinical relevance of HCC subtyping and to reappraise some of the major subtypes of HCC. Our archived cases (n = 589) were reclassified according to the 2019 WHO system. The percentage of each subtype was mostly similar to that in the WHO classification. However, on the contrary to the 2019 WHO system, clear cell type HCC was associated with more frequent recurrence or metastasis. Meanwhile, macrotrabecular massive HCC was related to poor prognosis as demonstrated in the 2019 WHO system and should be described in the pathology report. For steatohepatitic HCC, there is a debate on whether it is a true subtype because the steatohepatitis morphology may or may not be present in the background liver. In our study, 44 % of steatohepatitic HCCs (n = 19/43) presented underlying steatohepatitis. Additionally, the background cirrhosis did not influence survival in the HCC patients, although the 2019 WHO system indicates the presence of cirrhosis as a poor prognostic factor. In conclusion, although it is not perfect yet, HCC subtyping based on the 2019 WHO system provides valuable information to manage patients with HCC.