Diffuse leptomeningeal glioneuronal tumor in an 8-year-old girl: case report and review of the literature.

Diffuse leptomeningeal glioneuronal tumors (DLGNTs) are rare central nervous system tumors of childhood that were recently described as a new entity. DLGNTs usually manifest with symptoms related to increased intracranial pressure or spinal cord compression. The classic radiological feature is a widespread leptomeningeal enhancement that may involve the entire neuroaxis. Microscopic examination demonstrates oligodendroglial-like cells that are positive for OLIG2, MAP2, and S100 and negative for IDH-1. Anaplastic features occur in some cases. Molecularly, DLGNTs are characterized by chromosome arm 1p deletion and alteration of a mitogen-activated protein kinase (MAPK) pathway gene, most commonly BRAF-KIAA1549 fusion. There is no established grading system for these tumors, which may have an indolent or aggressive behavior. Treatment usually involves chemotherapy and radiation therapy.

Pineal anlage tumor: a case report and review of the literature.

PURPOSE: Pineal anlage tumors (PAT) are extremely rare malignant tumors in the pediatric population considered to be a variant of pineoblastomas. These tumors are characterized by a combination of neuroectodermal and heterologous ectomesenchymal components. PAT can be clinically aggressive and has a propensity to cerebrospinal fluid (CSF) dissemination. So far, only 13 cases of these tumors have been reported in the literature. CASE ARTICLE: In this article, we report the case of an anlage tumor in a 3-year-old girl with a rapidly aggressive course.

Medulloblastoma, WNT-activated/SHH-activated: clinical impact of molecular analysis and histogenetic evaluation.

PURPOSE: Medulloblastoma (MDB) is a small cell poorly differentiated embryonal tumor of the cerebellum, which more frequently compromises children. Overall prognosis is favorable, but dependent of stage, histopathological pattern and molecular group. Approximately 30% of the affected patients will die from the disease. WHO 2016 Classification of Tumors of the Central Nervous System (CNS) has been classified MDB into four principal groups: WNT-activated MDB, SHH-activated MDB, group 3 MDB, and group 4 MDB. WNT-activated MDB is associated to monosomy 6, CTNNB1, DDX3X and TP53 mutations, beta-catenin nuclear immunoexpression, and a better prognosis than SHH-activated MDB. DISCUSSION: WNT-activated tumors account approximately for 10% of cases of MDBs, and are thought to arise from cells in the dorsal brain stem/lower rhombic lip progenitor cells. SHH-activated MDB more frequently arises in the lateral hemispheres of the cerebellum, and clinical outcome in this group is variable. TP53-mutant SHHactivated MDB usually shows the large cell/anaplastic pattern, and can be related to MYCN amplification, GLI2 amplification and 17p loss. TP53-wildtype SHH-activated MDB is more commonly of desmoplastic/nodular morphology, and can be related to PTCH1 deletion and 10q loss. Gene expression and methylation profiling is the gold standard for defining molecular groups of MDB. In immunohistochemistry assays, anti-GAB1 antibody expression is positive in tumors showing SHH pathway activation or PTCH mutation, while positive immunoexpression for YAP1 antibody can be only found in WNT-activated and SHH-activated MDB.

The role of diabetes mellitus in the composition of coronary thrombi in patients presenting with acute ST-segment elevation myocardial infarction undergoing primary percutaneous coronary intervention.

BACKGROUND: Although diabetes mellitus (DM) is a predictor of poor outcomes in patients with ST-segment elevation myocardial infarction (STEMI), few studies have analyzed the impact of DM on the constituency of coronary thrombi. OBJECTIVES: Comparing morphologic and histopathologic aspects of coronary thrombi in STEMI patients with and without DM who underwent primary percutaneous coronary intervention. METHODS: All consecutive patients with STEMI admitted to our institution between April 2010 and December 2012 (n = 1,548) were considered for inclusion. Thrombus material was obtained by aspiration thrombectomy; morphologic and histopathologic aspects were assessed by 3 independent pathologists blinded to clinical characteristics and outcomes. Patients with DM were compared with those without DM. A sensitivity analysis was performed using a propensity score. RESULTS: During the study period, coronary thrombi material from 259 patients was obtained, of whom 19% (n = 49) had diabetes. Diabetic patients were older (P = .10), had a higher frequency of hypertension (P < .01) and dyslipidemia (P = .03), and had a trend to a longer time from the onset of chest pain to hospital arrival (P = .08). The number of retrieved fragments, the size of the thrombi and its composition (leukocytes, fibrin, and erythrocytes percent), and thrombus age and color were similar between patients with or without DM. There were also no statistically significant differences in thrombus constituency of the propensity score-matched patients (n = 92). CONCLUSIONS: In this study, morphologic and histopathologic constituency of coronary thrombi in the setting of a ST-elevation myocardial infarction was not significantly different between patients with or without DM. This finding was intriguing and deserves further investigation.

Diffuse parenchymal form of malakoplakia in renal transplant recipient: a case report.

Malakoplakia is an unusual chronic inflammatory disease related to prior urinary tract infection. It is characterized by the presence of macrophages with foamy cytoplasm exhibiting larger PAS positive inclusions that stain for calcium and iron. Malakoplakia affects renal allograft and is associated with severe morbidity. Herein, the authors report a new case of renal graft malakoplakia in a 23-year-old female patient. The patient received a living-related donor renal transplantation with a high immunological risk. Plasmapheresis and intravenous immunoglobulin (i.v. Ig) treatment, pre- and post-transplant, and induction with rabbit anti-thymocyte globulins were used due to presence of donor specific antibodies and positive B cross match by flow cytometry. The patient had an early urinary tract infection with a good outcome. On Day 36 post-transplant (PO), the patient returned to the clinic with fever, graft pain and acute renal dysfunction leading to hemodialysis. Escherichia coli (E. coli) was present in the blood and urine culture. At the time, the renal biopsy revealed numerous sheets of macrophages with foamy, eosinophilic cytoplasm showing several PAS positive granules and large inclusions that stained strongly with hematoxylin, calcium (von Kossa method) and iron (Prussian blue). The patient was diagnosed with malakoplakia related to a kidney transplant. Despite prolonged treatment with antibiotics, determined by a susceptibility test, the patient did not recover renal function and remained on dialysis.

Myocardial protection: comparing histological effects of single-dose cardioplegic solutions-study protocol for a secondary analysis of the CARDIOPLEGIA trial.

Background: Myocardial protection is crucial for successful cardiac surgery, as it prevents heart muscle damage that can occur during the procedure. Prolonged hypoxia without proper protection can lead to adenosine triphosphate consumption, microvilli loss, blister formation, and edema. Custodiol, del Nido, and modified del Nido are single-dose cardioplegic solutions with proven safety and significance in modern surgery. While each has been independently assessed for patient outcomes, limited research directly compares them. This study aims to compare their myocardial protection using histological analysis. Methods: In a double-blind clinical trial, at least 90 patients will be randomly assigned to receive one of the three cardioplegic solutions. Myocardial biopsies will be collected before cardiopulmonary bypass and 15 minutes after reperfusion. The surgical, anesthetic and perfusion techniques will be the same for all patients, following the Institution's standard protocols. Discussion: The ideal cardioplegic solution does not exist, and its selection remains challenging for surgeons. In modern surgical practice, understanding the behavior of these solutions and the ischemic tissue damage caused during induced cardiac arrest allows for safer surgical procedures. The results of this clinical trial can help in understanding the behavior of cardioplegic solutions and their tissue effects. Thus, by selecting the best cardioplegic solution, ischemic damage can be minimized, enhancing the effectiveness of this essential technique in cardiac procedures. The study may aid in implementing clinical protocols in several institutions, aiming to choose the solution with a superior myocardial protection profile, increasing safety, and reducing expenses. Trial Registration: Brazilian Clinical Trials Registry (ReBEC, http://ensaiosclinicos.gov.br/): RBR-997tqhh. Registered: January 26th, 2022.

CD68 in Cerebral Aneurysms of Smokers and Nonsmokers: An Immunohistochemical Analysis.

BACKGROUND: There is some evidence indicating that inflammation of the aneurysmal wall is related to aneurysmal growth and rupture. The presence of CD68 may indicate greater inflammatory activity. The objective of this study is to evaluate CD68 immunoexpression in surgically resected brain aneurysms and its association with smoking. METHODS: The resected brain aneurysmal walls after microsurgical clipping were envoyed to immunohistochemistry investigation. The objective was to evaluate the expression of CD68 and CD34 antibodies. The associations between inflammatory markers, smoking, and rupture were tested using Fischer's exact test. RESULTS: CD68 immunoexpression in the tunica media was associated with larger aneurysms: 7.0 mm (7.0-9.0 mm) versus 5.0 mm (3.5-5 mm; p = 0.011). There was no statistically significant association between smoking and CD68 expression in the tunica media (p = 0.234) or in either the tunica media or the tunica intima (p = 0.628). There was also no statistically significant association between hemorrhagic presentation of the aneurysm and CD68 expression in the tunica media (p = 0.689) or in either the tunica media or the tunica intima (p = 0.348). Therefore, the presence of CD68-positive cells in the aneurysmal walls indicates an association with size, especially if the tunica media is exclusively compromised (p = 0.011). CONCLUSION: Immunohistochemistry investigation for CD68 antibodies was used to determine histiocytic infiltration. Adequately powered studies are necessary to further investigate the association between CD68-positive cells and both smoking history and hemorrhagic presentation of aneurysms.

Red versus white thrombi in patients with ST-elevation myocardial infarction undergoing primary percutaneous coronary intervention: clinical and angiographic outcomes.

BACKGROUND: Aspiration thrombectomy is used in primary percutaneous coronary interventions, but the importance of thrombus constituency has been scarcely investigated. The objective of this study was to evaluate thrombus constituency and its association with clinical, laboratory, and angiographic findings in patients with ST-segment elevation myocardial infarction. METHODS: From April 2010 to May 2011, 562 patients with ST-segment elevation myocardial infarction undergoing primary percutaneous coronary interventions were considered for inclusion, and information on thrombi characteristics was available for 113 patients. Thrombus material were obtained and classified as white or red based on its constituency. Samples were analyzed by 3 independent pathologists blinded to clinical characteristics. RESULTS: The mean age of patients was 58.6 ± 12.7 years, and 69% were men. White thrombi were present in 31% of cases, and red thrombi, in 69%. Patients with white thrombi had smaller vessels and lower ischemic times. All other clinical, angiographic, and laboratory characteristics did not differ. White thrombi were smaller and associated with fibrin infiltration, whereas red thrombi were associated with red blood cell infiltration. Thirty-day death rates were lower in patients with white thrombi than red (0% vs 10.1%, respectively; P = .05), as were 30-day major adverse cardiac event rates (4.2% vs 13.9%; P = .10). Total ischemic time was well correlated with fibrin infiltration (R = -0.30; P < .01), red blood cell infiltration (R = 0.27; P < .01), and thrombus volume (R = 0.22; P = .02). CONCLUSIONS: White thrombi were present in one-third of cases and were associated with lower ischemic times, higher fibrin infiltration, smaller thrombus volume, and lower mortality. These findings suggest that thrombus constituency may be a useful prognostic tool in this setting.

Immunohistochemical characteristics of coronary thrombi in ST-elevation myocardial infarction.

Background and aims: The dynamics and implications of intracoronary thrombus constituency in patients with ST-segment elevation myocardial infarction (STEMI) are not fully understood. We evaluated the expression of CD34, CD61and factor VIII surface markers in thrombi of patients with STEMI and its association with clinical and angiographic characteristics and major adverse cardiovascular events (MACE). Methods: Patients presenting with STEMI undergoing aspiration thrombectomy during primary percutaneous coronary intervention (pPCI) were included. Morphological, histopathological and immunohistochemical aspects of thrombi were assessed by two pathologists blinded to clinical variables and outcomes. Results: The mean age of the 245 patients included was 58 ± 12 years old, and 70 % were men. Regarding the thrombi microscopic patterns, 61 % were classified as recent, 20 % as lytic and 19 % as organized. There were higher levels of the CD61 index in patients with a history of heart failure. Smokers presented lower CD61 positive cells and CD61 index, but this association did not remain significant after multivariable analysis. There was an inverse correlation between CD61 positive cells and CD61 index with the time from onset of pain to the first medical contact, but no other significant association amongst clinical characteristics and antigenic expression. There was higher expression of the CD61 antigen in patients with in-hospital MACE, but statistical significance was borderline (p = 0.06). Conclusions: In this cohort of patients with STEMI, immunohistochemistry of coronary thrombus showed a significantly higher platelet content in patients with previous heart failure and a trend in those with in-hospital MACE. Thrombus' platelet content was inversely related to ischemic time.

Prevalence of anal intraepithelial neoplasia in women with genital neoplasia.

BACKGROUND: Anal intraepithelial neoplasia is believed to be a precursor of anal cancer, and it appears to be related to high-risk human papillomavirus. Women with genital neoplasia have been shown to be at increased risk for anal cancer. OBJECTIVE: The aim of this study is to determine the prevalence of anal intraepithelial neoplasia in women with genital neoplasia. DESIGN: This is a cross-sectional study. SETTING: This study took place at Hospital Nossa Senhora da Conceição, Porto Alegre, Brazil, from May 2008 to April 2010. PATIENTS: Included in the study were 106 immunocompetent women with a histopathological diagnosis of genital neoplasia and 74 women without gynecologic neoplasia. MAIN OUTCOME MEASURE: All women underwent high-resolution anoscopy with biopsies of acetowhite areas or abnormal vascular features such as punctation and mosaics for histopathological diagnosis of anal intraepithelial neoplasia. RESULTS: : The overall prevalence of anal intraepithelial neoplasia was 6.6%. In women with genital neoplasia, the prevalence was 10.4% (95% CI, 5.6%-17.3%), whereas the prevalence was 1.4% (95% CI, 0.07%-6.5%) in women without genital neoplasia (P = .016). The prevalence ratio was 7.68 (95% CI, 1.01-58.21). LIMITATION: Women with normal results on high-resolution anoscopy were not submitted to random biopsies for ethical reasons. Small lesions could be missed, and thus our findings might be underestimated. CONCLUSION: The prevalence of anal intraepithelial neoplasia is higher in women with genital neoplasia.

Primary Intra-Axial Diffuse Large B-Cell Lymphoma in Immunocompetent Patients: Clinical Impact of Molecular Analysis and Histogenetic Evaluation.

Primary central nervous system (CNS) diffuse large B-cell lymphoma (DLBCL) represents less than 1% of non-Hodgkin lymphomas and 2%-3% of brain tumors. Primary CNS DLBCL occurs sporadically in healthy patients. Tumor development and progression have been associated with reduced/absent expression of human leukocyte antigen class I and II proteins; increased expression of CXCR4, CXCL12, CXCR5, and CCR7; mutations of VH4/34, BCL6, MYC, and PAX5 genes; and rearrangement of immunoglobulin heavy and light chain genes. Generally, DLBCL is a single supratentorial lesion (60%-70%), and stereotactic biopsy and intraoperative examination are the main diagnostic methods. Distinctive histologic features are a diffuse growth pattern and angioinvasiveness. Most neoplastic cells resemble centroblasts and exhibit positive CD20, CD22, PAX5, CD79a, and MUM1 expression. The prognosis of primary CNS DLBCL is less favorable than that of nodal DLBCL, and DLBCL subtype, strong FOXP1 immunoreactivity, MYC and BCL2 overexpression, and BCL6 translocations are associated with poor prognosis.

Intestinal lymphangitis carcinomatosa related to ovarian cancer: Case report and review of the literature.

We present a 57-year-old woman with ovarian cancer that presented to the Emergency Room with a proximal small bowel obstruction. Exploratory laparotomy evidenced a thickened 10 cm extension of the proximal jejunum without bowel peristalsis, with stenotic enteric lumen, with a lesion apparently originating from its submucosal and muscular layers. The patient underwent an exploratory laparotomy with total hysterectomy, bilateral salpingo-oophorectomy, omentectomy, small bowel resection and peritoneal biopsies. Final pathology and immunohistochemistry confirmed the intra-operative suspicion of lymphatic intestinal spread of malignant cells originating from a high grade serous carcinoma of ovarian origin. To the best of our knowledge, this is the first report in the literature of intestinal carcinomatous lymphangitis related to ovarian cancer, and the first report of involvement of the proximal portion of the jejunum.

Pathogenesis, histopathologic findings and treatment modalities of lipoprotein glomerulopathy: A review.

Lipoprotein glomerulopathy (LPG) is an uncommon cause of nephrotic syndrome and/or kidney failure. At microscopy, LPG is characterized by the presence of lipoprotein thrombi in dilated glomerular capillaries due to different ApoE mutations. ApoE gene is located on chromosome 19q13.2, and can be identified in almost all serum lipoproteins. ApoE works as a protective factor in atherosclerosis due its interaction with receptor-mediated lipoprotein clearance and cholesterol receptor. Most common polymorphisms include ApoE2/2, ApoE3/2, ApoE3/3, ApoE4/2, ApoE4/3, and ApoE4/4. All age-groups can be affected by LPG, with a discrete male predominance. Compromised patients typically reveal dyslipidemia, type III hyperlipoproteinemia, and proteinuria. LPG treatment includes fenofibrate, antilipidemic drugs, steroids, LDL aphaeresis, plasma exchange, antiplatelet drugs, anticoagulants, urokinase, and renal transplantation. Recurrence in kidney graft suggests a pathogenic component(s) of extraglomerular humoral complex resulting from abnormal lipoprotein metabolism and presumably associated to ApoE.

Prognostic value of the immunohistochemistry correlation of Ki-67 and p53 in squamous cell carcinomas of the larynx.

UNLABELLED: Prognostic histological factors may contribute to determine the evolution of this neoplasia. AIM: To correlate p53 and Ki-67 immunohistochemical expression with age, histological degree, lymph node involvement and pathological staging in patients with laryngeal epidermoid carcinomas. METHODS: We assessed thirty consecutive cases of laryngeal epidermoid carcinomas submitted to immunohistochemistry to check the expression of p53 e Ki-67 antibodies. RESULTS: Mean age was of 56.2 years and the immunoexpression of the markers was observed in the group with more than 50 years of age, especially that o the ki-67 antibody (p=0.032). There was no relation between p53 and Ki-67 with lymph node involvement. Ki-67 was expressed in 70% of the high histology level cases and in 80% in the low histology ones; while p53 was of 70% only in the high level cases. Pathology staging showed that in the group of advanced carcinomas, p53 expression was of 61.5%, while Ki-67 proved positive for the early cases (100%) and advanced (73.1%). CONCLUSION: There were no significant differences between p53 and Ki-67 immunoexpression in laryngeal epidermoid carcinoma, except in the group of patients with more than 50 years of age, when Ki-67 expression was significantly higher.

Atypical spinal epidural capillary hemangioma: Case report.

BACKGROUND: Hemangiomas are benign vascular malformations that can involve the spine. Pure epidural hemangiomas are rare and represent only 4% of all epidural lesions. Most hemangiomas are of the cavernous type; the capillary variant is atypical, and only ten cases have been reported in the literature. CASE DESCRIPTION: A 69-year-old female presented with nonspecific dorsal pain. Magnetic resonance imaging (MRI) showed a spinal epidural tumor at the T9-T10 level. Following a T9-T11 laminectomy, the lesion was completely resected en bloc. Histopathologic analysis showed a pure epidural capillary hemangioma with adipose tissue mesenchyma. CONCLUSIONS: Although epidural capillary hemangiomas are extremely rare, they should be considered among the differential diagnoses of extradural, extramedullary spinal lesions. Further, they must be differentiated from other more common lesions such as meningiomas and schwannomas. The recommended surgical management is en bloc gross total excision.

Enterogenous Cyst and Glioblastoma: A Brief Histopathological Review of Two Uncommon Cystic Lesions of the Central Nervous System / Cisto entérico e glioblastoma: Uma breve revisão histopatológica de duas lesões císticas incomuns do sistema nervoso central

Intracranial cystic lesions are common findings in cerebral imaging and might represent a broad spectrum of conditions. These entities can be divided into nonneoplastic lesions, comprising Rathke cleft cyst, arachnoid cyst, and colloid cyst, as well as neoplastic lesions, including benign and malignant components of neoplasms such as pilocytic astrocytoma, hemangioblastoma, and ganglioglioma. Surgical resection and histological evaluation are currently the most effective methods to classify cysts of the central nervous system. The authors report two uncommon cases presenting as cystic lesions of the encephalic parenchyma­a enterogenous cyst and a glioblastoma­and discuss typical histological findings and differential diagnosis.

Lesões císticas intracranianas são achados comuns em imagens cerebrais e podem representar um amplo espectro de condições. Essas entidades podem ser divididas em lesões não neoplásicas, compreendendo cisto da bolsa de Rathke, cisto aracnoide e cisto colóide, e lesões neoplásicas, incluindo componentes benignos e malignos de neoplasias, como astrocitoma pilocítico, hemangioblastoma e ganglioglioma. A ressecção cirúrgica e a avaliação histológica são atualmente os métodos mais eficazes para classificar os cistos do sistema nervoso central. Os autores relatam dois casos incomuns que se apresentam como lesões císticas do parênquima encefálico, um cisto entérico e um glioblastoma, e discutem achados histológicos típicos e diagnósticos diferenciais.

Recurrence of Anaplastic Large Cell Lymphoma in the Frontal Lobe After Eleven Years of the Initial Diagnosis: Histopathological Findings and Prognosis / Recorrência de linfoma anaplásico de grandes células no lobo frontal onze anos após o diagnóstico inicial: Achados histopatológicos e prognóstico

Anaplastic large cell lymphoma (ALCL) is a rare, high-grade, T-cell neoplasm classified into cutaneous primary, systemic primary ALK-positive (ALKþ), systemic primary ALK-negative, or breast-implant associated. Secondary involvement of the central nervous system (CNS) by systemic primary ALKþ ALCL is a rare occurrence. We present a case of CNS involvement by ALKþ ALCL eleven years after diagnosis of the primary tumor in the thoracic vertebra. The anatomopathological examination confirmed the diagnosis of ALKþ ALCL. A brief review of the treatment and the clinical and pathological aspects is presented.

O linfoma anaplásico de grandes células (LAGC) corresponde a uma neoplasia de alto grau rara, com imunofenótipo T, que podendo ser dividido em primário cutâneo, primário sistêmico ALK positivo (ALKþ), primário sistêmico ALK negativo, e associado a próteses mamárias. Acometimento secundário do sistema nervoso central (SNC) por LAGC primário sistêmico ALKþ é uma rara entidade. Os autores apresentam um caso de acometimento do SNC por LAGC ALKþ onze anos após o diagnóstico do tumor primário em vértebra torácica. O exame anatomopatológico confirmou o diagnóstico de LAGC ALKþ. Fez-se também uma breve revisão de aspectos clínicos e patológicos e tratamento.

The Effects of Passive Smoking on Laryngeal and Tracheal Mucosa in Male Wistar Rats During Growth: An Experimental Study.

Cigarettes contain toxic and carcinogenic substances. In this context, cigarette smoking, and similar activities, are associated with numerous pathologies, being considered a risk factor in up to 10% of the total number of deaths in adults. Recent evidence suggests that the exposure of children to smoking in the early days of their development causes many diseases. Using light microscopy, this study aims to analyze the possible histopathological effects of an experimental model of chronic inhalation of cigarette smoke (passive smoking) on the laryngeal and tracheal mucosa of young Wistar rats. A total of 24 young Wistar rats were studied for a period of 120 days. The animals were divided into two groups: passive smoking (n = 16) and control (n = 8). The level of exposure to cigarette smoke was evaluated from the urinary cotinine level. Although no cancerous lesions were identified, histopathological analysis in the laryngeal and tracheal mucosa of all the animals in the experimental group showed that the proportion of moderate and focal inflammation was higher in animals exposed to chronic inhalation of cigarette smoke (P = 0.041). Histopathologic analysis revealed moderate and focal inflammatory lesions in the region of the infraglottic mucosa in exposed animals, although without dysplastic or neoplastic lesions in the laryngeal and tracheal mucosa.