Mohs micrographic surgery in immunosuppressed vs immunocompetent patients: Results of a prospective nationwide cohort study (REGESMOHS, Spanish registry of Mohs surgery).

BACKGROUND: Immunosuppressed (IS) patients, particularly solid organ transplant recipients and those on immunosuppressive therapy, face a higher incidence and recurrence of nonmelanoma skin cancers (NMSC), including basal cell carcinoma (BCC) and squamous cell carcinoma (SCC). Mohs micrographic surgery (MMS) is the preferred treatment for high-risk NMSC due to its high cure rate and margin examination capabilities. However, IS patients may experience more complications, such as surgical site infections, and a greater risk of recurrence, making their outcomes a subject of interest. OBJECTIVES: This study aimed to compare IS and immunocompetent (IC) patients undergoing MMS for NMSC in terms of baseline characteristics, intra- and post-surgical complications, and postoperative recurrence rates. METHODS: The study utilized data from the REGESMOHS registry, a 7-year prospective cohort study in Spain. It included 5226 patients, categorizing them into IC (5069) and IS (157) groups. IS patients included solid organ transplant recipients, those on immunosuppressive treatments, individuals with haematological tumours and HIV-positive patients. Patient data, tumour characteristics, surgical details and outcomes were collected and analysed. RESULTS: IS patients demonstrated a higher proportion of SCC, multiple synchronous tumours and tumours invading deeper structures. Complex closures, unfinished MMS and more surgical sections were observed in the IS group. Although intra-operative morbidity was higher among IS patients, this difference became non-significant when adjusted for other variables such as year of surgery, antiplatelet/anticoagulant treatment or type of closure. Importantly, IS patients had a substantially higher recurrence rate (IRR 2.79) compared to IC patients. CONCLUSIONS: This study suggests that IS patients may be at a higher risk of development of AE such as bleeding or tumour necrosis and are at a higher risk of tumour recurrence. Close follow-up and consideration of the specific characteristics of NMSC in IS patients are crucial. Further research with extended follow-up is needed to better understand the long-term outcomes for this patient group.

Multimodal management of giant solid hemangioblastomas in two patients with preoperative embolization.

Background: Hemangioblastomas are benign vascular neoplasms, World Health Organization grade I, with the most frequent location in the cerebellum. Complete microsurgical resection can be a challenge due to excessive bleeding, which is why preoperative embolization takes importance. Case Description: Two clinical cases are presented, a 25-year-old woman and a 75-year-old man, who presented with intracranial hypertension symptoms due to obstructive hydrocephalus; a ventriculoperitoneal shunt was placed in both cases; in addition, they presented with cerebellar signs. Both underwent embolization with ethylene vinyl alcohol copolymer, with blood flow reduction. After that, they underwent microsurgical resection within the 1st-week post embolization, obtaining, in both cases, gross total resection without hemodynamic complications, with clinical improvement and good surgical outcome. It is worth mentioning that surgical management is the gold standard that allows a suitable surgical approach, like in our patients, for which a lateral suboccipital craniotomy was performed. Conclusion: Solid hemangioblastomas are less frequent than their cystic counterparts. The treatment is the surgical resection, which is a challenge and always has to be considered as an arteriovenous malformation in the surgical planning, including preoperative embolization to reduce perioperative morbidity and mortality and get good outcomes.

Detection and Surgical Approach to Pheochromocytoma: A Case Report.

Pheochromocytomas are neuroendocrine tumors that produce, store, and secrete catecholamines. They are found in the chromaffin tissue of the adrenal medulla and manifest clinical symptoms by producing an excessive amount of one or more catecholamines, such as dopamine, adrenaline, and noradrenaline, as well as their inactive metabolites, such as metanephrine, normetanephrine, and 3-methoxytyramine. This paper is the case report of a 53-year-old male patient with diabetes and hypertension who has been experiencing symptoms such as night sweats, frequent colds, weight loss, reduced appetite, and generalized anxiety. The patient presented with pelvic pain and sought medical attention, leading to an abdominal MRI scan that revealed a right adrenal mass. The patient's plasma metanephrine levels were found to be four-fold higher than the normal range. A contrast CT scan of the abdomen and pelvis revealed a right adrenal gland with increased dimensions and well-defined edges. A diagnosis of right adrenal pheochromocytoma was made. The patient underwent a right laparoscopic adrenalectomy, which resulted in a reduction in metanephrine levels and normal blood pressure readings. The patient presented a favorable clinical evolution in the post-surgical period, for which it was decided to be discharged home.

Concentraciones de progesterona en saliva en pacientes sintomáticas con parto pretérmino inminente y pacientes con partos más allá de 7 días

Objetivo: Comparar las concentraciones de progesterona en saliva en pacientes sintomáticas con parto pretérmino inminente y pacientes con partos más allá de 7 días. Metodología: Estudio prospectivo y longitudinal realizado en el Hospital Central "Dr. Urquinaona" de Venezuela. Fueron seleccionadas pacientes con parto pretérmino en los siguientes 7 días (grupo A) y con parto pretérmino más allá de los 7 días (grupo B). Se analizaron las características generales, concentraciones de progesterona en saliva, parto pretérmino inminente y eficacia pronóstica. Resultados: Fueron incluidas 327 pacientes, 75 mujeres en el grupo A y 251 pacientes en el grupo B. Las pacientes del grupo A presentaron valores menores de progesterona en saliva (3,003 +/- 447 pg/mL) comparado con las pacientes del grupo B (3,639 +/- 430 pg/mL; p < 0,0001). El valor de corte predictivo fue de 3,100 pg/mL, demostrando un valor área bajo la curva de 0,834 con sensibilidad de 58,7%, especificidad de 84,9%, valor predictivo positivo de 53,7%, valor predictivo negativo de 87,3% y exactitud pronostica de 78,8% para la predicción de parto pretérmino inminente. Conclusiones: Las concentraciones de progesterona en saliva son significativamente más bajas en las pacientes con parto pretérmino inminente, comparado con aquellas pacientes con partos más allá de los 7 días. (provisto por Infomedic International)

Objective: To compare progesterone concentrations in saliva in symptomatic patients with imminent preterm labor and patients with deliveries beyond 7 days. Methodology: Prospective and longitudinal study carried out at the Central Hospital Dr. Urquinaona of Venezuela. Patients with preterm delivery within 7 days (group A) and with preterm delivery beyond 7 days (group B) were selected. General characteristics, saliva progesterone concentrations, imminent preterm delivery and prognostic efficacy were analyzed. Results: A total of 327 patients were included, 75 women in group A and 251 patients in group B. Patients in group A had lower saliva progesterone values (3.003 +/- 447 pg/mL) compared to patients in group B (3.639 +/- 430 pg/mL; p < 0.0001). The predictive cutoff value was 3,100 pg/mL, demonstrating an area under the curve value of 0.834 with sensitivity of 58.7%, specificity of 84.9%, positive predictive value of 53.7%, negative predictive value of 87.3% and prognostic accuracy of 78.8% for prediction of impending preterm labor. Conclusions: Saliva progesterone concentrations are significantly lower in patients with imminent preterm labor compared to those patients with deliveries beyond 7 days. (provided by Infomedic International)

The paradigm of total body irradiation in acute lymphoblastic leukaemia: Therapeutic effectiveness versus the challenges of toxicity.

INTRODUCTION: Total body irradiation (TBI) is part of the myeloablative conditioning for hematopoietic stem cell transplantation (HSCT) in malignant hematologic disorders. This therapy has recently shown improved survival in acute lymphoblastic leukemia (ALL) compared to chemotherapy-based regimens. However, side effects are a significant limitation, especially in the pediatric population. PATIENTS AND METHODS: We retrospectively analyzed the survival of patients with ALL who underwent an HSCT at a tertiary hospital between 1996 and 2009 (N = 69 HSCT in 57 patients). We differentiated a cohort that received TBI (N = 44) from another that did not (N = 25). Subsequently, we interviewed the survivors from the TBI group with a minimum of 10 years of follow-up (N = 18), asking about the presence of side effects. RESULTS: The overall survival (OS) at 2 and 5 years was 79.1% and 65.2% respectively for the TBI group and 66.2% and 55.8% for the non-TBI group, although this difference was not significant (P=.31). The event-free survival (EFS) at 2 and 5 years was 77.3% and 63.6% respectively for the TBI group and 56% and 32% for the non-TBI group (P=.02). The probability of relapse (PR) at 2 years for those who received TBI was 10% compared to 28.6% for those who did not receive TBI (P=.005). Survivors who received TBI developed secondary neoplasms (39%), dyslipidemia (67%), cognitive impairments affecting memory (44%), recurrent respiratory infections (39%), thyroid abnormalities (45%), premature ovarian failure (89%), cataracts (22%), and psychological problems (44%). However, the quality of life, as self-assessed by the patients, was considered good for 83% of the participants.. CONCLUSIONS: Patients who received TBI had significantly higher EFS and lower PR. However, adverse effects are frequent and significant, although they do not subjectively affect quality of life.

Pearls & Oy-sters: KLHL11 IgG Paraneoplastic-Associated Hearing Loss and Rhombencephalitis in a Woman With Metastatic Müllerian Tumor.

Kelch-like protein-11 (KLHL11) immunoglobulin G (IgG) is a recently reported paraneoplastic autoantibody associated with rhombencephalitis, which commonly presents with ataxia, diplopia, vertigo, hearing loss, tinnitus, and gaze palsies. The association of this high-risk paraneoplastic autoantibody with testicular germ cell tumors is widely accepted, but it has not been associated with Müllerian tumors. In this study, we report a woman without a known germ cell tumor presenting with signs and symptoms suggesting autoimmune encephalitis. She was found to have metastatic ovarian serous carcinoma with KLHL11 immunoreactivity on histopathology. This case demonstrates a rare cancer association of KLHL11 IgG-seropositive rhombencephalitis with Müllerian tumor and highlights that this autoantibody can also be detected in female patients. Thus, this case expands on the current knowledge of KLHL11-related autoimmune encephalitis including the paraneoplastic presentation, associated tumor types, and management of this syndrome in women.

Interventions to Mitigate Financial Toxicity in Adult Patients with Cancer in the United States: A Scoping Review.

Financial toxicity adversely affects quality of life and treatment outcomes for patients with cancer. This scoping review examined interventions aimed at mitigating financial toxicity in adult patients with cancer and their effectiveness. We utilized five bibliographical databases to identify studies that met our inclusion criteria. The review included studies conducted among adult patients with cancer in the United States and published in English between January 2011 to March 2023. The review identified eight studies that met the inclusion criteria. Each of the studies discussed the implementation of interventions at the patient/provider and/or health system level. Collectively, the findings from this scoping review highlight both the limited number of published studies that are aimed at mitigating financial toxicity and the need to create and assess interventions that directly impact financial toxicity in demographically diverse populations of adult patients with cancer.

A tower of babel of acronyms? The shadowlands of MGUS/MBL/CHIP/TCUS.

With the advent of outperforming and massive laboratory tools, such as multiparameter flow cytometry and next-generation sequencing, hematopoietic cell clones with putative abnormalities for a variety of blood malignancies have been appreciated in otherwise healthy individuals. These conditions do not fulfill the criteria of their presumed cancer counterparts, and thus have been recognized as their precursor states. This is the case of monoclonal gammopathy of unknown significance (MGUS), the first blood premalignancy state described, preceding multiple myeloma (MM) or Waldenström macroglobulinemia (WM). However, in the last 2 decades, an increasing list of clonopathies has been recognized, including monoclonal B cell lymphocytosis (MBL), which antecedes chronic lymphocytic leukemia (CLL), clonal hematopoiesis of indeterminate potential (CHIP) for myeloid neoplasms (MN), and T-cell clones of uncertain significance (TCUS) for T-cell large chronic lymphocytic leukemia (LGLL). While for some of these entities diagnostic boundaries are precisely set, for others these are yet to be fully defined. Moreover, despite mostly considered of "uncertain significance," they have not only appeared to predispose to malignancy, but also to be capable of provoking set of immunological and cardiovascular complications that may require specialized management. The clinical implications of the aberrant clones, together with the extensive knowledge generated on the pathogenetic events driving their evolution, raises the question whether earlier interventions may alter the natural history of the disease. Herein, we review this Tower of Babel of acronyms pinpointing diagnostic definitions, differential diagnosis, and the role of genomic profiling of these precursor states, as well as potential interventional strategies.

Clinical validation of a blood-based liquid biopsy test integrating cell-free DNA quantification and next-generation sequencing for cancer screening in dogs.

OBJECTIVE: To validate the performance of a novel, integrated test for canine cancer screening that combines cell-free DNA quantification with next-generation sequencing (NGS) analysis. SAMPLE: Retrospective data from a total of 1,947 cancer-diagnosed and presumably cancer-free dogs were used to validate test performance for the detection of 7 predefined cancer types (lymphoma, hemangiosarcoma, osteosarcoma, leukemia, histiocytic sarcoma, primary lung tumors, and urothelial carcinoma), using independent training and testing sets. METHODS: Cell-free DNA quantification data from all samples were analyzed using a proprietary machine learning algorithm to determine a Cancer Probability Index (High, Moderate, or Low). High and Low Probability of Cancer were final result classifications. Moderate cases were additionally analyzed by NGS to arrive at a final classification of High Probability of Cancer (Cancer Signal Detected) or Low Probability of Cancer (Cancer Signal Not Detected). RESULTS: Of the 595 dogs in the testing set, 89% (n = 530) received a High or Low Probability result based on the machine learning algorithm; 11% (65) were Moderate Probability, and NGS results were used to assign a final classification. Overall, 87 of 122 dogs with the 7 predefined cancer types were classified as High Probability and 467 of 473 presumably cancer-free dogs were classified as Low Probability, corresponding to a sensitivity of 71.3% for the predefined cancer types at a specificity of 98.7%. CLINICAL RELEVANCE: This integrated test offers a novel option to screen for cancer types that may be difficult to detect by physical examination at a dog's wellness visit.

Síndrome de Kartagener y artritis reumatoide. Reporte de caso / Kartagener Syndrome and Rheumatoid Arthritis. Case Report

Presentación del caso. Se trata de una mujer de 26 años de edad, en seguimiento por la especialidad de reumatología desde los 17 años, cuando consultó con historia de un año de evolución de síndrome poliarticular de grandes y pequeñas articulaciones, aditivo, simétrico acompañado de fatiga, rigidez matutina mayor de una hora. Se reportó además factor reumatoide positivo. La radiografía de ambas manos presentó erosiones, que confirmó el diagnóstico de artritis reumatoide. Adicionalmente, la paciente tenía el antecedente de procesos sinobronquiales a repetición desde su infancia. En la evaluación médica se identificó dolor en los senos paranasales, dextrocardia y bronquiectasias, confirmados por los estudios de imágenes, que permitió concluir en el diagnóstico de síndrome de Kartagener. Intervención terapéutica. La paciente presentaba actividad clínica severa de la artritis reumatoide, se inició el tratamiento con metotrexato 10 mg vía oral un día a la semana, prednisona 5 mg al día y ácido fólico 5 mg a la semana y citas periódicas, controlando los datos de actividad y efectos adversos de los medicamentos, con pruebas hepáticas, hemograma y transaminasas. La especialidad de neumología recomendó la inclusión de la paciente en un programa de rehabilitación respiratoria, así como el uso de azitromicina 500 mg cada día por tres días en los períodos de agudización. Evolución clínica. El tratamiento logró mantener una actividad leve de la artritis reumatoide y sin exacerbación de los síntomas respiratorios

Case presentation. A 26-year-old woman, under follow-up by the rheumatology specialty since she was 17 years old, when she consulted with a history of one year of evolution of polyarticular disease of large and small joints, additive, symmetrical, accompanied by fatigue and morning stiffness for more than one hour. Positive rheumatoid factor was also reported. Additionally, the patient had a history of repeated sinobronchial processes since childhood. Medical examination revealed sinus pain in the paranasal sinuses, dextrocardia, and bronchiectasis, confirmed by imaging studies, which led to the diagnosis of Kartagener's syndrome. Treatment. The patient presented the severe clinical activity of rheumatoid arthritis. The treatment was started with methotrexate 10 mg orally one day a week, prednisone 5 mg a day, and folic acid 5 mg a week and periodic appointments, controlling the activity data and adverse effects of the drugs, with liver tests, hemogram, and transaminases. The pneumology department recommended the inclusion of the patient in a respiratory rehabilitation program as well as the use of azithromycin 500 mg every day for three days during periods of exacerbation. Outcome. The treatment was successful in maintaining a mild activity of the rheumatoid arthritis and without exacerbation of respiratory symptoms

Proteomic characterization and biological activities of the mucus produced by the zoanthid Palythoa caribaeorum (Duchassaing & Michelotti, 1860).

Mucus, produced by Palythoa caribaeorum has been popularly reported due to healing, anti-inflammatory, and analgesic effects. However, biochemical and pharmacological properties of this mucus remains unexplored. Therefore, the present study aimed to study its proteome profile by 2DE electrophoresis and MALDI-TOF. Furthermore, it was evaluated the cytotoxic, antibacterial, and antioxidant activities of the mucus and from its protein extract (PE). Proteomics study identified14 proteins including proteins involved in the process of tissue regeneration and death of tumor cells. The PE exhibited cell viability below 50% in the MCF-7 and S-180 strains. It showed IC50 of 6.9 µg/mL for the J774 lineage, and also, favored the cellular growth of fibroblasts. Furthermore, PE revealed activity against Escherichia coli, Klebsiella pneumoniae, Staphylococcus aureus, and Staphylococcus epidermidis (MIC of 250 µg/mL). These findings revealed the mucus produced by Palythoa caribaeorum with biological activities, offering alternative therapies for the treatment of cancer and as a potential antibacterial agent.

Next-generation sequencing-based liquid biopsy may be used for detection of residual disease and cancer recurrence monitoring in dogs.

OBJECTIVE: The purpose of this study was to evaluate the performance of a next-generation sequencing-based liquid biopsy test for cancer monitoring in dogs. SAMPLES: Pre- and postoperative blood samples were collected from dogs with confirmed cancer diagnoses originally enrolled in the CANcer Detection in Dogs (CANDiD) study. A subset of dogs also had longitudinal blood samples collected for recurrence monitoring. METHODS: All cancer-diagnosed patients had a preoperative blood sample in which a cancer signal was detected and had at least 1 postoperative sample collected. Clinical data were used to assign a clinical disease status for each follow-up visit. RESULTS: Following excisional surgery, in the absence of clinical residual disease at the postoperative visit, patients with Cancer Signal Detected results at that visit were 1.94 times as likely (95% CI, 1.21 to 3.12; P = .013) to have clinical recurrence within 6 months compared to patients with Cancer Signal Not Detected results. In the subset of patients with longitudinal liquid biopsy samples that had clinical recurrence documented during the study period, 82% (9/11; 95% CI, 48% to 97%) had Cancer Signal Detected in blood prior to or concomitant with clinical recurrence; in the 6 patients where molecular recurrence was detected prior to clinical recurrence, the median lead time was 168 days (range, 47 to 238). CLINICAL RELEVANCE: Next-generation sequencing-based liquid biopsy is a noninvasive tool that may offer utility as an adjunct to current standard-of-care clinical assessment for cancer monitoring; further studies are needed to confirm diagnostic accuracy in a larger population.

Estudio multicéntrico sobre multimorbilidad en internados en áreas clínicas / Multicenter Study on Multimorbidity in Patients in Clinical Areas

Introducción: El estudio de la comorbilidad requiere de un enfoque multilateral con vistas a mejorar la calidad de la atención de los enfermos por el sistema de atención. Objetivos: Explorar la magnitud de la comorbilidad de enfermedades crónicas en adultos internados en los hospitales. Métodos: Se realizó un estudio prospectivo-observacional-longitudinal-analítico. Se incluyeron pacientes internados en las Salas de Clínica Médica o pacientes clínicos en Salas de Internación Indiscriminada. Se realizó un estudio multicéntrico en 42 centros en un período de 2 años, con un muestreo consecutivo. Para el estudio se tuvo en cuenta la estadística descriptiva, inferencial y de regresión. Resultados: El total de pacientes en el estudio fue de 5925, masculinos con el 50,3 por ciento de edad 60,66 ± 0,25 años. Principal procedencia desde la guardia el 73 por ciento. La estadía hospitalaria de 12,61 ± 0,24 días, mayormente en pacientes quirúrgicos (15,45 ± 0,67 vs 11,76 ± 0,23; p < 0,00001). El 23 por ciento recibió tratamiento quirúrgico. El principal nivel educativo: secundario completo 21,6 por ciento. Dificultades económicas: 20 por ciento, mortalidad 9,26 por ciento; prevalencia de dislipemia, diabetes e hipertensión: 22,53 por ciento; 28,82 por ciento y 51,86 por ciento con 473 nuevos diagnósticos, IMC: 27,88 ± 0,65, Charlson global 2,09 ± 0,02 y en óbitos 3,84 ± 0,11. La media de patologías por paciente fue de 2,14 ± 0,01 y aumentó con la edad (p valor regresión lineal < 0,00001). Conclusiones: La hipertensión, la diabetes y la dislipemia representaron las entidades más prevalentes en Salas de Internación Clínica, Las enfermedades cardiovasculares, respiratorias, infectológicas, oncológicas, neurológicas, metabólicas y nefrológicas fueron predictores independientes de mortalidad(AU)

Introduction: The study of comorbidity requires a multilateral approach with a view to improving the quality of care for these patients by the care system. Objectives: To explore the magnitude of the comorbidity of chronic diseases in adults admitted to hospitals. Methods: Prospective-observational-longitudinal-analytical study. Patients hospitalized in a medical clinic room or clinical patients in indiscriminate hospitalization rooms are included, Multicenter study in 42 centers, with 2 years of recruitment. Consecutive sampling. Descriptive, inferential and regression statistics. Results: 5925 recruited, male gender 50,3percent, age 60,66 ± 0,25 years, main origin from the guard 73percent, stay 12,61 ± 0,24 days, longer in surgical (15,45 ± 0,67 vs 11,76 ± 0,23, p < 0,00001), 23percent received surgical treatment. Main educational level: complete secondary school 21,6%. Economic difficulties: 20percent, mortality 9,26percent, prevalence of dyslipidemia, diabetes and hypertension: 22,53percent, 28,82percent and 51,86percent with 473 new diagnoses in said pathologies, BMI: 27,88 ± 0,65, Global Charlson 2,09 ± 0,02 and in deaths 3,84 ± 0,11. The average number of pathologies per patient was 2,14 ± 0,01 and increased with age (p value for linear regression < 0,00001). Conclusions: Hypertension, diabetes and dyslipidemia represented the most prevalent entities in the clinical hospitalization room, cardiovascular, respiratory, infectious, oncological, neurological, metabolic and nephrological diseases were independent predictors of mortality(AU)

Immunotherapy in Acute Myeloid Leukemia: A Literature Review of Emerging Strategies.

In the last twenty years, we have witnessed a paradigm shift in the treatment and prognosis of acute myeloid leukemia (AML), thanks to the introduction of new efficient drugs or approaches to refine old therapies, such as Gemtuzumab Ozogamicin, CPX 3-5-1, hypomethylating agents, and Venetoclax, the optimization of conditioning regimens in allogeneic hematopoietic stem cell transplantation and the improvement of supportive care. However, the long-term survival of non-M3 and non-core binding factor-AML is still dismal. For this reason, the expectations for the recently developed immunotherapies, such as antibody-based therapy, checkpoint inhibitors, and chimeric antigen receptor strategies, successfully tested in other hematologic malignancies, were very high. The inherent characteristics of AML blasts hampered the development of these treatments, and the path of immunotherapy in AML has been bumpy. Herein, we provide a detailed review of potential antigenic targets, available data from pre-clinical and clinical trials, and future directions of immunotherapies in AML.

The Role of Nodes and Nodal Assessment in Diagnosis, Treatment and Prediction in ER+, Node-Positive Breast Cancer.

The majority of breast cancers are oestrogen receptor-positive (ER+). In ER+ cancers, oestrogen acts as a disease driver, so these tumours are likely to be susceptible to endocrine therapy (ET). ET works by blocking the hormone's synthesis or effect. A significant number of patients diagnosed with breast cancer will have the spread of tumour cells into regional lymph nodes either at the time of diagnosis, or as a recurrence some years later. Patients with node-positive disease have a poorer prognosis and can respond less well to ET. The nodal metastases may be genomically similar or, as is becoming more evident, may differ from the primary tumour. However, nodal metastatic disease is often not assessed, and treatment decisions are almost always based on biomarkers evaluated in the primary tumour. This review will summarise the evidence in the field on ER+, node-positive breast cancer, including diagnosis, treatment, prognosis and predictive tools.

An Ecchordosis Physaliphora, a Rare Entity, Involving the Central Nervous System: A Systematic Review of the Literature.

Ecchordosis physaliphora (EP) is a benign notochordal remnant, which is often an incidental finding; however, it can rarely present with neurological symptoms. We performed a systematic review of the literature for cases of symptomatic EP published in PubMed, Web of Science and Embase from January 1982 to May 2023. This is the largest review to date and revealed 60 cases including ours. Headache (55%) and CSF rhinorrhea (32%) were the most frequent clinical manifestations. The majority of symptomatic EP lesions were located in the prepontine region (77%) and required surgical resection (75%). EP should be considered in patients with neurologic symptoms in the setting of prepontine or posterior sphenoid sinus lesions. While symptomatic patients often require surgical intervention, rare cases may respond to oral corticosteroids.

A Rare Case of Gastrointestinal Stromal Tumor of the Abdominal Cavity: A Case Report.

Gastrointestinal stromal tumors (GISTs) are rare gastrointestinal neoplasms. We are presenting a 57-year-old female patient with a GIST of considerable dimensions that was deemed unresectable during the initial intervention. The patient had a previous surgical record of a peritoneal sarcomatosis tumor that covered approximately 50% of the abdominal cavity. The patient underwent surgical procedures, including exploratory laparotomy, lumpectomy, splenectomy, proximal gastrectomy, esophagogastric anastomosis, pyloroplasty, jejunostomy, and left diaphragm plasty. One of the observed results was the presence of a 50×40 cm exophytic multilobed cerebroid-like tumor in the abdomen region, specifically dependent on the gastric fundus. The primary treatment for patients without metastases is surgical removal of the tumor, with wedge resection being recommended for the preservation of organ function and quality of life postoperatively.

Paroxysmal Nocturnal Hemoglobinuria: Biology and Treatment.

Paroxysmal nocturnal hemoglobinuria (PNH) is a nonmalignant clonal hematopoietic disorder characterized by the lack of glycosylphosphatidylinositol-anchored proteins (GPI-APs) as a consequence of somatic mutations in the phosphatidylinositol glycan anchor biosynthesis class A (PIGA) gene. Clinical manifestations of PNH are intravascular hemolysis, thrombophilia, and bone marrow failure. Treatment of PNH mainly relies on the use of complement-targeted therapy (C5 inhibitors), with the newest agents being explored against other factors involved in the complement cascade to alleviate unresolved intravascular hemolysis and extravascular hemolysis. This review summarizes the biology and current treatment strategies for PNH with the aim of reaching a general audience with an interest in hematologic disorders.