Retos y estrategias en el manejo de la trombosis de la vena porta / Challenges and strategies in the management of portal vein thrombosis

La trombosis de la vena porta (TVP) en pacientes con o sin cirrosis hepática (CH) se define como una obstrucción de la vena porta debido a la formación de un trombo que puede extenderse a las venas mesentéricas superiores y esplénica. Esta es una complicación común de la enfermedad hepática avanzada. Se creía que la TVP se producía predominantemente debido al potencial protrombótico del paciente con CH, ya que se observaba una mayor incidencia de TVP en CH con una puntuación MELD y Child-Pugh más altas, con una prevalencia informada del 10 % al 25%.

Portal vein thrombosis (PVT) in patients with or without hepatic cirrhosis (CH) is defined as an obstruction of the portal vein due to the formation of a thrombus that may extend to the superior mesenteric and splenic veins. This is a common complication of advanced liver disease. It was believed that PVT predominantly occurred due to the prothrombotic potential of the patient with CH, as a higher incidence of PVT was observed in CH with higher MELD and Child-Pugh scores, with a reported prevalence of 10% to 25%.

Segmental congenital vascular anomaly with atrophy, ulceration, and scarring (SeCVAUS): Case series and review of literature.

BACKGROUND: Next-generation sequencing has greatly increased our understanding of vascular birthmarks. Many port-wine birthmarks are due to somatic mutations in GNAQ/GNA11 exon 183, but other genomic causes have been identified. Most congenital hemangiomas are due to somatic mutations in GNAQ/GNA11 at exon 209. Although genomically distinct, clinical overlap of congenital hemangiomas and port-wine birthmarks has occasionally been described. OBJECTIVE: We report a case series of a unique segmentally distributed vascular anomaly with overlapping characteristics of port-wine birthmarks and congenital hemangiomas with other distinctive features including ulceration, atrophy, and scarring. METHODS: This was a multicenter study with retrospective identification of patients via a detailed review of medical records. We also reviewed previously published cases. RESULTS: The clinical, histological, radiological, and genomic characteristics of 19 new and 13 previously reported cases characterized by segmental distribution, sharply demarcated borders, with variable thickening are presented. All cases had central atrophy with or without episodic ulceration. Those with genomic studies (13 out of 32) had somatic activating missense mutations in GNA11 or GNAQ codon 209. CONCLUSIONS: We describe the features and propose a descriptive name segmental congenital vascular anomaly with atrophy, ulceration, and scarring (SeCVAUS) for this condition.

Cellular Response of Adapted and Non-Adapted Tetrahymena thermophila Strains to Europium Eu(III) Compounds.

Europium is one of the most reactive lanthanides and humans use it in many different applications, but we still know little about its potential toxicity and cellular response to its exposure. Two strains of the eukaryotic microorganism model Tetrahymena thermophila were adapted to high concentrations of two Eu(III) compounds (EuCl3 or Eu2O3) and compared to a control strain and cultures treated with both compounds. In this ciliate, EuCl3 is more toxic than Eu2O3. LC50 values show that this microorganism is more resistant to these Eu(III) compounds than other microorganisms. Oxidative stress originated mainly by Eu2O3 is minimized by overexpression of genes encoding important antioxidant enzymes. The overexpression of metallothionein genes under treatment with Eu(III) compounds supports the possibility that this lanthanide may interact with the -SH groups of the cysteine residues from metallothioneins and/or displace essential cations of these proteins during their homeostatic function. Both lipid metabolism (lipid droplets fusing with europium-containing vacuoles) and autophagy are involved in the cellular response to europium stress. Bioaccumulation, together with a possible biomineralization to europium phosphate, seems to be the main mechanism of Eu(III) detoxification in these cells.

Una visión integral del manejo de la hepatitis C y el objetivo de erradicarla / A comprehensive view of hepatitis C management and the goal of eradication

La hepatitis C es una enfermedad viral causada por el virus de la hepatitis C (VHC), que fue identificada por primera vez en 1989 por un equipo de científicos liderado por Michael Houghton en Chiron Corporation. Esta forma de hepatitis era conocida como "hepatitis no-A no-B", ya que no se podía identificar el agente infeccioso responsable. Puede afectar a personas de diferentes géneros y orientaciones sexuales, incluidos los hombres que tienen sexo con hombres (HSH); y su transmisión ocurre a través de situaciones en las que hay un intercambio de sangre, como el uso compartido de agujas o equipo para la inyección de drogas, o durante prácticas sexuales que pueden causar microlesiones en la mucosa anal. Es importante destacar que la hepatitis C también puede transmitirse a través de otras vías, como la transfusión de sangre no segura, la exposición a instrumentos médicos contaminados, o el compartir objetos personales que puedan tener sangre infectada.

Hepatitis C is a viral disease caused by the hepatitis C virus (HCV), which was first identified in 1989 by a team of scientists led by Michael Houghton at Chiron Corporation. This form of hepatitis was known as "non-A non-B hepatitis" as the infectious agent responsible couldn't be identified. It can affect individuals of different genders and sexual orientations, including men who have sex with men (MSM); transmission occurs through situations involving blood exchange, such as needle sharing or equipment for drug injection, or during sexual practices that may cause microlesions in the anal mucosa. It's important to note that hepatitis C can also be transmitted through other routes, such as unsafe blood transfusion, exposure to contaminated medical instruments, or sharing personal items that may have infected blood.

Associations among environmental exposure to trace elements and biomarkers of early kidney damage in the pediatric population.

BACKGROUND: In kidney damage, molecular changes can be used as early damage kidney biomarkers, such as Kidney Injury Molecule-1 and Neutrophil gelatinase-associated lipocalin. These biomarkers are associated with toxic metal exposure or disturbed homeostasis of trace elements, which might lead to serious health hazards. This study aimed to evaluate the relationship between exposure to trace elements and early damage kidney biomarkers in a pediatric population. METHODS: In Tlaxcala, a cross-sectional study was conducted on 914 healthy individuals. The participants underwent a medical review and a socio-environmental questionnaire. Five early damage kidney biomarkers were determined in the urine with Luminex, and molybdenum, copper, selenium, nickel, and iodine were measured with ICP-Mass. RESULTS: The eGFR showed a median of 103.75 mL/min/1.73 m2. The median levels for molybdenum, copper, selenium, nickel, and iodine were 24.73 ng/mL, 73.35 ng/mL, 4.78 ng/mL, 83.68 ng/mL, and 361.83 ng/mL, respectively. Except for molybdenum and nickel, the other trace elements had significant associations with the eGFR and the early kidney damage biomarkers. Additionally, we report the association of different exposure scenarios with renal parameters. DISCUSSION: and Conclusions. Among the explored metals, exposure to Cu and iodine impairs renal function. In contrast, Se may manifest as a beneficial metal. Interactions of Mo-Se and Mo-Iodine seem to alter the expression of NGAL; Mo-Cu for CLU; Mo-Cu, Mo-Se, and Mo-iodine for Cys-C and a-1MG; and Mo-Cu and Mo-iodine for KIM-1; were noticed. Our study could suggest that trace element interactions were associated with early kidney damage biomarkers.

Middle Meningeal Artery Embolization in Acute Leukemia Patients Presenting With Subdural Hematoma.

Intracerebral hemorrhage is a potentially fatal complication in patients with acute leukemia and contributing factors include thrombocytopenia and coagulopathy. Patients with acute leukemia may develop subdural hematoma (SDH) spontaneously or secondary to trauma. In patients with acute leukemia and SDH, the surgical evacuation of the hematoma causes significant morbidity and mortality. New approaches and strategies to reduce the need for surgical evacuation are needed to improve outcomes in patients with acute leukemia and intracerebral hemorrhage. We report two cases of acute SDH in patients with acute leukemia successfully treated with middle meningeal artery embolization, a minimally invasive interventional radiology technique, obviating the need for a surgical intervention. The first patient with acute promyelocytic leukemia (APL) presented with coagulopathy and developed an acute SDH after a fall. The second patient with acute myeloid leukemia presented with gum bleeding and also sustained an acute SDH after a fall. Both patients underwent middle meningeal artery embolization for treating their SDHs while actively receiving induction chemotherapy for acute leukemia. Both patients had resolution of their acute SDH and are in remission from their acute leukemia. Middle meningeal artery embolization is a very effective, and within the context of this setting, a novel, minimally invasive technique for management of SDH in acute leukemia patients, which can prevent the need for surgical interventions with its associated comorbidities and high risk of fatal outcomes in patients with acute leukemia and acute SDH.

Middle meningeal artery embolization reduces recurrence following surgery for septated chronic subdural hematomas.

BACKGROUND: Septated chronic subdural hematomas (cSDH) have high rates of recurrence despite surgical evacuation. Middle meningeal artery embolization (MMAE) has emerged as a promising adjuvant for secondary prevention, yet its efficacy remains ill-defined. METHODS: This is a retrospective review of septated cSDH cases treated at our institution. The surgery-only group was derived from cases performed before 2018, and the surgery+MMAE group was derived from cases performed 2018 or later. The primary outcome was reoperation rate. Secondary outcomes were recurrence, change in hematoma thickness, and midline shift. RESULTS: A total of 34 cSDHs in 28 patients (surgery+MMAE) and 95 cSDHs in 83 patients (surgery-only) met the inclusion criteria. No significant difference in baseline characteristics between groups was identified. The reoperation rate was significantly higher in the surgery-only group (n = 16, 16.8%) compared with the surgery+MMAE cohort (n = 0, 0.0%) (p=0.006). A reduced incidence of recurrence (p=0.011) was also seen in the surgery+MMAE group. CONCLUSIONS: MMAE for septated cSDH was found to be highly effective in preventing recurrence and reoperation. MMAE is an adjunct to surgical evacuation may be of particular benefit in this patient cohort.

Assessing coagulopathy and endothelial dysfunction in pediatric venous malformation: A thromboelastometry and syndecan-1 study.

OBJECTIVE: The occurrence of unpredictable pain crises are the principal determinant of the quality of life for patients with venous malformations (VM). A definite coagulation phenomenon, characterized by an increase in D-dimer levels and the presence of phleboliths within the malformation, has been previously reported. By applying Virchow's triad and evaluating intralesional samples, our objective is to delineate the coagulation profile and the extent of endothelial dysfunction within the malformation. METHODS: With the authorization of the Ethics Committee, a research project was undertaken on intralesional and extralesional blood samples from 30 pediatric patients afflicted with spongiform VM. Thromboelastometry analyses were performed using ROTEM Sigma, and the concentration of syndecan-1 was determined by ELISA. RESULTS: In the ROTEM analyses, the A5, A10, and maximum clot firmness (MCF) values were below the established reference ranges in the intralesional samples in both the EXTEM and INTEM assays, indicating that intralesional clots had significant instability. Furthermore, during the investigation of the delayed fibrinolysis phase using recombinant tissue plasminogen activator (rtPA) in EXTEM analysis, widespread hyperfibrinolysis was observed intralesional. Additionally, analysis of syndecan-1 showed significant differences between extralesional and intralesional levels (p < .026) and controls (p < .03), suggesting differences in the state of endothelium. CONCLUSIONS: For the first time, we developed a comprehensive understanding of the coagulopathic profile of VM and the role of endothelial dysfunction in its pathogenesis. These findings will enable the implementation of targeted therapies based on the individual coagulation profiles.

Efficacy and Safety of the Extreme Lateral Interbody Fusion (XLIF) Technique in Spine Surgery: Meta-Analysis of 1409 Patients.

(1) Objectives: The objective of this study was to quantify the exact clinical-radiological efficacy and safety of the extreme lateral interbody fusion (XLIF) technique in spinal surgery; (2) Methods: A meta-analysis was performed using PubMed, Embase, Scopus, and Cochrane Collaboration Library. Studies focusing on patients surgically treated with XLIF were included. The outcomes were as follows: visual analog scale (VAS) and Oswestry disability index (ODI), radiological outcomes, and adverse events. Cohort studies and case series were also included. Clinical outcomes were assessed at 12 months of age. Data were combined using Review Manager 5.4 and WebPlotDigitizer 13.1.4; (3) Results: Nineteen studies with a pool of 1409 patients were included in this meta-analysis. Leg pain VAS and back pain VAS significantly improved at 12 months (SMD 2.75, 95% CI 0.59-4.90; SMD 4.54, 95% CI 1.39-7.69). ODI showed significant improvement (MD 32.51, 95% CI 24.01-41.00) at 12 months. Disc height increased significantly (SMD -2.73, 95% CI -3.58 to -1.88). Lumbar lordosis and segmental lordosis were significantly corrected postoperatively (MD -2.44, 95% CI -3.45 to -1.43; MD -2.55, 95% CI -3.61 to -1.48). The fusion rates at 12 months ranged from 85.0% to 93.3%. The most frequent complications were transient neurological conditions (2.2%), hardware failure (1.9%), and transient pain (1.8%). The most frequent serious complications were nerve root injury (1.0%), gastrointestinal impairment (0.7%), and vertebral fractures (0.6%); (4) Conclusions: This is the first meta-analysis of the specific use of XLIF in spinal surgery. This study demonstrates that the XLIF technique in spine surgery is associated with good clinical and radiological results and a low complication rate.

La piel como reflejo de trastornos hepáticos subyacentes / The skin as a reflection of underlying liver disorders

La piel es el órgano más grande y visible del cuerpo humano; en ella se pueden reflejar diversos hallazgos de enfermedades sistémicas, incluidas las hepatopatías crónicas y agudas, las cuales se asocian a múltiples lesiones dermatológicas como principal manifestación extrahepática. Las manifestaciones cutáneas son comunes pero inespecíficas y pueden encontrarse en diferentes enfermedades; por lo tanto, la piel funciona como una ventana a nuestra salud general, de ahí que el examen clínico de la piel, las uñas y el cabello pueda permitir el reconocimiento adecuado, el diagnóstico y tratamiento temprano de las enfermedades hepáticas y la mejoría de la calidad y esperanza de vida de los pacientes afectados.

La piel es el órgano más grande y visible del cuerpo humano; en ella se pueden reflejar diversos hallazgos de enfermedades sistémicas, incluidas las hepatopatías crónicas y agudas, las cuales se asocian a múltiples lesiones dermatológicas como principal manifestación extrahepática. Las manifestaciones cutáneas son comunes pero inespecíficas y pueden encontrarse en diferentes enfermedades; por lo tanto, la piel funciona como una ventana a nuestra salud general, de ahí que el examen clínico de la piel, las uñas y el cabello pueda permitir el reconocimiento adecuado, el diagnóstico y tratamiento temprano de las enfermedades hepáticas y la mejoría de la calidad y esperanza de vida de los pacientes afectados.

Lymphatic Malformations in Parkes Weber's Syndrome: Retrospective Review of 16 Cases in a Vascular Anomalies Center.

INTRODUCTION: Parkes Weber's syndrome (PWS) is a rare genetic disorder characterized by overgrowth and vascular malformations, primarily affecting the extremities. While PWS is known to be associated with arteriovenous and capillary malformations, the potential involvement of lymphatic malformations (LMs) has not been previously reported. The objective of this study is to investigate the presence of lymphatic anomalies in PWS patients and their role in the development of limb asymmetry. MATERIALS AND METHODS: This is a retrospective study of patients diagnosed with PWS in a Vascular Anomalies Center from 1994 to 2020. Clinical data were obtained from medical records including diagnostic imaging, lymphoscintigraphy, and genetic testing. The Institutional Review Board and Ethics Committee have approved this study. RESULTS: A total of 16 patients aged 18 interquartile range 14.7 years diagnosed with PWS were included (50% female). Six of the 16 patients with PWS had clinical and imaging data suggestive of LM (37.5%) and 3 of them had genetic variants in RASA1 (2/3) or KRAS (1/3). Limb asymmetry was greater in patients with isolated PWS (2.6 ± 0.8 cm) than in the PWS-lymphatic anomalies population (2 ± 0.7 cm), although not significant (p = 0.247). One in 6 patients with PWS-LM required amputation (16.6%) versus 1 in 10 in isolated PWS (10%). CONCLUSION: Lymphatic anomalies may be present in a significant number of patients with PWS and could have a role in limb asymmetry and outcomes. It is paramount to investigate their existence and distinguish them from true overgrowth.

Risk factors for sequelae after surgery for lymphatic malformations in children.

OBJECTIVE: The first-line treatment of lymphatic malformations (LMs) is pharmacological or interventional; however, surgery is still necessary in selected cases. Our aim was to identify factors associated with the occurrence of permanent postoperative complications. METHODS: This was a case series study of children operated on for LMs between 2001 and 2021 and followed-up in our institution. Patients who presented sequelae derived from surgical treatment (cases) and those who did not (controls) were compared. RESULTS: We included 112 children who underwent surgery for LMs in different centers. Forty-nine cases and 63 controls were included (58% male), with a mean age of 34 months. Patients younger than 1 year presented more complications than older children, 59% (n = 29/49) vs 41% (n = 24/49), respectively (P = .02). LMs were in the cervicofacial region in seven patients in the control group compared with 30 of the cases (P ≤ .001), with microcystic malformations the most associated with sequelae (n = 11/15; P = .019). Concerning permanent complications, 88% were neurological (n = 43/49), mainly peripheral facial palsy (n = 17). There was greater postoperative residual disease in controls compared with cases (65% vs 14%, respectively; P ≤ .0001). However, following a second procedure in the control group, there was no significant difference in long-term cure rates (P = .38). CONCLUSIONS: The risk of sequelae following surgery for LM increases significantly in patients younger than 12 months in cervicofacial and microcystic malformations. Because non-radical resections are associated with fewer complications and an optimal long-term cure rate, we consider that aggressive surgical approaches should be avoided if the absence of sequelae is not guaranteed.

MicroRNAs in Tetrahymena thermophila: An epigenetic regulatory mechanism in the response to cadmium stress.

Among the epigenetic mechanisms based on non-coding RNA are microRNAs (miRNAs) that are involved in the post-transcriptional regulation of mRNAs. In many organisms, the expression of genes involved in the cellular response to biotic or abiotic stress depends on the regulation, generally inhibitory, performed by miRNAs. For the first time in the eukaryotic microorganism (ciliate-model) Tetrahymena thermophila, miRNAs involved in the post-transcriptional regulation of transcripts linked to the response to cadmium have been isolated and analyzed. Forty de novo miRNAs (we named tte-miRNAs) have been isolated from control and Cd-treated populations (1 or 24 h exposures). An exhaustive comparative analysis of the features of these mature tte-miRNAs and their precursor sequences (pre-tte-miRNAs) confirms that they are true miRNAs. In addition to the three types of miRNA isoforms previously described in other organisms, two new types are also described among the tte-miRNAs studied. A certain percentage of the pre-tte-miRNA sequences are in introns from genes with many introns, and have been defined as 5', 3'-tailed mirtrons. A qRT-PCR analysis of selected tte-miRNAs together with some of their targets has validated them. Cd is one of the most toxic metals for the cell, which must defend itself against its toxicity by various mechanisms, such as expulsion by membrane pumps, chelation by metallothioneins, among others. Like other toxic metals, Cd also causes a well-known series of cellular effects such as intense proteotoxicity. Many of the targets that are regulated by the tte-miRNAs are transcripts encoding proteins that fit into these defense mechanisms and toxic metal effects.

Utility of a clinical risk scale to predict the requirement of advanced airway management in patients with a diagnosis of deep neck abscess,

Abstract Objectives To analyze the clinical utility of a clinical risk scale to predict the need for advanced airway management in patients with deep neck abscess. Methods Observational, analytical, cross-sectional study. Patients over 18 years old, both genders, with surgical management of a deep neck abscess, between January 1st, 2015 to December 31th, 2021, who were applied the clinical risk scale (https://7-414-5-19.shinyapps.io/ClinicalRiskScore/). The sensitivity, specificity, and predictive values of the scale were calculated based on the identified clinical outcomes. A p < 0.05 was considered significant. Results A sample of 213 patients was obtained, 121 (56.8%) men, of whom 50 (23.5%) required advanced airway management. Dyspnea was the variable with the most statistical weight in our study, (p = 0.001) as well as the multiple spaces involvement, (p = 0.001) the presence of air corpuscles, (p = 0.001) compromise of the retropharyngeal space (p = 0.001) and age greater than 55 years (p = 0.001). Taking these data into account, were found for the clinical risk scale a sensitivity of 97% and a specificity of 65% (p = 0.001, 95% CI 0.856-0.984). Conclusions The clinical risk scale developed to predict advanced airway management in patients with a diagnosis of deep neck abscess may be applicable in our environment with high sensitivity and specificity. Level of evidence: IV.

Alpelisib decreases nevocytes of congenital melanocytic nevi.

BACKGROUND: Multiple, large or giant congenital melanocytic nevi (CMN) are uncommon and affected patients can show progressive growth and thickening, associate neurocutaneous melanocytosis or develop melanoma. Current treatment modalities are mostly complex surgeries that frequently do not solve the disease and its risks completely. Thus, investigation on new treatment options for CMN and its complications must continue. MAPK pathway inhibitors are being investigated, also targeting PI3K-AKT. Omipalisib (PI3K inhibitor, with no indications approved yet) has been studied for CMN in vitro and in mice with promising results. However, alpelisib, a PI3K inhibitor approved with an adequate safety profile for patients with severe manifestations of PROS (PIK3CA-Related Overgrowth Spectrum), had not yet been tested for CMN. OBJECTIVE: To evaluate the effect of alpelisib in nevocytes of congenital melanocytic nevi. METHODS: Nevomelanocytic tissue samples of 10 patients were collected prospectively and, following a previously reported preclinical ex vivo model, explants were placed in organotypic culture for 5 days, with or without alpelisib. Consecutively, tissue sections were stained and using scanned images with Qupath and ImageJ softwares, representative regions from the dermis were analysed (using Wilcoxon test and Spearman's correlation). RESULTS: When comparing alpelisib-treated explants with respect to control explants, we found a decrease in cell density (p = 0.0273), in density of SOX10+ -cells (p = 0.0391) and also in the % of S-100+ area (p = 0.0078), in alpelisib samples. The three markers showed a positive correlation (p < 0.05). CONCLUSIONS: This study provides first-time evidence that alpelisib induces nevocyte reduction in CMN from patient-derived explants, probably inducted by autophagy. Alpelisib is an approved drug with an adequate safety profile used in another mosaicism affecting PI3K (PROS). Further studies are needed to evaluate its efficacy in treating CMN and potentially, their complications, either with local or systemic administration, alone or in combination.

Activity of a TIE2 inhibitor (rebastinib) in a patient with a life-threatening cervicofacial venous malformation.

Targeted therapy has become the first therapeutic option in many patients with vascular anomalies. A male 28-year-old patient presented a severe cervicofacial venous malformation involving half-lower face, anterior neck, and oral cavity with progression despite multiple previous treatments, with a somatic variant in TEK (endothelial-specific protein receptor tyrosine kinase) (c.2740C>T; p.Leu914Phe). The patient had facial deformity, daily episodes of pain and inflammation needing massive amount of medication, and difficulty in speech and swallowing, so rebastinib (a TIE2 kinase inhibitor) was approved for compassionate use. After 6 months of treatment, venous malformation had decreased in size and lightened, as well as improved quality-of-life scores.

Successful Sirolimus Treatment for Recurrent Pericardial Effusion in a Large Cervicomediastinal Provisionally Unclassified Vascular Anomaly: A Case Report.

Provisionally unclassified vascular anomalies (PUVA) are a group of diseases with unique characteristics that make them unclassifiable within vascular tumors or malformations. We describe a PUVA as the cause of recurrent pericardial effusion and its response to sirolimus. A 6-year-old girl was referred with a cervicothoracic vascular anomaly, a violaceous, and irregular lesion in the neck and upper chest, diagnosed as "hemangioma". She had pericardial effusion at the neonatal age that required pericardiocentesis, propranolol, and corticosteroids. She remained stable for 5 years, when she presented with a severe pericardial effusion. A magnetic resonance visualized a diffuse vascular image in the cervical and thoracic region with mediastinal extension. The pathological study showed a vascular proliferation in the dermis and hypodermis with positive staining for Wilms' Tumor 1 Protein (WT1) and negative for Glut-1. Genetic testing found a variant in GNA14 , for which the diagnosis of PUVA was established. When a pericardial drain was placed without response, treatment with sirolimus was started with resolution of the effusion. Sixteen months later, the malformation is stable and there has been no recurrence of pericardial effusion. In a significant group of patients, definitive diagnosis is not possible despite pathological and genetic analysis. Mammalian target of rapamycin inhibitors may become a therapeutic option if symptoms are severe enough, with a low rate of reported side effects.

Therapeutic strategies and clinical evolution of patients with infantile fibrosarcoma: a unique paediatric case series.

BACKGROUND: Infantile fibrosarcoma is the most frequent soft tissue sarcoma in newborns or children under one year of age. This tumour often implies high local aggressiveness and surgical morbidity. The large majority of these patients carry the ETV6-NTRK3 oncogenic fusion. Hence, the TRK inhibitor larotrectinib emerged as an efficacious and safe alternative to chemotherapy for NTRK fusion-positive and metastatic or unresectable tumours. However, real-world evidence is still required for updating soft-tissue sarcoma practice guidelines. OBJECTIVE: To report our experience with the use of larotrectinib in pediatric patients. METHODS: Our case series shows the clinical evolution of 8 patients with infantile fibrosarcoma under different treatments. All patients enrolled in this study received informed consent for any treatment. RESULTS: Three patients received larotrectinib in first line. No surgery was needed with larotrectinib, which led to the rapid and safe remission of tumours, even in unusual anatomical locations. No significant adverse effects were observed with larotrectinib. CONCLUSION: Our case series supports that larotrectinib may be a therapeutic option for newborn and infant patients with infantile fibrosarcoma, especially in uncommon locations.