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Abstract: Carbon capture and geological storage are among the most valuable technologies capable of reducing CO2 emissions. Long‐term interactions between CO2 and a reservoir, and the integrity of geological formations, are key factors in the selection of adequate reservoirs for permanent storage. Numerical models of CO2‐water‐rock geochemical interactions are often employed to predict the fate of CO2 stored in a reservoir over time. The Hygiene Sandstone, in the Denver Basin, Colorado, USA, is a geological formation with potential for CO2 storage, and was therefore studied in this work, in which we collected and characterized outcrop samples in order to supply the input parameters for numerical simulations. Four representative thin sections of Hygiene Sandstone outcrops were quantified in terms of detrital constituents, diagenesis, and porosity on the basis of conventional petrography. Sandstone mineralogy included, in decreasing order, quartz, K‐feldspar, muscovite, albite, illite, smectite, kaolinite, poikilotopic calcite, and siderite. Porosity ranged from 4% to 13%. A geochemical modeling study of CO2‐water‐rock interactions performed with two characterized samples and brine data from the Hygiene Sandstones, simulating reservoir conditions, suggested that the mineralogy of the sandstone is quite stable under the conditions that were tested and only minor mineralogical and porosity alterations would occur within a thousand years of storage. © 2018 Society of Chemical Industry and John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Carbon dioxide injection in geological formations is currently a common procedure in several reservoirs worldwide. More recently, it has been considered a permanent storage solution, avoiding emission to the atmosphere from large industrial sources. Also, it is largely employed in the oil & gas exploration industry, for enhanced oil recovery (EOR) operations. However, it is a known fact that injection of large amounts of CO2 into geological reservoirs may lead to a series of alterations due to chemical and physical interactions with minerals and fluids, especially in carbonate or carbonate-rich reservoirs. Experimental and numerical models have been employed in many studies in the past, to investigate these effects on the geological environment. So far, most of these studies focused on siliciclastic formations, whereas carbonate reservoirs, which are known to be much more chemically reactive when interacting with CO2, were much less investigated. We present a review of experimental and numerical models that have been employed for studying CO2-water-rock interactions, and their application to the investigation of the impact in carbonate reservoir quality and integrity caused by the injection of carbon dioxide. © 2017 Society of Chemical Industry and John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Brazil is recognized for possessing a low carbon-intensive energy matrix, with most of its power being generated from hydroelectricity. Its greenhouse gas emissions profile is dominated by deforestation and land-use change. Despite this characteristic, the country has been committed to the development of carbon capture and geological storage (CCS) research since this technology started to be recognized as a relevant solution for greenhouse gas emission reductions. This development has gained attention recently owing to the beginning of the production of pre-salt reservoirs, which may contain significant amounts of CO2 in the produced fluids. The work has been carried out mostly through the efforts of the academia and industrial enterprises. This paper presents a summary and a brief description of the recent activities being carried out by these sectors, aiming to develop and promote CCS in Brazil. [ABSTRACT FROM AUTHOR]

A study of the rift to early drift phase, fluvial, deltaic and shallow-marine Lower Cretaceous (Upper Aptian to Lower Albian) siliciclastic sandstones, hybrid arenites and calcarenites from the Jequitinhonha Basin, eastern Brazilian margin, has shown that the distribution of diagenetic alterations and of related reservoir quality evolution can be constrained within a sequence stratigraphic framework. Description of cores, wireline logs, thin sections and petrophysical (porosity and permeability) analyses were integrated in order to investigate the controls on patterns of diagenesis in these rocks, and hence of reservoir quality distribution. The results of this study will be relevant to the exploration of rift and early drift successions in Atlantic-type passive margin settings. The study suggests that depositional facies and detrital composition impact the diagenetic evolution and the characterization and prediction of reservoir quality. Reservoir quality assessment was based on the concept of reservoir petrofacies, which is defined in terms of the main attributes affecting reservoir quality including depositional structures, textures, primary composition, diagenetic processes and products, and pore types. This paper demonstrates the practical use of the reservoir petrofacies concept in the evaluation of the controls on porosity and permeability, as well as on seismic and log signatures. [ABSTRACT FROM AUTHOR]

The reservoir quality of Jurassic and Triassic fluvial and lacustrine-deltaic sandstones in the intracratonic Ordos Basin is strongly influenced by depositional facies and various types of diagenetic modifications. The fluvial sandstones have higher average He-porosity and permeability (14.8% and 12.7 mD, respectively) than the deltaic sandstones (9.8% and 5.8 mD, respectively). In addition to extensive mechanical compaction, eodiagenesis (220–97 Ma; depth < 2000 m; T < 70°C) has resulted in dissolution and kaolinitization of detrital silicates in the Jurassic fluvial sandstones, and in smectite infiltration and minor cementation by calcite and siderite in the Triassic fluvial and deltaic sandstones. Pervasive eogenetic carbonate cementation (> 20 vol.%) occurred in Triassic deltaic siltstones and very fine-grained sandstones which are closely associated with organic-rich mudstones. Mesodiagenesis (97–65 Ma), which occurred during rapid subsidence to depths of 3700–4400 m, resulted in the albitization of plagioclase, checmical compaction, the conversion of kaolinite into dickite, and cementation by quartz overgrowths, chlorite, illite, ankerite (δ13CVPDB=−2.4‰ to +2.6‰; δ18OVPDB=−21.5‰ to −10‰) and calcite (δ13CVPDB=−4.7‰ to +3.7‰; δ18OVPDB=−21.8‰ to −13.4‰). Oil emplacement (95 Ma) retarded cementation by mesogenetic quartz and carbonate but had little influence on dickite, illite and chlorite formation. Retardation of quartz cementation was also due to the presence of chlorite fringes around detrital quartz grains. Dickitization of eogenetic kaolinite together with the short residence time at maximum burial temperatures (105–124°C) has retarded the albitization of K-feldspars and illite formation and hence prevented severe permeability destruction. Telodiagenesis, which occurred after uplift (Eocene to end-Neogene), caused slight dissolution and kaolinitization of feldspars. This study demonstrates that despite complex patterns of diagenetic modifications in the Triassic and Jurassic successions, depositional porosity and permeability are better preserved in fluvial meandering channel sandstones than in deltaic sandstones. These results should be important for modelling of reservoir-quality distribution and exploration risk evaluation in the basin. [ABSTRACT FROM AUTHOR]

Turbidite sandstone reservoir rocks in the Campos Basin, offshore eastern Brazil, show evidence of a number of interactions among organic and mineral phases during diagenesis. These include extensive dissolution of plagioclase, precipitation of kaolinite into intergranular and intragranular pores, dissolution of carbonate cements and grains, as well as selective deposition of organic films on kaolinite aggregates, dissolved plagioclase and carbonate grains. These processes are connected with the biodegradation of hydrocarbons within the reservoir, related to the influx of meteoric fluids possibly during sea-level lowstands, through major stratigraphic sequence boundaries and large-scale faults. Oil biodegradation generated organic solvents responsible for mineral alteration, as well as heavy oils rich in asphaltenes. The action of organic solvents in the reservoirs is indicated by the extensive dissolution of feldspars, quartz, garnets and kaolinite, and the negative mass balance between dissolved feldspar and precipitated kaolinite, indicating aluminum mobilization. Asphaltene films and coatings may affect the wettability of the reservoir rocks and the evaluation of hydrocarbon saturation in the reservoirs by resistivity logs. These various inorganic and organic processes are apparently interrelated in the diagenesis of the Campos Basin turbidite reservoir sandstones. These interactions should be considered in future models of the basin and its petroleum system, as well as for the creation of geochemical models capable of predicting the quality of the reservoirs and the occurrence of degraded heavy oils. [ABSTRACT FROM AUTHOR]

Discusses the use of Kaolinitic meniscus bridges as an indicator of early diagenesis in Nubian sandstones in Sinai, Egypt. Optical photomicrograph; Timing of kaolinization; Implication for porosity evolution; Color of the crystalline menisci aggregates; Coarsely crystalline accordion formation.

Abstract Linking siliciclastic diagenesis to sequence stratigraphy allows a better understanding of the parameters controlling the spatial and temporal distribution of diagenetic alterations, and hence of reservoir quality. A study of the coal-bearing, alluvial, deltaic, estuarine and shallow-marine sandstones of the Rio Bonito Formation, early Permian, Paraná Basin (southern Brazil), reveals that the distribution of diagenetic alterations and of related reservoir quality evolution can be constrained within a sequence stratigraphic framework. Calcite, dolomite, siderite, kaolinite and pyrite cementation is consistently linked to sequence and parasequence boundaries, transgressive and maximum flooding surfaces and is systematically distributed within lowstand, transgressive and highstand systems tracts. Diagenesis of coal layers at parasequence boundaries has promoted the formation of stratabound calcite (detectable in resistivity wire line logs), concretionary pyrite and kaolinite and of silicate grain dissolution in sandstones located above and below these boundaries, particularly in the transgressive systems tract. Meteoric water diagenesis caused grain dissolution and the formation of kaolinite in sandstones below sequence boundaries and in lowstand systems tract sandstones. Carbonate bioclasts and low sedimentation rates in lag deposits at parasequence boundaries, transgressive and maximum flooding surfaces favoured the formation of grain-rimming siderite. The results of this study are relevant to the exploration of coal-bed methane and other coal-bearing reservoirs, where it is crucial to unravel and predict the distribution and quality of reservoirs and compartments. [ABSTRACT FROM AUTHOR]

The spatial and temporal distribution of diagenetic alterations in siliciclastic sequences is controlled by a complex array of interrelated parameters that prevail during eodiagenesis, mesodiagenesis and telodiagenesis. The spatial distribution of near-surface eogenetic alteration is controlled by depositional facies, climate, detrital composition and relative changes in sea-level. The most important eogenetic alterations in continental sediments include silicate dissolution and the formation of kaolinite, smectite, calcrete and dolocrete. In marine and transitional sediments, eogenetic alterations include tile precipitation of carbonate, opal, microquartz, Fe-silicates (glaucony, berthierine mad nontronite), sulphides and zeolite. The eogenetic evolution of marine and transitional sediments can probably be developed within a predictable sequence stratigraphic context. Mesodiagenesis is strongly influenced by the induced eogenetic alterations, as well as by temperature, pressure and tile composition of basinal brines. The residence time of sedimentary sequences under certain burial conditions is of key importance in determining the timing, extent and patterns of diagenetic modifications induced. The most important mesogenetic alterations include feldspar albitization, illitization and c.hloritization of smectite and kaolinite, dickitization of kaolinite, chemical compaction as well as quartz and carbonate cementation. Various aspects of deep-burial mesodiagenesis are still poorly understood, such as: (i) whether reactions are accomplished by active fluid flow or by diffusion; (ii) the pattern mad extent of mass transfer between mudrocks and sandstones; (iii) the role of hydrocarbon emplacement on sandstone diagenesis; mad (iv) the importance and origin of fluids involved in the formation of secondary inter- and intragranular porosity during mesodiagenesis. Uplift and incursion of meteoric waters induce telogenetic alterations that include kaolinitization and carbonate-cement dissolution down to depths of tens to a few hundred metres below the surface. [ABSTRACT FROM AUTHOR]

Abstract: Although coal is not the primary source of energy in Brazil there is growing interest to evaluate the potential of coal from the south of the country for various activities. The I2B coal seam in the Charqueadas coal field has been considered a target for enhanced coal bed methane production and CO2 sequestration. A detailed experimental study of the samples from this seam was conducted at the NETL with assistance from the Pontifícia Universidade Católica Do Rio Grande Do Sul. Such properties as sorption capacity, internal structure of the samples, porosity and permeability were of primary interest in this characterization study. The samples used were low rank coals (high volatile bituminous and sub-bituminous) obtained from the I2B seam. It was observed that the temperature effect on adsorption capacity correlates negatively with as-received water and mineral content. Langmuir CO2 adsorption capacity of the coal samples ranged 0.61–2.09mmol/g. The upper I2B seam appears to be overall more heterogeneous and less permeable than the lower I2B seam. The lower seam coal appears to have a large amount of micro-fractures that do not close even at 11MPa of confining pressure. [Copyright &y& Elsevier]

Background: This study aimed to examine the experiences of children with colorectal conditions who spent time in the Neonatal Intensive Care Unit (NICU) and their caregivers., Methods: In March 2024, a 36-question survey was sent to the Colorectal Support Network Facebook community, to gather information from caregivers of children who have a congenital colorectal diagnosis and spent time in the NICU., Results: Fifty-two families completed the survey. Most patients were diagnosed after birth (89.47%). Approximately half of respondents stayed in the NICU for one to two weeks (50.88%), lived less than 60 minutes away from the hospital (54.91%), and felt somewhat uncomfortable (28.07%) or very uncomfortable (21.05%) caring for their child's medical needs after discharge. Also 49.12% of caregivers were informed of their child's future bowel control prognosis. When asked for suggestions to improve care in the NICU, common themes included the importance of having colorectal congenital anomalies addressed by colorectal surgeons, and the need for families to be informed about support groups., Conclusions: Counseling families in the NICU with congenital colorectal conditions can be improved by providing additional information and support for families prior to discharge, informing them about their child's prognosis for bowel control, and connecting them with other families., Competing Interests: The authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

Background: Chronic constipation and fecal incontinence are devastating problems for patients with anorectal malformations, Hirschsprung's disease, idiopathic constipation, and spina bifida/spinal cord injuries. Finding the proper regimen allows these patients to be out of diapers and free of stool accidents. A typical bowel management regimen consists of daily laxatives or enemas; because these products are sold over the counter, insurance companies do not cover them. This study reviews the cost of bowel regimens and analyzes their economic impact on the families we treat., Methods: A retrospective review of patients undergoing bowel management between January 2016 and September 2023 was done. The mean annual income of families was calculated using their zip codes., Results: Upon review, 430 patients met inclusion criteria; 167 were on laxatives, and 263 were using enemas. There was significant variation in the cost of medications based on the distributors they were bought from and the dose of the medication in their regimen. The cost of laxatives ranged from $15.70 to $2938.10 annually. The cost of enemas containing glycerin ranged from $29.20 to $4380.00 annually. In comparison, diapers/incontinence briefs ranged from $131.40 to $4343.50 annually. The median annual income of patients across 41 states ranged from $32,192 to $225,119., Conclusions: Our findings emphasize the importance of proper counseling families regarding medication costs in the market, intending to promote long-term treatment adherence. They also serve as a data source to advocate for improved insurance coverage of the medications required to manage these chronic conditions successfully., Level of Evidence: IV., Competing Interests: Conflict of interest The authors have no conflict of interests or competing interests to declare., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Introduction: Epithelioid hemangioendothelioma (EHE) is an ultra-rare sarcoma, marked by distinctive molecular and pathological features and with a variable clinical behavior. Its natural history is still partially understood, reliable prognostic and predictive factors are lacking and many questions are still open on the optimal management. In the context of EURACAN, a prospective registry specifically dedicated to EHE was developed and launched with the aim of providing, through high-quality prospective data collection, a better understanding of this disease., Study Design: Registry-based cohort study including only new cases of patients with a pathological and molecularly confirmed diagnosis of EHE., Objectives: To improve the understanding of EHE natural history, validate and identify new prognostic and predictive factors, clarify the activity and efficacy of currently available treatment options, describe treatment pattern., Methods: Settings and participantsIt is an hospital-based registry established in centers with expertise in EHE including adult patients with a new pathological and molecularly confirmed diagnosis of EHE starting from the 1st December 2023. The characteristics of each patient in the facility who meets the above-mentioned inclusion criteria will be collected prospectively and longitudinally with follow-up at cancer progression and / or cancer relapse or patient death. It is a secondary use of data which will be collected from the clinical records. The data collected for the registry will not entail further examinations or admissions to the facility and/or additional appointments to those normally provided for routine patient follow-up. VariablesFull details on patients and disease features, treatment and outcome will be collected, according to common clinical practice guidelines developed and shared with all the contributing centers. In addition, data on potential confounders (e.g. comorbidity; functional status etc.) will also be collected. Statistical methodsThe data analyses will include descriptive statistics and analytical analyses. Multivariable Cox's proportional hazards model and Hazard ratios (HR) for all-cause or cause-specific mortality will be used to determine independent predictors of overall survival, recurrence and progression., Results: The registry has been joined by 21 sarcoma reference centers across EU and UK, covering 10 countries. Patients' recruitment started in December 2023. The estimated completion date is December 2033 upon agreement on the achievement of all the registry objectives. The already established collaboration and participation of EHE patient's associations involved in the project will help in promoting the registry and fostering accrual., Competing Interests: Anna Maria Frezza declares institutional research funding from Advenchen Laboratories, Amgen Dompé, AROG Pharmaceuticals, Ayala Pharmaceuticals, Bayer, Blueprint Medicines, Boehriger Ingelheim, Daiichi Sankyo, Deciphera, Eisai, Eli Lilly, Epizyme Inc, Foghorn Therapeutics Inc., Glaxo, Hutchison MediPharma Limited, Inhibrx, Inc., Karyopharm Pharmaceuticals, Novartis, Pfizer, PharmaMar, PTC Therapeutics, Rain Oncology, SpringWorks Therapeutics. Alexia Bertuzzi declares support for attending meeting and/or travels from Pharmamar and Istituto Gentili; support for scientific activities from Istituto Gentili. Antonella Brunello declares consulting fees or serving on advisory boards for Eli Lilly, Roche, GSK, Eisai, Pharmamar, Boehringer Ingelheim, Deciphera; payment or honoraria for educational events by GSK and Pharmamar; travel grants by Pharmamar, Istituto Gentili. Giacomo G. Baldi declares consulting fees from Eli Lilly, Pharmamar, AboutEvents; honoraria from Pharmamar, Eli Lilly, Glaxo Smith Kline, Merck Sharp & Dome, Eisai, IstitutoGentili; support for attending meetings and/or travels from Novartis, Pharmamar, Eli Lilly; participation on the advisory board from Pharmamar, Eli Lilly, Glaxo Smith Kline, Merck Sharp & Dome, Eisai. Paolo G. Casali declares institutional research funding from Advenchen Laboratories, Amgen Dompé, AROG Pharmaceuticals, Ayala Pharmaceuticals, Bayer, Blueprint Medicines, Boehriger Ingelheim, Daiichi Sankyo, Deciphera, Eisai, Eli Lilly, Epizyme Inc, Foghorn Therapeutics Inc., Glaxo, Hutchison MediPharma Limited, Inhibrx, Inc., Karyopharm Pharmaceuticals, Novartis, Pfizer, PharmaMar, PTC Therapeutics, Rain Oncology, SpringWorks Therapeutics. Virginia Ferraresi declares consultancy fees or honoraria from PharmaMar, Boehringer Ingelheim, Gentili e Serb Pharmaceuticals. Matilde Ingrosso reports institutional research funding from Advenchen Laboratories, Amgen Dompé, AROG Pharmaceuticals, Ayala Pharmaceuticals, Bayer, Blueprint Medicines, Boehriger Ingelheim, Daiichi Sankyo, Deciphera, Eisai, Eli Lilly, Epizyme Inc, Foghorn Therapeutics Inc., Glaxo, Hutchison MediPharma Limited, Inhibrx, Inc., Karyopharm Pharmaceuticals, Novartis, Pfizer, PharmaMar, PTC Therapeutics, Rain Oncology, SpringWorks Therapeutics. Laura Monteleone reports institutional research funding from Advenchen Laboratories, Amgen Dompé, AROG Pharmaceuticals, Ayala Pharmaceuticals, Bayer, Blueprint Medicines, Boehriger Ingelheim, Daiichi Sankyo, Deciphera, Eisai, Eli Lilly, Epizyme Inc, Foghorn Therapeutics Inc., Glaxo, Hutchison MediPharma Limited, Inhibrx, Inc., Karyopharm Pharmaceuticals, Novartis, Pfizer, PharmaMar, PTC Therapeutics, Rain Oncology, SpringWorks Therapeutics. Johanna Szkandera reports participation in advisory boards or invited speaker fees for PharmaMar, Bayer, Roche, Lilly, Amgen; travel expenses coverage from PharmaMar, Roche, Lilly, Amgen, Bristol Myers Squibb; research funding from PharmaMar, Roche, Eisai. Bruno Vincenzi reports consulting fees from Eisai, Lilly, Bayer, Deciphera, PharmaMar, Blueprint, Pfizer, GSK, Accord, Abbott and research support from PharmaMar, Novartis, Lilly Silvia Stacchiotti declares advisory board roles with Bayer, Boehringer, Daiichi, Ikena, Nec Oncology, Pharma Essentia, Regeneron, Servier; invited speaker roles for Bayer, Boehringer, Gentili, Pharmamar; institutional research funding from Advenchen Laboratories, Amgen Dompé, AROG Pharmaceuticals, Ayala Pharmaceuticals, Bayer, Blueprint Medicines, Boehriger Ingelheim, Daiichi Sankyo, Deciphera, Eisai, Eli Lilly, Epizyme Inc, Foghorn Therapeutics Inc., Glaxo, Hutchison MediPharma Limited, Inhibrx, Inc., Karyopharm Pharmaceuticals, Novartis, Pfizer, PharmaMar, PTC Therapeutics, Rain Oncology, SpringWorks Therapeutics. Carlo Cicala, Lorenzo D’Ambrosio, Angelo Paolo Dei Tos, Alessandro Gronchi, Nadia Hindi, Robin Jones, Iwona Lugowska, Julia Ketzer, Anna Mariuk-Jarema, Andrea Napolitano, Andri Papakonstantinou, Sandro Pasquali, Marta Sbaraglia, Federico Scolari, Elisa Crotti, Irene Benzonelli, Jean-Yves Blay, Christophe Bouvier, Javier Martin Broto, Hugh Leonard, Ninna Aggerholm-Pedersen, Andri Papakonstantinou, Giuseppe Bianchi, Federica Zuccheri, Paolo Baili, Simone Bonfarnuzzo, Domenico Campanacci, Armelle Dufresne, Sebastian Bauer, Serena Bazzurri, Stefano Gabellini, Claudia Giani, Vincenzo Giannusa, Melissa Gil-Sanjines, Teresa Grassani, Paolo Lasalvia, Stefan Lindskog, Salvatore Vizzaccaro, Nils Dieckmann, Alessandro Mazzocca, Andrei Ivanescu, Giuseppe Badalamenti, Carlo Morosi, Stephanie Elston, Kyetil Boye, Francesca Nardozza, Elisabetta Neri, Maria Nilsson and Annalisa Trama declares no conflict of interest., (Copyright: © 2024 Frezza et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Purpose: To evaluate individual and community sociodemographic factors that predict bowel regimen adherence in youth and young adults with Spina Bifida (SB) following participation in a bowel management program (BMP)., Methods: Participants were drawn from clinical cases seen through an International Center for Colorectal and Urogenital Care. Area deprivation index (ADI) scores were extracted from participant addresses and bowel regimen adherence data were collected from the electronic medical record (EMR)., Results: Participants' mean age was 8.06 years old, 51.7% were male, 72.4% white, 37.9% Hispanic, 56.9% government insurance, 89.7% myelomeningocele, 15.5% non-adherent. Average neighborhood disadvantage was 5.19 (SD:2.83, range:1-10). After controlling for variables correlated with adherence (p < .20), every one decile higher neighborhood disadvantage score was associated with a 48% decrease in the odds of being adherent (OR = 0.52, p = .005, 95% CI: - 101.90, - 0.21)., Conclusion: Our results suggest that neighborhood disadvantage is a strong predictor of medical adherence following a BMP, more so than other sociodemographic and health-related variables. These results may assist with identifying which individuals may be at higher risk for poor health outcomes due to neighborhood socioeconomic disadvantage and help health care systems intervene proactively., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Women born with anorectal malformation (ARM) or Hirschsprung disease (HD) may have impaired urologic function resulting in sequelae in adulthood. This study assessed and compared self-reported urinary outcomes in adult females born with ARM or HD to a reference population., Methods: This was an IRB approved, cross-sectional study of female-born patients with ARM or HD, who completed surveys between November 2021 and August 2022. Female patients between the ages of 18 and 80 years were included. Lower Urinary Tract Symptom Questionnaires were administered through REDCap and the responses were compared to a reference population using Chi-squared or Fisher's exact tests., Results: Sixty-six born female patients answered the questionnaires, two of them identified as non-binary. The response rate was 76%. Median age was 31.6 years. The majority were born with cloaca (56.3%), followed by other type of ARMs (28.1%), complex malformation (9.4%), and HD (6.3%). A history of bladder reconstruction was present for 26.6%. Catheterization through a channel or native urethra was present in 18.8%. Two had ureterostomies and were excluded from the analysis. Seven had chronic kidney disease or end-stage renal disease, three with a history of kidney transplantation. Patients with cloaca had significantly higher rates of urinary incontinence, urinary tract infection, and social problems due to impaired urological functioning, when compared to an age-matched reference population (Table 3)., Conclusion: This study emphasizes the need for a multi-disciplinary team that includes urology and nephrology following patients with ARM long term, especially within the subgroup of cloaca., Level of Evidence: III., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To present the functional results after a transanal proximal rectosigmoidectomy in patients with severe idiopathic constipation in which medical treatment has failed., Methods: Patients with severe idiopathic constipation who underwent transanal proximal rectosigmoidectomy (TPRS) at Children's Hospital Colorado between June 2019 and March 2024 were included in the study. We compared multiple pre- and post-operative outcome measures and the patient's bowel regimen before and after resection., Results: Fourteen patients underwent TPRS, 10 of whom were male. The average age at the time of surgery was 10.1 years (range 5-19). Seven patients have moderate to severe autism. Constipation-related clinic visits, family calls, procedural intervention, emergency room visits, and hospitalizations notably decreased frequency after TPRS. Laxative dosages and enema volume requirements were also reduced after surgery. Before surgery, all the patients suffered from daily fecal accidents, while post-operatively, all were completely free of stool accidents., Conclusion: In our experience, for patients who suffer from severe medically refractory idiopathic constipation, TPRS has provided improvement in their symptoms and decreased the complications inherent to this chronic disease. Parents and patients attest to a profound positive transformation in their quality of life after surgery., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Postoperative anal dilations (PAD) are the standard of care for patients after a posterior sagittal anorectoplasty (PSARP) for anorectal malformation (ARM) or a transanal pull-through (TP) procedure for Hirschsprung disease (HD). This study assessed the psychosocial impact of PAD among caregivers of children with ARM or HD, which may inform postoperative care strategies., Methods: Caregivers of patients with ARM and HD who underwent PSARP or TP within five years participated in the online survey. Questions included demographics, patient and caregiver experiences with PAD, and baseline psychosocial functioning. Quantitative results were reported descriptively, while qualitative responses were summarized as major themes., Results: The survey indicated a response rate of 26% caregivers, with most being female (91%) and biological mothers (85%). Patients were mostly male (65%), born with ARM (74%), and were five months old on average when PAD began. Caregivers reported that during PAD, children experienced distress (56%), pain (44%), and fear (41%), while a third noted no negative reactions. Over time, their child's ability to cope with PAD got easier (38%) or stayed the same (41%). Caregivers reported worry/anxiety (88%), guilt (71%), stress (62%), and frustration (35%), noting that additional coping strategies to manage the emotional and logistical challenges of daily PAD would be helpful., Conclusion: Although PAD is necessary, it can be highly stressful for the patients and their caregivers. Key findings emphasized the need for additional coping strategies and highlighted the importance of integrating psychosocial support into the postoperative care regimen.

Background: There is growing evidence supporting multidisciplinary molecular tumor boards (MTB) in solid tumors whereas hematologic malignancies remain underrepresented in this regard., Objective: The present study aimed to assess the clinical relevance of MTBs in primary refractory diffuse large B-cell lymphomas/high-grade B-cell lymphomas with MYC and BCL2 rearrangements (prDLBCL/HGBL-MYC/BCL2) (n = 13) and HGBL, not otherwise specified (NOS), with MYC and BCL6 rearrangements (prHGBL, NOS-MYC/BCL6) (n = 6) based on our previously published whole-exome sequencing (WES) cohort., Patients and Methods: For genomic analysis, the institutional MTB WES pipeline (University Cancer Center Schleswig-Holstein: UCCSH), certified for routine clinical diagnostics, was employed and supplemented by a comprehensive immunohistochemical work-up. Consecutive database research and annotation according to established evidence levels for molecularly stratified therapies was performed (NCT-DKTK/ESCAT)., Results: Molecularly tailored treatment options with NCT-DKTK evidence level of at least m2A were identified in each case. We classified mutations in accordance with biomarker/treatment baskets and detected a heterogeneous spectrum of targetable alterations affecting immune evasion (IE; n = 30), B-cell targets (BCT; n = 26), DNA damage repair (DDR; n = 20), tyrosine kinases (TK; n = 13), cell cycle (CC; n = 7), PI3K-MTOR-AKT pathway (PAM; n = 2), RAF-MEK-ERK cascade (RME; n = 1), and others (OTH; n = 11)., Conclusion: Our virtual MTB approach identified potential molecularly targeted treatment options alongside targetable genomic signatures for both prDLBCL/HGBL-MYC/BCL2 and prHGBL, NOS-MYC/BCL6. These results underline the potential of MTB consultations in difficult-to-treat lymphomas early in the treatment sequence., (© 2023. The Author(s).)

Purpose: To assess the quality of life and disease-specific functioning of adults with anorectal malformations (ARM) or Hirschsprung disease (HD) compared to healthy reference scores., Methods: Patients with the diagnosis of ARM or HD from the Adult Colorectal Research Registry completed the Short Form 36 Health Survey (SF-36), the Gastrointestinal Quality of Life Index (GIQoLI), and the Bowel Function Score (BFS) between October 2019 and August 2022. One-sample Wilcoxon test compared the results to reported healthy references with a significance level of < 0.05., Results: The response rate was 67%. All three surveys were completed by 133 adults with a slight preponderance of males (51%). Median age was 31 years, 117 were born with ARM and 16 with HD. All subgroups had significantly lower BFS than healthy references. ARM patients scored significantly lower than the healthy reference population when assessed for GIQoL. All showed significant impairment with the mental component summary (MCS) of SF-36. Patients with a successful bowel management had significantly higher scores on all three questionnaires than those with fecal accidents., Conclusion: Our results emphasize the importance of a successful bowel management and its impact on the quality of life and bowel function. Long-term follow-up is recommended with attention to mental health., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: This study aimed to assess our bowel management program (BMP) and identify predictive factors for bowel control in patients with Spina Bifida (SB) and Spinal Cord Injuries (SCI). Additionally, in patients with SB, we examined the impact of fetal repair (FRG) on bowel control., Methods: We included all patients with SB and SCI seen in the Multidisciplinary Spinal Defects Clinic at Children's Hospital Colorado from 2020 to 2023., Results: 336 patients included. Fecal incontinence was present in 70% and bowel control in 30%. All patients with urinary control also had bowel control. Fecal incontinence prevalence was higher in patients with ventriculoperitoneal (VP) shunt (84%), urinary incontinence (82%), and wheelchair users (79%) compared to those who did not need a VP shunt (56%), had urinary continence (0%) and non-wheelchair users (52%), respectively (p =  < 0.001 in all three scenarios). After completing BMP, 90% remained clean for stool. There was no statistical significance when comparing bowel control in FRG with non-fetal repair group., Conclusions: Urinary continence predicts bowel control in patients with SB and SCI. Risk factors for fecal incontinence were the need for a VP shunt, urinary incontinence, and wheelchair usage. We did not find any positive impact of fetal repair on bowel and urinary control., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: This study aimed to analyze our radiologically supervised bowel management program (RS-BMP) outcomes in patients with chronic idiopathic constipation (CIC)., Methods: A retrospective study was conducted. We included all patients with CIC who participated in our RS-BMP at Children´s Hospital Colorado from July 2016 to October 2022., Results: Eighty patients were included. The average time with constipation was 5.6 years. Before our RS-BMP, 95% had received non-radiologically supervised treatments, and 71% had attempted two or more treatments. Overall, 90% had tried Polyethylene Glycol and 43% Senna. Nine patients had a history of Botox injections. Five underwent anterograde continence procedure, and one a sigmoidectomy. Behavioral disorders (BD) were found in 23%. At the end of the RS-BMP, 96% of patients had successful outcomes, 73% were on Senna, and 27% were on enemas. Megarectum was detected in 93% of patients with successful outcomes and 100% with unsuccessful outcomes (p = 0.210). Of the patients with BD, 89% had successful outcomes, and 11% had unsuccessful., Conclusion: Our RS-BMP has been proven to be effective in treating CIC. The radiologically supervised use of Senna and enemas was the appropriate treatment in 96% of the patients. BD and megarectum were associated with unsuccessful outcomes., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To assess fertility concerns and to describe pregnancy outcomes in patients with anorectal malformations (ARM)., Methods: This is an IRB approved, cross-sectional study of patients in the Adult Colorectal Research Registry who completed reproductive health surveys between November 2021 and August 2022. Patients assigned female at birth with age 18 or older and ARM were included., Results: Sixty-four patients with ARM, age 18 or older, were included. Fertility concerns were reported in 26 (40.6%) patients, 11 of which had seen a fertility specialist, including four who had not yet tried to conceive. Fertility concerns were highest amongst cloaca patients who had not yet tried to conceive (37.5%). 26 (40.6%) patients had tried to conceive, of which 16 (25%) reported fertility problems, most frequently uterine abnormalities and damaged or blocked fallopian tubes. 22 (34.4%) participants were able to conceive and 18 (28.1%) had at least one live birth. Patients with ARM who had concerns of fertility, had better FertiQoL when compared to published reference scores for patients experiencing fertility issues., Conclusion: Providers should be aware of fertility concerns in patients with ARM. Proactive counseling with referrals to a fertility specialist should be considered in patients who desire future fertility., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Resection margin adequacy plays a critical role in the local control of sarcomas. Fluorescence-guided surgery has increased complete resection rates and local recurrence-free survival in several oncological disciplines. The purpose of this study was to determine whether sarcomas exhibit sufficient tumor fluorescence (photodynamic diagnosis (PDD)) after administration of 5-aminolevulinic acid (5-ALA) and whether photodynamic therapy (PDT) has an impact on tumor vitality in vivo. Sixteen primary cell cultures were derived from patient samples of 12 different sarcoma subtypes and transplanted onto the chorio-allantoic membrane (CAM) of chick embryos to generate 3-dimensional cell-derived xenografts (CDXs). After treatment with 5-ALA, the CDXs were incubated for another 4 h. Subsequently accumulated protoporphyrin IX (PPIX) was excited by blue light and the intensity of tumor fluorescence was analyzed. A subset of CDXs was exposed to red light and morphological changes of both CAMs and tumors were documented. Twenty-four hours after PDT, the tumors were excised and examined histologically. High rates of cell-derived engraftments on the CAM were achieved in all sarcoma subtypes and an intense PPIX fluorescence was observed. PDT of CDXs resulted in a disruption of tumor-feeding vessels and 52.4% of CDXs presented as regressive after PDT treatment, whereas control CDXs remained vital in all cases. Therefore, 5-ALA mediated PDD and PDT appear to be promising tools in defining sarcoma resection margins (PDD) and adjuvant treatment of the tumor bed (PDT).

Purpose: Most patients with idiopathic constipation achieve daily voluntary bowel movements with stimulant laxatives after a "Structured Bowel Management Program" (BMP). A small percentage require rectal enemas. One week in a BMP to find the right enema recipe results in a success rate great than 95%. Once the enema is radiologically and clinically effective, antegrade continent enema procedures (ACE) can afford patients an alternative route of enema administration. This study summarized the outcomes of children with idiopathic constipation who receive antegrade enemas (AE) with or without a prior BMP., Methods: This was a single institution, retrospective cohort study of children with idiopathic constipation who underwent ACE procedures indicated by different providers from 2015-2020. We categorized the outcomes with AE after the ACE procedure as: "successful outcome" when the AE produced a daily bowel movement, no involuntary bowel movements, and no more fecal impactions, "unsuccessful outcome" was defined when the patient continued having involuntary bowel movements or fecal impaction requiring cleanouts despite a daily AE, and "unnecessary outcome" was defined when the patient was no longer doing AE, but had daily bowel movements, and no involuntary bowel movements or fecal impactions., Results: Thirty-eight children with idiopathic constipation had an ACE. The most frequent indication for ACE was a failure of medical treatment. The most common medical treatment was polyethylene glycol. Before ACE, 34 (89%) patients did not have a BMP; 18 patients were on rectal enemas and 16 on laxatives. All four with BMP (100%) had a successful rectal enema. After ACE, 12 (31%) patients had successful antegrade enemas, including the four with previous successful BMP with rectal enemas. Twenty patients (52%) had unsuccessful antegrade enemas, and in 6 (15%), the ACE was unnecessary (Fig. 1)., Conclusion: Using antegrade enemas without a previously successful formula for rectal enemas has resulted in a high rate of unsuccessful and unnecessary procedures. BMP for children with idiopathic constipation who needs rectal enemas offers a high possibility to find the proper rectal enema recipe and ensures higher rates of successful AE., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Long-term urinary outcomes for patients born with Hirschsprung disease (HD) and anorectal malformations (ARM) may impact their health and wellbeing into adulthood. This study describes self-reported long-term urinary outcomes in males with HD and ARM., Methods: This was a prospective study of male patients in the Adult Colorectal Research Registry who completed surveys on urinary function between October 2019 and March 2022. Self-reported health and functional outcomes were summarized, and differences based on type of condition were compared., Results: Sixty-seven patients completed the questionnaire (response rate: 59.1%), of which 17.9% (12) had HD and 82.1% (55) had an ARM. Rates of urinary incontinence and stress urinary incontinence were 16.4% (11) and 4.5% (3), respectively. On sub-analysis of patients with ARM, patients with sacral ratio (SR) of 0.4-0.69 reported higher UTI rates compared to those with SR ≥ 0.7 (57.9 vs 25.8%, p = 0.023). Renal failure rates were highest among patients with recto-bladder neck fistulas (66.0%, p = 0.012)., Conclusion: Patients with HD and ARM report a variety of urological sequelae in adulthood. Outcomes appear to be more common in patients with ARM and may be impacted by both anatomy and sacral ratios. Transitional care to monitor and manage renal and urological function is imperative., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: The long-term impact of anorectal malformations (ARM) and Hirschsprung disease (HD) on sexual function is well recognized but understudied. This study evaluated self-reported sexual and fertility outcomes in adult males with ARM and HD., Methods: This was an IRB approved, prospective study of males in the Adult Colorectal Research Registry who completed surveys between October 2019 and March 2022. Electronic surveys were administered after consenting to being contacted for research. Patients completed the International Index of Erectile Function (IIEF) questionnaire and provided information on fertility outcomes., Results: Sixty-five patients completed outcome questionnaires: 11 (16.9%) had HD and 54 (83.1%) had an ARM. Nineteen patients reported some degree of erectile dysfunction per IIEF criteria, a greater proportion of whom have ARM (p = 0.046). Twenty (30.7%) have reported having children; there were no differences in rates between HD and ARM patients. Most patients had not attempted to conceive, but eight patients, all of whom have ARM, have pursued fertility investigation or treatments., Conclusion: More patients with ARM reported some degree of erectile dysfunction compared to those with HD. Additionally, some have required fertility treatments. Further investigation is warranted to ensure true low rates of sexual and fertility dysfunction in patients with HD., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Pediatric colorectal studies have shown enhanced recovery after surgery (ERAS) protocols can safely improve outcomes. This study sought to determine the impact of an ERAS pathway on the outcomes of children with colorectal conditions who underwent colostomy closure or Malone procedure., Methods: A single-institution, retrospective cohort study of children who underwent colostomy closure or Malone procedure between 2016 and 2020 was performed. Differences in outcomes between pre-ERAS and ERAS cohorts were tested. A sub-analysis based on procedure type was performed., Results: There were 96 patients included: 22 prior to ERAS implementation and 74 after. Patients who underwent ERAS had shorter mean time (hours) to oral intake, mean days until regular diet, post-operative opioid volume, and median length of stay (p < 0.05). There was no difference in complication rates in the ERAS and pre-ERAS cohort (12.2 vs 9.1%, p = 0.99). Patients who underwent colostomy closure after ERAS had lower post-operative opioid use, but no differences were seen in those who underwent Malone., Conclusion: Implementation of an ERAS protocol resulted in quicker time to oral intake, normal diet, and decreased opioid use without increasing complication rates. Differences seen based on procedure type may reflect that the effect of an ERAS protocol is procedure specific., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Congenital or acquired anatomic differences of the genital tract in patients with anorectal malformations (ARM) may make pelvic exams more challenging. The purpose of this study was to describe office-based pelvic exams and cervical cancer screening among female patients with a history of ARM., Methods: This was an IRB approved, cross-sectional study of female patients with ARM who completed our Reproductive Health Surveys from November 2021 to March 2022., Results: Fifty-four patients with ARM were included in the study. Ages ranged from 22 to 80 years (mean age 34.5 years). Thirty-four patients had a cloaca, 16 had an ARM other than cloaca, and four patients had a complex malformation. Most patients (79.6%) reported having had a pelvic exam in the office. On a scale of 0-100, the mean pain score with pelvic exam was 42. Pain scores were higher for patients with complex malformations and neo-vagina. Forty-three participants (79.6%) had cervical cancer screening. Fifteen participants (28%) had a didelphys uterus but only two patients specified that both cervices were screened for cervical cancer., Conclusion: The majority of female patients with ARM had cervical cancer screening in clinic, those with complex malformations reported greater discomfort. Providers should be aware of barriers to performing pelvic exams in patients with ARM as well as the need to screen both cervices in didelphys uterus., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: This study aimed to obtain information about the psychosocial functioning of adults with various congenital colorectal conditions (e.g., anorectal malformation, Hirschsprung disease)., Methods: A research registry of adult patients with colorectal conditions was developed. Items included demographics, medical diagnosis/treatment, and measures of anxiety and depression. Descriptive and inferential statistical approaches were applied to summarize data and determine significant differences in the average scores for depression and anxiety between various groupings of diagnoses, gender, race, and the use of psychotropic medication., Results: Study measures were completed by 131 adults. Depression and anxiety scores were significantly higher for women than men and those self-identified as non-binary (p = 0.012, < 0.001, respectively). No significant differences in depression and anxiety scores were found due to colorectal diagnosis (p > 0.05). Participants who identified as Asian had significantly higher depression scores than participants of other races (p = 0.002); but no significant difference was noted for anxiety scores (p = 0.065)., Conclusions: Results suggest that depression and anxiety scores were significantly influenced by gender and race. However, colorectal diagnosis was not a predictor of depression or anxiety. It is important for colorectal providers to be aware of the psychosocial implications of congenital colorectal conditions and consider how to provide adequate support to address patients' psychosocial needs., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: The aim of this study was to determine and analyze the stricture rate in patients who underwent a PSARP or PSARVUP and followed a postoperative protocol of anal dilation (Fig. 1)., Methods: A retrospective review of patients with anorectal malformation (ARM) who underwent a primary PSARP or PSARVUP from February 2016 to October 2021 was performed. Data collected included patients' demographics, type of ARM, age at the time of operation, postoperative complications, with emphasis on whether there were any strictures or any difficulties during dilations, and on follow-up. During the surgical repair, emphasis was placed on preserving the blood supply of the bowel and performing a tension-free bowel-to-skin anastomosis., Results: Eighty-four patients met the inclusion criteria. Forty-four patients were females: 21 recto-perineal fistula, 12 cloaca, 9 recto-vestibular fistula, one imperforate anus without fistula, and one patient had a complex anorectal and vaginal malformation with an anal stricture and a rectovaginal fistula. Forty patients were males: 14 recto-perineal fistula, 11 recto-urethral bulbar fistula, 6 recto-urethral prostatic fistula, 6 imperforate anus without fistula, and 2 bladder neck fistula. One patient had an anal stenosis with sacral agenesis, without a presacral mass. Patient ages ranged from 0 to 79 months (mean 7.5 months, median 5 months) at the time of surgery. Follow-up time ranged from 7 to 73 months (mean 38 months, median 35 months). No patient suffered of a postoperative anal stricture. Six patients suffered of a rectal prolapse that required a surgical repair., Conclusion: Post-operative anal stricture after PSARP and PSARVUP can be avoided with proper surgical technique and postoperative care. Namely, by preserving adequate blood supply of the bowel and avoiding tension at the anoplasty, and by adhering to a structured protocol of anal dilations., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Circulating tumor DNA (ctDNA) from circulating free DNA (cfDNA) in GIST is of interest for the detection of heterogeneous resistance mutations and treatment monitoring. However, methodologies for use in a local setting are not standardized and are error-prone and difficult to interpret. We established a workflow to evaluate routine tumor tissue NGS (Illumina-based next generation sequencing) panels and pipelines for ctDNA sequencing in an academic setting. Regular blood collection (Sarstedt) EDTA tubes were sufficient for direct processing whereas specialized tubes (STRECK) were better for transportation. Mutation detection rate was higher in automatically extracted (AE) than manually extracted (ME) samples. Sensitivity and specificity for specific mutation detection was higher using digital droplet (dd)PCR compared to NGS. In a retrospective analysis of NGS and clinical data (133 samples from 38 patients), cfDNA concentration correlated with tumor load and mutation detection. A clinical routine pipeline and a novel research pipeline yielded different results, but known and resistance-mediating mutations were detected by both and correlated with the resistance spectrum of TKIs used. In conclusion, NGS routine panel analysis was not sensitive and specific enough to replace solid biopsies in GIST. However, more precise methods (hybridization capture NGS, ddPCR) may comprise important research tools to investigate resistance. Future clinical trials need to compare methodology and protocols.

Background: Previous studies have examined characteristics critical to make a successful applicant in the pediatric surgery match. No previous study has focused on what applicants consider important as they interview pediatric surgery training programs., Study Design: Successfully matched applicants to our training program from 2018 to 2020 were identified and a 49-question survey sent electronically. In addition to a descriptive analysis of responses, comparisons between those who matched into one of their top three programs and those who matched outside of their top three programs were made. A Likert scale was used and data are reported as mean (95% confidence intervals)., Results: One hundred and thirty individuals were surveyed. Sixty-three (48%) responded. The most important characteristics identified were total index case volume 1.94 (1.87, 2.00), interactions with faculty on the interview day 1.84 (1.74, 1.94), neonatal case volume 1.75 (1.61, 1.88) and degree of autonomy 1.63 (1.50, 1.77). Respondents also placed a high importance on the in-person interview experience 1.63 (1.49, 1.77). The least important training program characteristics were quantity of vacation time -0.24 (-0.52, 0.05), opportunity to participate in medical mission trips -0.25 (-0.54, 0.06), paid accommodation at the interview -0.29 (-0.57, 0.00), and access to a simulation laboratory -0.51 (-0.78, -0.24)., Conclusion: Clinical volume, neonatal volume, faulty interactions and the experience on the interview day are the most important characteristics to applicants. There were few, if any, characteristics applicants felt were truly unimportant as they weighed their rank lists. Applicants also placed a high level of importance on an in-person interview LEVEL OF EVIDENCE: Level IV., (Copyright © 2022. Published by Elsevier Inc.)

High-grade B-cell lymphoma accompanied with double/triple-hit MYC and BCL2 and/or BCL6 rearrangements (HGBLDH/ TH) poses a cytogenetically-defined provisional entity among aggressive B-cell lymphomas that is traditionally associated with unfavorable prognosis. In order to better understand the mutational and molecular landscape of HGBLDH/ TH we here performed whole-exome sequencing and deep panel next-generation sequencing of 47 clinically annotated cases. Oncogenic drivers, mutational signatures and perturbed pathways were compared with data from follicular lymphoma (FL), diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma (BL). We find an accumulation of oncogenic mutations in NOTCH, IL6/JAK/STAT and NFκB signaling pathways and delineate the mutational relationship within the continuum between FL/DLBCL, HGBL-DH/TH and BL. Further, we provide evidence of a molecular divergence between BCL2 and BCL6 rearranged HGBL-DH. Beyond a significant congruency with the C3/EZB DLBCL cluster in BCL2 rearranged cases on an exome-wide level, we observe an enrichment of the SBS6 mutation signature in BCL6 rearranged cases. Differential gene set enrichment and subsequent network propagation analysis according to cytogenetically defined subgroups revealed an impairment of TP53 and MYC pathway signaling in BCL2 rearranged cases, whereas BCL6 rearranged cases lacked this enrichment, but instead showed impairment of E2F targets. Intriguingly, HGBL-TH displayed intermediate mutational features considering all three aspects. This study elucidates a recurrent pattern of mutational events driving FL into MYC-driven BCL2-rearranged HGBL, unveiling the mutational pathogenesis of this provisional entity. Through this refinement of the molecular taxonomy for aggressive, germinal center-derived B-cell lymphomas, this calls into question the current World Health Organization classification system, especially regarding the status of MYC/BCL6- rearranged HGBL.