RESUMO
OBJECTIVES: The purpose of this study was to identify the preoperative CT features that are associated with inadvertent enterotomy (IE) during adhesive small bowel obstruction (ASBO) surgery. METHODS: From January 2015 to December 2019, all patients with ASBO who underwent an abdominal CT were reviewed. Abdominal CT were retrospectively reviewed by two radiologists with a consensus read in case of disagreement. IE during ASBO surgery was retrospectively recorded. Univariate and multivariate analyses of CT features associated with IE were performed and a simple CT score was built to stratify the risk of IE. This score was validated in an independent retrospective cohort. Abdominal CT of the validation cohort was reviewed by a third independent reader. RESULTS: Among the 368 patients with ASBO during the study period, 169 were surgically treated, including 129 ASBO for single adhesive band and 40 for matted adhesions. Among these, there were 47 IE. By multivariate analysis, angulation of the transitional zone (OR = 4.19, 95% CI [1.10-18.09]), diffuse intestinal adhesions (OR = 4.87, 95% CI [1.37-19.76]), a fat notch sign (OR = 0.32, 95% CI [0.12-0.85]), and mesenteric haziness (OR = 0.13, 95% CI [0.03-0.48]) were independently associated with inadvertent enterotomy occurrence. The simple CT score built to stratify risk of IE displayed an AUC of 0.85 (95% CI [0.80-0.90]) in the study sample and 0.88 (95% CI [0.80-0.96]) in the validation cohort. CONCLUSION: A simple preoperative CT score is able to inform the surgeon about a high risk of IE and therefore influence the surgical procedure. KEY POINTS: ⢠In this retrospective study of 169 patients undergoing abdominal surgery for adhesive small bowel obstruction, 47 (28%) inadvertent enterotomy occurred. ⢠A simple preoperative CT score enables accurate stratification of inadvertent enterotomy risk (area under the curve 0.85). ⢠By multivariable analysis, diffuse intestinal adhesions and angulation of the transitional zone were predictive of inadvertent enterotomy occurrence.
Assuntos
Adesivos , Obstrução Intestinal , Humanos , Obstrução Intestinal/diagnóstico por imagem , Obstrução Intestinal/etiologia , Obstrução Intestinal/cirurgia , Intestino Delgado/diagnóstico por imagem , Intestino Delgado/cirurgia , Estudos Retrospectivos , Tomografia Computadorizada por Raios X/métodosRESUMO
INTRODUCTION: To identify factors associated with irreversible transmural necrosis (ITN) among critically ill patients experiencing nonocclusive mesenteric ischemia (NOMI) and to compare the predictive value regarding ITN risk stratification with that of the previously described Clichy score. METHODS: All consecutive patients admitted to the intensive care unit between 2009 and 2019 who underwent exploratory laparotomy for NOMI and who had an available contrast-enhanced computed tomography with at least 1 portal venous phase were evaluated for inclusion. Clinical, laboratory, and radiological variables were collected. ITN was assessed on pathological reports of surgical specimens and/or on laparotomy findings in cases of open-close surgery. Factors associated with ITN were identified by univariate and multivariate analysis to derive a NOMI-ITN score. This score was further compared with the Clichy score. RESULTS: We identified 4 factors associated with ITN in the context of NOMI: absence of bowel enhancement, bowel thinning, plasma bicarbonate concentration ≤15 mmol/L, and prothrombin rate <40%. These factors were included in a new NOMI-ITN score, with 1 point attributed for each variable. ITN was observed in 6%, 38%, 65%, 88%, and 100% of patients with NOMI-ITN score ranging from 0 to 4, respectively. The NOMI-ITN score outperformed the Clichy score for the prediction of ITN (area under the receiver operating characteristics curve 0.882 [95% confidence interval 0.826-0.938] vs 0.674 [95% confidence interval 0.582-0.766], respectively, P < 0.001). DISCUSSION: We propose a new 4-point score aimed at stratifying risk of ITN in patients with NOMI. The Clichy score should be applied to patients with occlusive acute mesenteric ischemia only.
Assuntos
Intestino Delgado/patologia , Isquemia Mesentérica/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Bicarbonatos/sangue , Estado Terminal , Feminino , Humanos , Intestino Delgado/irrigação sanguínea , Intestino Delgado/diagnóstico por imagem , Laparotomia , Masculino , Isquemia Mesentérica/sangue , Isquemia Mesentérica/complicações , Isquemia Mesentérica/diagnóstico por imagem , Pessoa de Meia-Idade , Análise Multivariada , Necrose , Complicações Pós-Operatórias/diagnóstico por imagem , Complicações Pós-Operatórias/patologia , Modelos de Riscos Proporcionais , Tempo de Protrombina , Medição de Risco , Sepse/complicações , Tomografia Computadorizada por Raios XRESUMO
OBJECTIVES: To evaluate the diagnostic performance of CT for transmural necrosis (TN) in non-occlusive mesenteric ischemia (NOMI) according to the bowel segment involved. METHODS: From January 2009 to December 2019, all patients admitted to the intensive care unit (ICU) and requiring laparotomy for NOMI were retrospectively studied. CT had to have been performed within 24 h prior to laparotomy and were reviewed by two abdominal radiologists, with a consensus reading in case of disagreement. A set of CT features of mesenteric ischemia were assessed, separating the stomach, jejunum, ileum, and right (RC) and left colon (LC). Univariate and multivariate analyses were performed to identify features associated with TN. Its influence on overall survival (OS) was assessed. RESULTS: Among 145 patients, 95 (66%) had ≥ 1 bowel segment with TN, including 7 (5%), 31 (21%), 43 (29%), 45 (31%), and 52 (35%) in the stomach, jejunum, ileum, RC, and LC, respectively. Overall inter-reader agreement of CT features was significantly lower in the colon than in the small bowel (0.59 [0.52-0.65] vs 0.74 [0.70-0.77] respectively). The absence of bowel wall enhancement was the only CT feature associated with TN by multivariate analysis, whatever the bowel segment involved. Proximal TN was associated with poorer OS (p < 0.001). CONCLUSIONS: The absence of bowel wall enhancement remains the most consistent CT feature of transmural necrosis, whatever the bowel segment involved in NOMI. Inter-reader agreement of CT features is lower in the colon than in the small bowel. Proximal TN seems to be associated with poorer OS. KEY POINTS: ⢠The absence of bowel wall enhancement is the most consistent CT feature associated with transmural necrosis in NOMI, whatever is the bowel segment involved. ⢠Inter-reader agreement is lower in the colon than in the small bowel in NOMI. ⢠In NOMI, the more proximal the bowel necrosis, the worse the prognosis.
Assuntos
Enteropatias , Isquemia Mesentérica , Humanos , Isquemia/diagnóstico por imagem , Isquemia Mesentérica/diagnóstico por imagem , Necrose , Estudos Retrospectivos , Tomografia Computadorizada por Raios XRESUMO
BACKGROUND: Long-term outcomes of accessory renal artery (ARA) coverage after endovascular aneurysm repair (EVAR) are unknown. We analyzed the impact of ARA coverage on renal function long-term. METHODS: This retrospective, monocentric study included patients treated by EVAR between 2008 and 2016. Patients with at least one ARA covered with EVAR (ARA group) were compared with patients with no covered ARA (control group). Renal function was determined by estimating the glomerular filtration rate (eGFR) and graded according to chronic kidney disease (CKD) classification stages. RESULTS: A total of 184 patients were included (ARA group, n = 25; control group, n = 159). Renal risk factors were similar in the 2 groups. Mean (±standard deviation) duration of follow-up was 41.6 ± 25.8 months. Preoperative eGFR (mL/min/1.73 m2) was 68.9 ± 17.8 in the ARA group and 72.5 ± 17.4 in the control group (P = 0.33), with a similar decline in the 2 groups during follow-up (-6.52 ± 11.6 ARA group vs. -6.43 ± 13.8 control group; P = 0.97). At the end of the study, 8 ARA patients and 56 controls had deteriorated by one CKD stage (32% vs. 35.2%, respectively; P = 0.75). Rate of renal infarction was significantly higher in the ARA group (96% vs. 1.9%; P < 0.0001). In multivariate analysis, suprarenal fixation was identified as a risk factor for a decline in renal function (odds ratio = 2.01 [95% confidence interval: 1.05-3.84]; P = 0.04). CONCLUSIONS: ARA coverage after EVAR does not appear to affect renal function long-term. Suprarenal fixation led to a greater decline in renal function.
Assuntos
Aneurisma Aórtico/cirurgia , Implante de Prótese Vascular , Procedimentos Endovasculares , Rim/fisiopatologia , Artéria Renal/cirurgia , Insuficiência Renal Crônica/fisiopatologia , Idoso , Idoso de 80 Anos ou mais , Aneurisma Aórtico/diagnóstico por imagem , Prótese Vascular , Implante de Prótese Vascular/efeitos adversos , Implante de Prótese Vascular/instrumentação , Progressão da Doença , Procedimentos Endovasculares/efeitos adversos , Procedimentos Endovasculares/instrumentação , Feminino , Taxa de Filtração Glomerular , Humanos , Masculino , Artéria Renal/anormalidades , Artéria Renal/diagnóstico por imagem , Insuficiência Renal Crônica/diagnóstico , Estudos Retrospectivos , Fatores de Tempo , Resultado do TratamentoRESUMO
OBJECTIVES: To establish national reference levels (RLs) in interventional procedures under CT guidance as required by the 2013/59/Euratom European Directive. METHODS: Seventeen categories of interventional procedures in thoracic, abdominopelvic, and osteoarticular specialties (percutaneous infiltration, vertebroplasty, biopsy, drainage, tumor destruction) were analyzed. Total dose length product (DLP), number of helical acquisitions (NH), and total DLP for helical, sequential, or fluoroscopic acquisitions were recorded for 10 to 20 patients per procedure at each center. RLs were calculated as the 3rd quartiles of the distributions and target values for optimization process (TVOs) as the median. RLs and TVOs were compared with previously published studies. RESULTS: Results on 5001 procedures from 49 centers confirmed the great variability in patient dose for the same category of procedures. RLs were proposed for the DLPs and NHs in the seventeen categories. RLs in terms of DLP and NH were 375 mGy.cm and 2 NH for spinal or peri-spinal infiltration, 1630 mGy.cm and 3 NH for vertebroplasty, 845 mGy.cm and 4 NH for biopsy, 1950 mGy.cm and 8 NH for destruction of tumors, and 1090 mGy.cm and 5 NH for drainage. DLP and NH increased with the complexity of procedures. CONCLUSIONS: This study was the first nationwide multicentric survey to propose RLs for interventional procedures under CT guidance. Heterogeneity of practice in centers were found with different levels of patient doses for the same procedure. The proposed RLs will allow imaging departments to benchmark their practice with others and optimize their protocols. KEY POINTS: ⢠National reference levels are proposed for 17 categories of interventional procedures under CT guidance. ⢠Reference levels are useful for benchmarking practices and optimizing protocols. ⢠Reference levels are proposed for dose length product and the number of helical acquisitions.
Assuntos
Doses de Radiação , Radiografia Intervencionista/normas , Valores de Referência , Tomografia Computadorizada por Raios X/normas , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Biópsia , Feminino , Fluoroscopia/métodos , França , Humanos , Masculino , Pessoa de Meia-Idade , Radiografia Intervencionista/métodos , Reprodutibilidade dos Testes , Estudos Retrospectivos , Coluna Vertebral , Inquéritos e Questionários , Tomografia Computadorizada por Raios X/métodos , Vertebroplastia , Adulto JovemRESUMO
The original version of this article, published on 02 May 2020, unfortunately contained a mistake.
RESUMO
OBJECTIVE. The purpose of this study was to assess whether transmural bowel necrosis has distinct CT features based on the three main causes: occlusive acute mesenteric ischemia (AMI), nonocclusive AMI, and strangulated small-bowel obstruction (SBO). MATERIALS AND METHODS. From January 2010 to December 2017, the records of all patients with a pathologic diagnosis of transmural bowel necrosis were extracted from the pathology department database of a university hospital. The inclusion criteria for the study were presence of transmural bowel necrosis at pathologic examination and available contrast-enhanced CT images obtained within the 24 hours before surgery. Seventy-seven patients were finally included. The CT scans were retrospectively independently reviewed by two abdominal radiologists to identify the classic CT findings of transmural bowel necrosis. Statistical analyses were performed. RESULTS. Pneumatosis intestinalis was statistically more frequent in nonocclusive AMI (59%) than in occlusive AMI (29%) and strangulated SBO (7%) (p < 0.01), as were superior mesenteric venous gas (55%, 29%, and 0%; p < 0.01) and portal venous gas (48%, 10%, and 0%; p < 0.01). Decreased or absent bowel wall enhancement was more frequent in AMI than in SBO (nonocclusive AMI, 83%; occlusive AMI, 81%; SBO, 56%; p = 0.02), as was thinned bowel wall (nonocclusive AMI, 52%; occlusive AMI, 48%; SBO, 18%; p = 0.02). Spontaneous hyperattenuation of the bowel wall was more frequent in strangulated SBO (41%) than in nonocclusive AMI (10%) and occlusive AMI (14%) (p < 0.01). CONCLUSION. Transmural bowel necrosis has distinct CT findings according to its three main causes. Occlusive AMI is characterized by an absence of bowel wall enhancement and less mesenteric fat stranding, nonocclusive AMI by a high prevalence of pneumatosis intestinalis and portal venous gas, and strangulated SBO by spontaneous hyperattenuation of the bowel wall and an absence of pneumatosis intestinalis and portal venous gas.
Assuntos
Obstrução Intestinal/complicações , Obstrução Intestinal/diagnóstico por imagem , Intestino Delgado/diagnóstico por imagem , Intestino Delgado/patologia , Isquemia Mesentérica/complicações , Isquemia Mesentérica/diagnóstico por imagem , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Necrose/etiologia , Estudos Retrospectivos , Tomografia Computadorizada por Raios XAssuntos
Azetidinas/uso terapêutico , Histiocitose/tratamento farmacológico , Pericardite/tratamento farmacológico , Piperidinas/uso terapêutico , Inibidores de Proteínas Quinases/uso terapêutico , Trombose/tratamento farmacológico , Histiocitose/complicações , Humanos , Pericardite/complicações , Trombose/complicaçõesRESUMO
Our objective was to investigate the indications, effectiveness, and safety of Amplatzer™ Vascular Plugs (AVPs) in clinical practice. To retrospectively identify patients managed with AVPs at the Dijon University Hospital between January 2011 and April 2021, we searched materials vigilance registries and procedure reports. The 110 identified patients underwent 111 procedures with delivery of 202 AVPs into 118 vessels; 84% of the procedures were performed by radiologists with over 10 years' experience and 67% were scheduled. Varicocele, haemostasis, pelvic varicose veins, and arterio-venous dialysis fistulas accounted for 69% of procedures. The technical and clinical success rates were 99% and 97%, respectively. The single major complication was AVP migration in a high-flow internal iliac vein, with no residual abnormalities after successful device retrieval. Several AVPs and/or concomitant injection of coils or liquid agents were used in 80% of cases. The use of AVPs alone occurred chiefly for splenic artery embolisation in trauma patients and for collateral vein occlusion in dysfunctional arterio-venous dialysis fistulas. No cases of recanalisation occurred during the 19 ± 29 month follow-ups. Based on their good safety and effectiveness profile, AVPs deserve to be part of the therapeutic armamentarium of every interventional radiologist.
RESUMO
The Micro Vascular Plug® (MVP, Medtronic) is a mechanical embolic agent available in small sizes that allows for distal embolisation. The objective of this retrospective observational single-centre study was to assess MVP embolisation procedures performed at a university hospital. The 33 patients who underwent MVP embolisation in 2021 were included (mean age, 64; 24 males and 9 females). The primary endpoint was technical success, which was defined as a full first-attempt occlusion with one or more MVPs, as documented on the end-of-procedure angiogram. In all patients, 51 MVPs were used overall, with other embolic agents in 23 of the 33 cases (usually coils and/or glue); 22 of the 33 procedures were emergent for bleeding and 11 were planned for other indications. Of the three technical failures, two were due to an angled target artery configuration precluding microcatheterisation and one to failure of the device to release from its wire. The technical success rate was thus 90.9%. No patient experienced MVP migration or other major complications. Five patients had recurrent clinical symptoms; in four cases, the cause was collateral development, and in one case, the cause was incomplete initial embolisation. No instances of recanalisation were documented during the short follow-up of 12 months, for a 100% secondary clinical success rate. At our tertiary-level centre, the MVP was both effective and safe for peripheral applications. Interventional radiologists should be conversant with the techniques and indications of MVP embolisation.
RESUMO
BACKGROUND: Radiology has always been an attractive specialty for residents, but its attractiveness has recently decreased in France regarding the median choice rank after at the National Residency Board. AIM: To study Radiologists' perceptions and social representations (SRs) among a group of medical students, residents and graduated physicians in France, to better understand the view of Radiologists to debunk stereotypes. METHODS: The nationwide web-based survey was based on valid hierarchical evocation methods. We determined the corpus's central core and SRs' principal themes with prototypical and correspondence factor analysis (CFA), respectively. RESULTS: Overall, 419 answers were analyzed. Radiologists' SRs were divided into 3 classes: negative stereotypes of Radiologists, negative stereotypes of the Radiologists' daily practice and Radiologists' skills. After multivariate analysis, variables that seemed to have a positive influence on Radiologists' SRs were considering radiology as a potential choice of specialty (p < 0.001) and the existence of practical experience in Radiology (p = 0.008). Women seemed to have a more negative SR of Radiologists than men (p = 0.035). DISCUSSION: This was the largest qualitative study on the subject and the only one among medical students, residents and graduated physicians, allowing a global picture. SRs of Radiologists seemed to be negative, potentially caused by poor knowledge of the Radiologists' profession. CONCLUSION: SRs of Radiologists among medical students and graduated physicians appears to be negative. Promoting the specialty among medical students and encouraging their immersion in a Radiology department could help to debunk many stereotypes about the daily life and missions of Radiologists.
Assuntos
Médicos , Radiologia , Estudantes de Medicina , Masculino , Humanos , Feminino , Radiologistas , Radiologia/educação , RadiografiaRESUMO
PURPOSE: The purpose of this study was to evaluate whether concomitant left gastric vein embolization (LGVE) during transjugular intrahepatic portosystemic shunt (TIPS) for acute variceal hemorrhage could reduce the risk of bleeding recurrence. MATERIAL AND METHOD: A national multicenter observational study was conducted in 14 centers between January 2019 and December 2020. All cirrhotic patients who underwent TIPS placement for acute variceal bleeding were included. During TIPS procedure, size of left gastric vein (LGV), performance of LGVE, material used for LGVE and portosystemic pressure gradient (PPG) before and after TIPS placement were collected. A propensity score for the occurrence of LGVE was calculated to assess effect of LGVE on rebleeding recurrence at six weeks and one year. RESULTS: A total of 356 patients were included (mean age 57.3 ± 10.8 [standard deviation] years; 283/356 [79%] men). Median follow-up was 11.2 months [interquartile range: 1.2, 13.3]. The main indication for TIPS was pre-emptive TIPS (162/356; 46%), rebleeding despite secondary prophylaxis (105/356; 29%), and salvage TIPS (89/356; 25%). Overall, 128/356 (36%) patients underwent LGVE during TIPS procedure. At six weeks and one year, rebleeding-free survival did not differ significantly between patients who underwent LGVE and those who did not (6/128 [5%] vs. 15/228 [7%] at six weeks, and 11/128 [5%] vs. 22/228 [7%] at one year, P = 0.622 and P = 0.889 respectively). A total of 55 pairs of patients were retained after propensity score matching. In patients without LGVE, the rebleeding rate was not different from those with LGVE (3/55 [5%] vs. 4/55 [7%], P > 0.99, and 5/55 [9%] vs. 6/55[11%], P > 0.99, at six weeks and one year respectively). Multivariable analysis identified PPG after TIPS placement as the only predictor of bleeding recurrence (hazard ratio = 1.09; 95% confidence interval: 1.02-1.18; P = 0.012). CONCLUSION: In this multicenter national real-life study, we did not observe any benefit of concomitant LGVE during TIPS placement for acute variceal bleeding on bleeding recurrence rate.
Assuntos
Varizes Esofágicas e Gástricas , Derivação Portossistêmica Transjugular Intra-Hepática , Masculino , Humanos , Pessoa de Meia-Idade , Idoso , Feminino , Varizes Esofágicas e Gástricas/complicações , Varizes Esofágicas e Gástricas/cirurgia , Derivação Portossistêmica Transjugular Intra-Hepática/métodos , Hemorragia Gastrointestinal/etiologia , Hemorragia Gastrointestinal/cirurgia , Recidiva , Veia PortaRESUMO
PURPOSE: The purpose of this study was to identify computed tomography (CT) features associated with early recurrence of sigmoid volvulus (SV) after a first uncomplicated episode and to develop a score for early SV recurrence risk stratification. MATERIALS AND METHODS: A total of 95 patients (59 men, 36 women; mean age, 72 ± 15 [SD] years; age range: 57-87 years) who underwent abdominal CT examination for a first uncomplicated SV episode from January 1st 2006 to July 31st 2020 in two French University Hospitals were retrospectively included. A SV recurrence occurring within six months was defined as early SV recurrence. CT findings associated with SV were searched for using univariable analysis. CT features associated with early recurrence were computed into a multivariable logistic regression model that was further used to build a score to stratify SV recurrence risk. Kaplan-Meier curves were built to evaluate recurrence-free survival. RESULTS: Early SV recurrence occurred in 53 patients (56%). At multivariable analysis, left lateral section volume < 150 cm3 and maximal colon distension > 10 cm were associated with early SV recurrence (Odds ratio [OR] = 4.62; 95% CI: 1.77-13.33; P = 0.002 and OR = 4.43 95% CI: 1.63-13.63; P = 0.005) respectively), and an early SV recurrence score with 1 point attributed to each of these two variables was built. Early SV recurrence was observed in 26%, 54% and 89% of patients with score of 0, 1 and 2, respectively (P < 0.001). CONCLUSION: A simple CT score allows stratification of early SV recurrence after a first episode and helps to select patient who would not benefit from prophylactic colonic surgery because of a low SV recurrence risk.
Assuntos
Volvo Intestinal , Doenças do Colo Sigmoide , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Volvo Intestinal/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Recidiva , Estudos Retrospectivos , Doenças do Colo Sigmoide/diagnóstico por imagem , Tomografia Computadorizada por Raios XRESUMO
Percutaneous sclerotherapy is used to treat venous and lymphatic vascular malformations, which can cause significant discomfort and/or disfigurement. The purpose of this study is to describe the bleomycin sclerotherapy technique and to evaluate its clinical and radiological efficacy and safety. We retrospectively identified consecutive patients with venous malformations (VMs) and lymphatic malformations (LMs) who underwent bleomycin sclerotherapy in 2011-2020 at our institution. We collected the clinical and radiological success rates, complications and recurrences separately in the VM and LM groups. We identified 26 patients, 15 with VMs and 11 with LMs. The significant volume reductions obtained were 45% in the VM group and 76% in the LM group (p = 0.003 and p = 0.009, respectively). Significant reductions in discomfort/pain and in cosmetic disfigurement were obtained in both groups. An overall improvement was reported by 69% and 82% of patients in the VM and LM groups, respectively. No major complications occurred during the mean follow-up of 51 ± 34 months in the VM group and 29 ± 18 months in the LM group. A recurrence developed within 2 years in 23% of patients. Bleomycin is clinically and radiologically effective for the treatment of venous and lymphatic malformations, with a high level of patient safety.
RESUMO
Erdheim-Chester disease is a rare histiocytosis characterized by iconic features associated with compatible histology. Most patients have somatic mutations in the MAP-kinase pathway gene, and the mutations occur in CD14+ monocytes. Differentiation of the myeloid lineage plays a central role in the pathogenesis of histiocytosis. Monocytes are myeloid-derived white blood cells, divided into three subsets, but only the CD14++CD16- "classical monocyte" can differentiate into dendritic cells and tissue macrophages. Since most mutations occur in CD14+ cells and since ECD patients have a particular monocytic phenotype resembling CMML, we studied the correlation between disease activity and monocytic subset distribution during the course of a severe vascular form of ECD requiring liver transplantation. During early follow-up, increased CD14++CD16- "classical monocyte" associated with decreased CD14lowCD16++ "non-classical monocyte" correlated with disease activity. Further studies are needed to confirm the use of monocyte as a marker of disease activity in patients with ECD.
Assuntos
Doença de Erdheim-Chester/patologia , Falência Hepática Aguda/etiologia , Transplante de Fígado , Monócitos/imunologia , Monócitos/patologia , Adulto , Doença de Erdheim-Chester/complicações , Doença de Erdheim-Chester/imunologia , Evolução Fatal , Feminino , Humanos , Imunofenotipagem , Falência Hepática Aguda/patologia , Falência Hepática Aguda/cirurgiaRESUMO
OBJECTIVE: Peritoneal or mesenteric tumours may correspond to several tumour types or tumour-like conditions, some of them being represented by histiocytosis. This rare condition often poses diagnostic difficulties that can lead to important time delay in targeted therapies. Our aim was to describe main features of histiocytoses with mesenteric localisation that can improve the diagnostic process. DESIGN: We performed a retrospective study on 22 patients, whose peritoneal/mesenteric biopsies were infiltrated by histiocytes. RESULTS: Abdominal pain was the revealing symptom in 10 cases, and 19 patients underwent surgical biopsies. The diagnosis of histiocytosis was proposed by initial pathologists in 41% of patients. The other initial diagnoses were inflammation (n=7), sclerosing mesenteritis (n=4) and liposarcoma (n=1). The CD163/CD68+CD1a- histiocytes infiltrated subserosa and/or deeper adipose tissues in 16 and 14 cases, respectively. A BRAFV600E mutation was detected within the biopsies in 11 cases, and two others were MAP2K1 mutated. The final diagnosis was histiocytosis in 18 patients, 15 of whom had Erdheim-Chester disease. The median diagnostic delay of histiocytosis was 9 months. Patients treated with BRAF or MEK inhibitors showed a partial response or a stable disease. One patient died soon after surgery, and five died by the progression of the disease. CONCLUSION: Diagnosis of masses arising in the mesentery should be carefully explored as one of the possibilities in histiocytosis. This diagnosis is frequently missed on mesenteric biopsies. Molecular biology for detecting the mutations in BRAF or in genes of the MAP kinase pathway is a critical diagnostic tool.