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Non-invasive differentiation of paediatric kidney tumours is particularly important in the SIOP-RTSG protocols, which recommend pre-operative chemotherapy without histological confirmation. The identification of clinical and tumour-related parameters may enhance diagnostic accuracy. Age, metastases, and tumour volume (TV) were retrospectively analysed in 3306 patients enrolled in SIOP/GPOH 9, 93-01, and 2001 including Wilms tumour (WT), congenital mesoblastic nephroma (CMN), clear cell sarcoma (CCSK), malignant rhabdoid tumour of the kidney (MRTK), and renal cell carcinoma (RCC). WT was diagnosed in 2927 (88.5%) patients followed by CMN 138 (4.2%), CCSK 126 (3.8%), MRTK 58 (1.8%) and RCC 57 (1.7%). CMN, the most common localized tumour (71.6%) in patients younger than 3 months of age, was diagnosed earliest and RCC the latest (median age [months]: 0 and 154, respectively) both associated with significantly smaller TV (median TV [mL]: 67.2 and 45.0, respectively). RCC occurred in >14% of patients older than 120 months or older than 84 months with TV <100 mL. Receiver operating characteristic analyses discriminated WT from CMN, RCC and MRTK regarding age (AUC = 0.976, 0.929 and 0.791) and TV (AUC = 0.768, 0.813 and 0.622). MRTK had the highest risk of metastasis (37.9%) despite young age, whereas the risk of metastasis increased significantly with age in WT. Age and TV at diagnosis can differentiate WT from CMN and RCC. MRTK must be considered for metastatic tumours at young age. Identification of CCSK without histology remains challenging. Combined with MRI-characteristics, including diffusion-weighted imaging, and radiomics and liquid biopsies in the future, our approach allows optimization of biopsy recommendations and prevention of misdiagnosis-based neoadjuvant treatment.
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Carcinoma de Células Renales , Neoplasias Renales , Nefroma Mesoblástico , Tumor Rabdoide , Tumor de Wilms , Humanos , Niño , Lactante , Carcinoma de Células Renales/diagnóstico , Carcinoma de Células Renales/patología , Estudios Retrospectivos , Neoplasias Renales/patología , Tumor de Wilms/diagnóstico , Tumor de Wilms/patología , Nefroma Mesoblástico/congénito , Nefroma Mesoblástico/patología , Nefroma Mesoblástico/cirugía , Tumor Rabdoide/diagnóstico , Tumor Rabdoide/patologíaRESUMEN
OBJECTIVE: This study aimed to identify parameters that allow the estimation of tumor-infiltrated lymph nodes (LN) after pretreatment for unilateral Wilms tumor (WT). SUMMARY BACKGROUND DATA: Complete tumor resection with removal of regional LN is always necessary. Positive LNs require local irradiation influencing benefits in case of NSS in long-term follow-up. Clinical and tumor-related data available at the time of surgery in combination with intraoperative findings (IAF) were used to estimate the LN status during surgery. METHODS: Altogether, 2115 patients with unilateral WT were prospectively enrolled in SIOP-93-01 / GPOH and SIOP-2001 / GPOH over a period of 30 years (1993-2023). LN infiltration by tumor was calculated for age, sex, metastases at diagnosis, tumor volume (TV), TV shrinkage, and intraoperative findings (IAF) using logistic regression models. RESULTS: Age ≥48 months (P<0.001, OR 2.17, CI 1.57 - 3.00), TV at diagnosis ≥300 (P<0.001, OR 3.72, CI 2.37 - 5.85), metastasis at diagnosis (P<0.001, OR 6.21, CI 4.47 - 8.62) and IAF (>1: P<0.001, OR 3.54, CI 2.13 - 5.88) correlated with positive LNs. TV shrinkage was not predictive of positive LN. Three flow charts were developed based on age, TV at diagnosis, metastasis, and IAF. These flowcharts defined risks between 0% and 41.5% for LN infiltration by tumor. CONCLUSIONS: The combination of age, TV at diagnosis, and metastasis with IAF allows the estimation of the frequency of positive LNs, which may help surgeons deciding about NSS.
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This narrative review discusses basic principles of the perioperative antibiotic prophylaxis (PAP) in premature and at term newborns and refers to some particularities concerning the indication and dosing issues. Although this is a vulnerable patient population, the spectrum of activity should not be unnecessarily broad and the regular PAP must not be prolonged beyond 24 hours.
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Profilaxis Antibiótica , Neonatología , Recién Nacido , Humanos , Antibacterianos/uso terapéuticoRESUMEN
PURPOSE: To our knowledge, there is no German study, which has examined the relationship between a postponement of surgery (from emergency service to standard working time) and the corresponding risk of postoperative complications in children and adolescents with acute appendicitis. The aim of this study is to examine if surgery of acute appendicitis in childhood can be postponed from night shift to the next working day without negative effects for the patient. METHODS: In a retrospective analysis (September 2001 to June 2007), the files of 225 paediatric surgical patients with acute appendicitis have been analysed concerning history, histology, course of treatment and development of complications. The cohort was divided into groups by their histology (common, "A", complicated appendicitis, "B") and by the median time (10 h) from admission to surgery (immediate, "C", delayed surgery, "D"). These groups have been analysed and compared. RESULTS: Groups A and B did not differ concerning time to admittance (p = 0.922). Seven patients developed complications (n = 7/225, 3.1%). Six complications were seen in group C (n = 6/113, 5.3%) compared to 1 in group D (n = 1/112, 0.9%), the difference was not significant (p = 0.119). None of the patients of group B developed complications after delayed surgery. Median follow-up was 10 days (IQR 7-15). CONCLUSIONS: In view of the development of complications, there was no evidence that the time between inpatient admission and surgery had any impact on the postoperative result. Thus, there is usually no need to perform surgery in common appendicitis during night shift.
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Apendicectomía/métodos , Apendicitis/cirugía , Complicaciones Posoperatorias/epidemiología , Tiempo de Tratamiento/tendencias , Apendicectomía/efectos adversos , Apendicitis/diagnóstico , Niño , Preescolar , Estudios de Cohortes , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Humanos , Masculino , Tempo Operativo , Pediatría , Complicaciones Posoperatorias/fisiopatología , Estudios Retrospectivos , Medición de Riesgo , Índice de Severidad de la Enfermedad , Factores de Tiempo , Resultado del Tratamiento , Espera Vigilante/métodosRESUMEN
Mycobacterium fortuitum peritonitis is a rare complication in patients undergoing continuous ambulatory peritoneal dialysis (CAPD). A 47-year-old patient was admitted to our tertiary hospital because of culture-negative peritonitis with persisting signs of infection despite adequate empirical antibiotic treatment. Although M. fortuitum was detected and the antibiotic regime subsequently amended, catheter removal was inevitable and the dialysis modality converted to hemodialysis (HD). After long-term antibiotic treatment and an additional latency of 4 months without signs of residual infection, reinitiation of CAPD was planned. Explorative laparoscopy prior to catheter reinsertion revealed multiple adhesions within the peritoneal cavity, preventing adequate catheter function. The clinical course of M. fortuitum peritonitis, the need for catheter removal and the description of peritoneal changes are discussed regarding to recent literature.
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Infecciones Relacionadas con Catéteres/microbiología , Fallo Renal Crónico/terapia , Infecciones por Mycobacterium no Tuberculosas/etiología , Mycobacterium fortuitum , Diálisis Peritoneal Ambulatoria Continua/efectos adversos , Peritonitis/etiología , Antibacterianos/uso terapéutico , Infecciones Relacionadas con Catéteres/diagnóstico , Infecciones Relacionadas con Catéteres/terapia , Catéteres de Permanencia , Remoción de Dispositivos , Humanos , Masculino , Persona de Mediana Edad , Infecciones por Mycobacterium no Tuberculosas/diagnóstico , Infecciones por Mycobacterium no Tuberculosas/terapia , Peritonitis/diagnóstico , Peritonitis/terapiaRESUMEN
INTRODUCTION: Wilms tumor (WT) is the most common childhood kidney cancer. It is a rapid growing embryonal tumor in young children and can be diagnosed with and without tumor related symptoms. METHODS: We retrospectively analyzed the route to diagnosis of WT treated prospectively according to the SIOP 93-01/GPOH and 2001/GPOH in Germany between 1993 and 2022. Four routes were defined: diagnosis due to tumor-related symptoms, incidental diagnosis during another disease, diagnosis by preventive examinations, and diagnosis within a surveillance program. For these groups we compared clinical and tumor characteristics and outcome. RESULTS: Of 2549 patients with WT 1822 (71.5%) were diagnosed by tumor-related symptoms, 472 (18.5%) incidentally, 213 (8.4%) by preventive medical examinations, and 42 (1.6%) by surveillance. Age, general health status, tumor volume, and local and overall stage varied significantly between these groups. The youngest patients were those diagnosed by preventive medical examination (mean: 1.70 years). These patients also showed the best general health status. Tumor volume at diagnosis (549 mL) and after preoperative chemotherapy (255 mL) was significantly higher for children with tumor-related symptoms. The highest percentage of local stage I (78.6%) and the lowest percentage of metastatic disease (4.8%) was found in the surveillance group. The outcome of patients was not significantly different, with up to 19.0% relapses in the surveillance group and 3.0% deaths in the group with tumor-related symptoms. CONCLUSION: The route to diagnosis of WT correlates with age, general health status, tumor volume, and stage distribution, but does not impact the outcome of patients. Nonetheless, diagnosis without tumor related symptoms results in lower treatment burden and thus improved quality of life.
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Neoplasias Renales , Tumor de Wilms , Humanos , Tumor de Wilms/diagnóstico , Tumor de Wilms/patología , Tumor de Wilms/terapia , Tumor de Wilms/mortalidad , Tumor de Wilms/epidemiología , Masculino , Femenino , Preescolar , Neoplasias Renales/diagnóstico , Neoplasias Renales/terapia , Neoplasias Renales/patología , Neoplasias Renales/mortalidad , Lactante , Estudios Retrospectivos , Niño , Alemania/epidemiología , Estadificación de Neoplasias , Carga Tumoral , AdolescenteRESUMEN
(1) Background: Wilms tumor (WT) treated preoperatively is cured in over 90% of cases. However, how long preoperative chemotherapy can be given is unknown. (2) Methods: 2561/3030 patients with WT (age < 18 years) treated between 1989 and 2022 according to SIOP-9/GPOH, SIOP-93-01/GPOH, and SIOP-2001/GPOH are retrospectively analyzed to assess the risk of time to surgery (TTS) for relapse-free survival (RFS) and overall survival (OS). (3) Results: TTS was calculated for all surgeries, with the mean being 39 days (38.5 ± 12.5) for unilateral tumors (UWT) and 70 days (69.9 ± 32.7) for bilateral disease (BWT). Relapse occurred in 347 patients, of which 63 (2.5%) were local, 199 (7.8%) were metastatic, and 85 (3.3%) were combined. Moreover, 184 patients (7.2%) died, 152 (5.9%) due to tumor progression. In UWT, recurrences and mortality are independent of TTS. For BWT without metastases at diagnosis, the incidence of recurrence is less than 18% up to 120 days and increases to 29% after 120 days, and to 60% after 150 days. The risk of relapse (Hazard Ratio) adjusted for age, local stage, and histological risk group increases to 2.87 after 120 days (CI 1.19-7.95, p = 0.022) and to 4.62 after 150 days (CI 1.17-18.26, p = 0.029). In metastatic BWT, no influence of TTS is detected. (4) Conclusions: The length of preoperative chemotherapy has no negative impact on RFS or OS in UWT. In BWT without metastatic disease, surgery should be performed before day 120, as the risk of recurrence increases significantly thereafter.
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(1) Background: Vena cava thrombus (VCT) is rare in Wilms tumor (WT) (4−10%). The aim of this study is to identify factors for an outcome to improve treatment for better survival. (2) Methods: 148/3015 patients with WT (aged < 18 years) and VCT, prospectively enrolled over a period of 32 years (1989−2020) by the German Society for Pediatric Oncology and Hematology (SIOP-9/GPOH, SIOP-93-01/GPOH and SIOP-2001/GPOH), are retrospectively analyzed to describe clinical features, response to preoperative chemotherapy (PC) (142 patients) and surgical interventions and to evaluate risk factors for overall survival (OS). (3) Results: 14 VCT regressed completely with PC and another 12 in parts. The thrombus was completely removed in 111 (85.4%), incompletely in 16 (12.3%), and not removed in 3 (2.3%). The type of removal is unknown in four patients. Patients without VCT have a significantly (p < 0.001) better OS (97.8%) than those with VCT (90.1%). OS after complete resection is (89.9%), after incomplete (93.8%) and with no resection (100%). Patients with anaplasia or stage IV without complete remission (CR) after PC had a significantly worse OS compared to the remaining patients with VCT (77.1% vs. 94.4%; p = 0.002). (4) Conclusions: As a result of our study, two risk factors for poor outcomes in WT patients with VCT emerge: diffuse anaplasia and metastatic disease, especially those with non-CR after PC.
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In the original article [...].
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(1) Background: about 10% of Wilms Tumor (WT) patients have a malformation or cancer predisposition syndrome (CPS) with causative germline genetic or epigenetic variants. Knowledge on CPS is essential for genetic counselling. (2) Methods: this retrospective analysis focused on 2927 consecutive patients with WTs registered between 1989 and 2017 in the SIOP/GPOH studies. (3) Results: Genitourinary malformations (GU, N = 66, 2.3%), Beckwith-Wiedemann spectrum (BWS, N = 32, 1.1%), isolated hemihypertrophy (IHH, N = 29, 1.0%), Denys-Drash syndrome (DDS, N = 24, 0.8%) and WAGR syndrome (N = 20, 0.7%) were reported most frequently. Compared to others, these patients were younger at WT diagnosis (median age 24.5 months vs. 39.0 months), had smaller tumors (349.4 mL vs. 487.5 mL), less often metastasis (8.2% vs. 18%), but more often nephroblastomatosis (12.9% vs. 1.9%). WT with IHH was associated with blastemal WT and DDS with stromal subtype. Bilateral WTs were common in WAGR (30%), DDS (29%) and BWS (31%). Chemotherapy induced reduction in tumor volume was poor in DDS (0.4% increase) and favorable in BWS (86.9% reduction). The event-free survival (EFS) of patients with BWS was significantly (p = 0.002) worse than in others. (4) Conclusions: CPS should be considered in WTs with specific clinical features resulting in referral to a geneticist. Their outcome was not always favorable.
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Bezoares , Síntomas Conductuales/complicaciones , Bezoares/diagnóstico , Bezoares/etiología , Bezoares/cirugía , Niño , Femenino , Humanos , SíndromeRESUMEN
PURPOSE: Rhabdoid tumor of the kidney (RTK) is one of the most aggressive childhood renal tumors. Overall survival ranges from 22% to 47%. The indication for radiation therapy (RT) in usually very young patients is an ongoing discussion. Recent protocols recommend RT independent of local stage, the latter being a good discriminator in other childhood kidney tumors. In this study, we analyze the evidence for RT in regard to risk factors, including tumor stage. METHODS AND MATERIALS: This study analyzed 58 patients with RTK from Austria, Switzerland, and Germany treated in the framework of 4 consecutive, prospective renal/rhabdoid tumor studies from 1991 to 2014. All treatment protocols included multimodality treatment, including high-intensity chemotherapy, surgery, and RT. RESULTS: Local stage distribution was not applicable, I, II, and III in 1, 6, 11, and 40, respectively. Twenty-nine (50%) patients had stage IV disease at diagnosis. Thirty-seven patients (64%) achieved complete remission, and 49% (18/37) relapsed. Thirty-four patients (60%) patients had progressive disease and died, 17 had local disease, 10 had combined disease, and 7 had distant disease; 2 treatment-related deaths were reported (3%). Twenty-one patients received RT during first-line treatment, 18 of them to all involved sites. Eight of the 34 cases of progressive disease occurred in irradiated patients. The local failure rate of treated patients with local stage II or III disease was 29% (6/18) in patients irradiated to all sites compared with 68% (15/22) in nonirradiated patients. One of 6 stage I patients received RT, and 1 patient experienced distant relapse (2-year progression-free and overall survival both 83% ± 15%). Progression-free survival for local stage II and III disease treated with RT, adjusted for early relapse or treatment abandonment, was 67% ± 11%, compared with 15% ± 7% without RT (P < .0001). CONCLUSION: The 68% local failure rate in nonirradiated patients underlines the importance of local treatment. Our experience supports the use of RT for local control in higher stage disease. In contrast, no local relapse in 6 local stage I patients, including 5 nonirradiated patients, suggests omission of RT in this favorable subset of usually infant patients with RTK.
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Neoplasias Renales/radioterapia , Tumor Rabdoide/radioterapia , Austria , Preescolar , Terapia Combinada/métodos , Alemania , Humanos , Lactante , Estimación de Kaplan-Meier , Neoplasias Renales/mortalidad , Neoplasias Renales/patología , Neoplasias Renales/cirugía , Recurrencia Local de Neoplasia , Estadificación de Neoplasias , Supervivencia sin Progresión , Dosificación Radioterapéutica , Inducción de Remisión , Tumor Rabdoide/mortalidad , Tumor Rabdoide/patología , Tumor Rabdoide/cirugía , Factores de Riesgo , Estadísticas no Paramétricas , SuizaRESUMEN
We present a female neonate with a sternal cleft (SC) and additional aortic aneurysm who presented with respiratory failure. Stabilization of the SC was achieved by using the xyphoid process as an autologous graft bridging the upper part of the SC. We conclude that a step-wise correction of the SC with the use of an autologous graft may improve respiratory function, and should be considered when complete surgical correction is not feasible.
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Aspergilosis , Recién Nacido , Humanos , Aspergilosis/microbiología , Antifúngicos/uso terapéutico , Hongos , VoriconazolRESUMEN
Interleukin-1 receptor-associated kinase 4 (IRAK-4) deficiency predisposes to severe invasive bacterial infections in infancy and early childhood, often with a fatal course caused by a defect in Toll-like receptor and interleukin-1 receptor signaling. Despite severe invasive infections, acute phase responses are often diminished. We report the successful treatment of a child with multiple liver abscesses, diaphragm perforation and pleural empyema, accompanied by strong acute phase responses as a unique presentation of IRAK-4 deficiency.