Tumorprädispositionssyndrome und Nephroblastom

Welter, Nils; Furtwängler, Rhoikos; Schneider, Gregoire; Graf, Norbert; Schenk, Jens‐Peter

doi:10.1007/s00117-022-01056-w

Cited by 4 publications

(5 citation statements)

References 44 publications

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“…Welter et al. also discussed the importance of early diagnosis in CPS associated with WT 27 being in accordance with our findings that patients with tumor‐related symptoms have larger tumor volumes and more metastatic disease. In addition, Qian et al 18 .…”

Section: Discussionsupporting

confidence: 92%

“…This will result in less late effects and better quality of life, which underlines that an early diagnosis of WT, characterized by missing tumorrelated symptoms, is relevant. Welter et al also discussed the importance of early diagnosis in CPS associated with WT 27 being in accordance with our findings that patients with tumor-related symptoms have larger tumor volumes and more metastatic disease. In addition, Qian et al 18 could show that older age in patients diagnosed with WT is independent of other factors associated with worse OS, which further emphasizes the need to ensure early diagnosis, In osteosarcoma, the prognostic impact of diagnostic and treatment delay resulting in higher tumor load was analyzed by Vasquez et al finding that latency to diagnosis did not influence EFS and OS, but outcome was poorer in patients with a longer time to complete treatment.…”

Section: Outcome Analysissupporting

confidence: 92%

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The impact of the route to diagnosis in nephroblastoma

Mergen,

Welter,

Furtwängler

et al. 2024

Cancer Medicine

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IntroductionWilms 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.MethodsWe 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.ResultsOf 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.ConclusionThe 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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Section: Discussionsupporting

confidence: 92%

Section: Outcome Analysissupporting

confidence: 92%

The impact of the route to diagnosis in nephroblastoma

Mergen,

Welter,

Furtwängler

et al. 2024

Cancer Medicine

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“…Wilms tumor should always be suspected in the presence of a palpable abdominal mass in childhood, and abdominal ultrasonography is the simplest noninvasive diagnostic method that can aid in the differential diagnosis of hydronephrosis or other benign lesions of the kidney. On ultrasound, Wilms' tumor has the characteristics of a solid and inhomogeneous mass, in the context of which hypoechoic areas may be highlighted as the expression of intratumor necrosis [28]. On the other hand, contrast-enhanced computed tomography is more accurate and has the advantage of precisely defining the intra-abdominal extension of the tumor (for example, the invasion of lymph nodes or the presence of thrombi in the renal vein or the vena cava).…”

Section: Diagnosismentioning

confidence: 99%

Wilms Tumor: Updates about Pathogenesis and New Possible Clinical Treatments of the Most Frequent Pediatric Urogenital Cancer: A Narrative Review

Perrotta,

Castellani

2023

Surgeries

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Background: Wilms tumor (or nephroblastoma) is a malignant and solid neoplasm that derives from the primitive renal bud. It represents the most frequent primary tumor of the urogenital tract in childhood, and treatment consists of surgery and chemo-radiotherapy. However, concerning quality of life, the new therapeutic frontier is exploring other safer and potentially more effective options, such as minimally invasive surgery and biological drugs. Method: Literature (PubMed) from January 2013 to July 2023 was reviewed, checking for innovations in diagnosis and treatment. Results: A total of 130 articles was included in the review. Conclusions: In addition to the therapeutic strategies already identified, such as classic surgery and pharmacological therapies, recent studies focus attention on the new frontiers of minimally invasive surgery, such as diagnostics using biomarkers and immunotherapy, which could represent a new therapeutic option and is possibly less risky than in the past, contributing in fact to the current knowledge of the scientific panorama in terms of “tumor microenvironment” and systemic implications deriving from oncological disease.

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“…On ultrasound, Wilm's tumor has the characteristics of a solid and inhomogeneous mass, in the context of which hypoechoic areas may be highlighted as the expression of intratumor necrosis. [23] On the other hand, contrast-enhanced computed tomography is more accurate and has the advantage of precisely defining the intra-abdominal extension of the tumor (for example, the invasion of lymph nodes or the presence of thrombi in the renal vein or the vena cava). Magnetic resonance imaging defines the invasion of the great vessels much better than ultrasound [24] or computed tomography [25] or Positron Emission Tomography [26], although the latter is rarely used in U.S. medical practice.…”

Section: Diagnosismentioning

confidence: 99%

Updates about Wilms' Tumor: Pathogenesis, and New Possible Treatments of the Most Frequent Pediatric Urogenital Cancer

Perrotta¹,

Castellani²

2023

Preprint

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Background: Wilms' tumor (or nephroblastoma) is a malignant and solid neoplasm that derives from the primitive renal bud. It represents the most frequent primary tumor of the urogenital tract in childhood, and treatment consists of surgery and chemo-radiotherapy. However, concerning the quality of life, the new therapeutic frontier is exploring other safer and potentially more effective options, such as minimally invasive surgery and biological drugs. Method: Literature (Pubmed) from January 2013 to March 2023 was reviewed. Results: One hundred five articles were included in the review. Conclusion: In addition to the therapeutic strategies already identified, such as classic surgery and pharmacological therapies, recent studies focus attention on the new frontiers of minimally invasive surgery, such as diagnostics using biomarkers and immunotherapy, which could represent a new therapeutic option and possibly less risky than in the past, contributing in fact to the current knowledge of the scientific panorama in terms of "tumor microenvironment" and systemic implications deriving from oncological disease.

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Tumorprädispositionssyndrome und Nephroblastom

Cited by 4 publications

References 44 publications

The impact of the route to diagnosis in nephroblastoma

The impact of the route to diagnosis in nephroblastoma

Wilms Tumor: Updates about Pathogenesis and New Possible Clinical Treatments of the Most Frequent Pediatric Urogenital Cancer: A Narrative Review

Updates about Wilms' Tumor: Pathogenesis, and New Possible Treatments of the Most Frequent Pediatric Urogenital Cancer

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