Wilms Tumor With Raised Serum Alpha-Fetoprotein: Highlighting the Need for Novel Circulating Biomarkers.

Autor: Green R; Department of Paediatric Haematology and Oncology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK., Ahmed A; Department of Paediatrics, Norfolk and Norwich University Hospitals NHS Foundation Trust, Norwich, Norfolk, UK., Fleming B; Department of Radiology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK., Long AM; Department of Paediatric Surgery, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK., Behjati S; Department of Paediatric Haematology and Oncology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.; Wellcome Trust Sanger Institute, Hinxton, Cambridge, UK., Trotman J; East Genomics Laboratory Hub (GLH) Genetics Laboratory, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK., Tarpey P; East Genomics Laboratory Hub (GLH) Genetics Laboratory, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK., Nicholson JC; Department of Paediatric Haematology and Oncology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.; Department of Paediatrics, Level 8, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK., Coleman N; Department of Paediatric Histopathology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.; Department of Pathology, University of Cambridge, Cambridge, UK., Elizabeth Hook C; Department of Paediatric Histopathology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.; Department of Pathology, University of Cambridge, Cambridge, UK., Murray MJ; Department of Paediatric Haematology and Oncology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.; Department of Pathology, University of Cambridge, Cambridge, UK.
Jazyk: angličtina
Zdroj: Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society [Pediatr Dev Pathol] 2024 May-Jun; Vol. 27 (3), pp. 260-265. Date of Electronic Publication: 2023 Dec 14.
DOI: 10.1177/10935266231213467
Abstrakt: Wilms tumor (WT) is the commonest cause of renal cancer in children. In Europe, a diagnosis is made for most cases on typical clinical and radiological findings, prior to pre-operative chemotherapy. Here, we describe a case of a young boy presenting with a large abdominal tumor, associated with raised serum alpha-fetoprotein (AFP) levels at diagnosis. Given the atypical features present, a biopsy was taken, and histology was consistent with WT, showing triphasic WT, with epithelial, stromal, and blastemal elements present, and positive WT1 and CD56 immunohistochemical staining. During pre-operative chemotherapy, serial serum AFP measurements showed further increases, despite a radiological response, before a subsequent fall to normal following nephrectomy. The resection specimen was comprised of ~55% and ~45% stromal and epithelial elements, respectively, with no anaplasia, but immunohistochemistry using AFP staining revealed positive mucinous intestinal epithelium, consistent with the serum AFP observations. The lack of correlation between tumor response and serum AFP levels in this case highlights a more general clinical unmet need to identify WT-specific circulating tumor markers.
Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Databáze: MEDLINE