Renal Embriyonal Neoplaziler
Özet
Nefrojenik kalıntılar (NK), fetal böbrek gelişimi sırasında tam olarak gerilemeyen ve nefroblastom (Wilms tümörü) için prekürsör lezyon olarak kabul edilen immatür renal dokulardır. Prenatal dönemde metanefrik mezenşimin farklılaşmasının tamamlanamaması sonucu gelişirler ve özellikle prematüre bebeklerde daha sık görülür. Genellikle asemptomatiktirler ve rastlantısal olarak saptanırlar; nadiren hipertansiyon veya hematüri ile belirti verebilirler. Görüntülemede, ultrasonografide küçük hipoekoik odaklar, BT/MRG’de minimal kontrast tutan küçük nodüller şeklinde izlenir. Makroskopik olarak, iyi sınırlı ve 0,5–2 cm çapında lezyonlardır. Mikroskopik olarak, blastemal, epitelyal ve stromal bileşenler içerir; hücresel atipi minimaldir. İmmünohistokimyasal olarak WT1 ve PAX8 pozitif, keratin negatif veya zayıf pozitiftir. Histokimyasal olarak PAS boyası ile az miktarda glikojen gösterilebilir. Moleküler düzeyde WT1 gen mutasyonları ve IGF2 gen ekspresyon artışı bildirilmiştir. Wilms tümörüne dönüşüm riski nedeniyle hastalar düzenli takip edilmelidir. Wilms tümöründen ayrımı, destrüktif büyümenin ve belirgin atipinin olmaması ile yapılır. Nefrojenik kalıntıların tanınması, aşırı tanı ve gereksiz nefrektomilerin önlenmesi açısından önemlidir.
Referanslar
Van den Heuvel-Eibrink, M.M.; Hol, J.A.; Pritchard-Jones, K.; van Tinteren, H.; Furtwangler, R.; Verschuur, A.C.; Vujanic, G.M.; Leuschner, I.; Brok, J.; Rübe, C.; et al. Position paper: Rationale for the treatment of Wilms tumour in the UMBRELLA SIOP-RTSG 2016 protocol. Nat. Rev. Urol. 2017, 14, 743–752. [CrossRef] [PubMed]
Van Den Hoek, J.; De Krijger, R.; Van De Ven, K.; Lequin, M.; Van Den Heuvel-Eibrink, M.M. Cystic nephroma, cystic partially differentiated nephroblastoma and cystic Wilms’ tumor in children: A spectrum with therapeutic dilemmas. Urol. Int. 2009, 82, 65–70. [CrossRef]
Luithle, T.; Szavay, P.; Furtwängler, R.; Graf, N.; Fuchs, J. Treatment of Cystic Nephroma and Cystic Partially Differentiated Nephroblastoma-A Report From the SIOP/GPOH Study Group. J. Urol. 2007, 177, 294–296. [CrossRef]
Cajaiba, M.M.; Khanna, G.; Smith, E.A.; Gellert, L.; Chi, Y.Y.; Mullen, E.A.; Hill, D.A.; Geller, J.I.; Dome, J.S.; Perlman, E.J. Pediatric cystic nephromas: Distinctive features and frequent DICER1 mutations. Hum. Pathol. 2016, 48, 81–87. [CrossRef] [PubMed]
Gimpel, C.; Avni, E.F.; Breysem, L.; Burgmaier, K.; Caroli, A.; Cetiner, M.; Haffner, D.; Hartung, E.A.; Franke, D.; König, J.; et al. Imaging of Kidney Cysts and Cystic Kidney Diseases in Children: An International Working Group Consensus Statement. Radiology 2019, 290, 769–782. [CrossRef]
Vujaniç, G.M.; Gessler, M.; Ooms, A.H.A.G.; Collini, P.; Coulomb-l’Hermine, A.; D’Hooghe, E.; de Krijger, R.R.; Perotti, D.; Pritchard-Jones, K.; Vokuhl, C.; et al. The UMBRELLA SIOP-RTSG 2016 Wilms tumour pathology and molecular biology protocol. Nat. Rev. Urol. 2018, 15, 693–701. [CrossRef] [PubMed]
Kurian, J.J.; Jehangir, S.; Korula, A. Multiloculated Cystic Renal Tumors of Childhood: Has the Final Word Been Spoken. J. Indian Assoc. Pediatr. Surg. 2018, 23, 22–26. [CrossRef]
Baker, J.M.; Viero, S.; Kim, P.C.; Grant, R.M. Stage III cystic partially differentiated nephroblastoma recurring after nephrectomy and chemotherapy. Pediatr. Blood Cancer 2008, 50, 129–131. [CrossRef]
Billiet, I.; Van Poppel, H.; Baert, L. Actual approach to cystic nephroma. Eur. Urol. 1988, 14, 280–286. [CrossRef] [PubMed]
Wu, M.K.; Cotter, M.B.; Pears, J.; McDermott, M.B.; Fabian, M.R.; Foulkes, W.D.; O’Sullivan, M.J. Tumor progression in DICER1-mutated cystic nephroma—witnessing the genesis of anaplastic sarcoma of the kidney. Hum. Pathol. 2016, 53, 114–120. [CrossRef]
Faria, P.A.; Zerbini, M.C. Dedifferentiated cystic nephroma with malignant mesenchymoma as the dedifferentiated component. Pediatr. Pathol. Lab. Med. J. Soc. Pediatr. Pathol. Affil. Int. Paediatr. Pathol. Assoc. 1996, 16, 1003–1011.
Pastore, G. et al. Malignant renal tumours incidence and survival in European children (1978–1997): report from the Automated Childhood Cancer Information System project. Eur. J. Cancer 42, 2103–2114 (2006).
Nakata, K., Colombet, M., Stiller, C. A., Pritchard-Jones, K. & Steliarova-Foucher, E. Incidence of childhood renal tumours: an international population-based study. Int. J. Cancer 147, 3313–3327 (2020).
Shamberger, R. C. et al. Intravascular extension of Wilms tumor. Ann. Surg. 234, 116–121 (2001).
Ritchey, M. et al. Ureteral extension in Wilms’ tumor: a report from the National Wilms’ Tumor Study Group (NWTSG). J. Pediatr. Surg. 43, 1625–1629 (2008).
Gow, K. W. et al. Primary nephrectomy and intraoperative tumor spill: report from the Children’s Oncology Group (COG) renal tumors committee. J. Pediatr. Surg. 48, 34–38 (2013).
Ehrlich, P. F. et al. Surgical protocol violations in children with renal tumors provides an opportunity to improve pediatric cancer care: a report from the Children’s Oncology Group. Pediatr. Blood Cancer 63, 1905–1910 (2016).
Aldrink, J. H. et al. Technical considerations for nephron-sparing surgery in children: what is needed to preserve renal units? J. Surg. Res. 232, 614–620 (2018).
Murphy, A. & Davidoff, A. Bilateral Wilms tumor: a surgical perspective. Children 5, 134 (2018). 145. Cox, S., Büyükünal, C. & Millar, A. J. W. Surgery for the complex Wilms tumour. Pediatr. Surg. Int. 36, 113–127 (2020).
Malek, M. M. et al. Minimally invasive surgery for pediatric renal tumors: a systematic review by the APSA Cancer Committee. J. Pediatr. Surg. 55, 2251–2259 (2020).
Fernandez, C. V. et al. Clinical outcome and biological predictors of relapse after nephrectomy only for very low-risk Wilms tumor: a report from Children’s Oncology Group AREN0532. Ann. Surg. 265, 835–840 (2017).
Green, D. M. The treatment of stages I-IV favorable histology Wilms’ tumor. J. Clin. Oncol. 22, 1366–1372 (2004).
Green, D. M. The evolution of treatment for Wilms tumor. J. Pediatr. Surg. 48, 14–19 (2013).
Qian Zhao Qi Xiong , Qi Song . Metastatic adult Wilms' tumor managed by chemotherapy, immunotherapy and target therapy: a case report. 2024 May 16;10(1): FSO915. doi: 10.2144/fsoa-2023-0156. eCollection 2024
Dix, D. B. et al. Augmentation of therapy for combined loss of heterozygosity 1p and 16q in favorable histology Wilms tumor: a Children’s Oncology Group AREN0532 and AREN0533 study report. J. Clin. Oncol. 37, 2769–2777 (2019).
