Wilms' tumor surgery

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]

Overview

Surgery is the mainstay of treatment for wilms' tumor. There are two schools of thought as to the best therapy for wilms' tumor, The National Wilms' Tumor Study (NWTS) and The International Society of Paediatric Oncology(SIOP). Both have different approach to surgery.

Surgery

National Wilms Tumor Study (NWTS) / Children's Oncology Group (COG) approach: [1]

  • The most important role for the surgeon is to ensure complete tumor removal without rupture and assess the extent of disease.
  • Radical nephrectomy and lymph node sampling via a transabdominal or thoracoabdominal incision is the procedure of choice.
  • A flank incision is not performed because it provides limited exposure to the kidney.
  • For patients with resectable tumors, preoperative or intraoperative biopsy is not performed because it potentially upstages the tumor.
  • Routine exploration of the contralateral kidney is not necessary if technically adequate imaging studies do not suggest a bilateral process.
  • If the initial imaging studies are suggestive of bilateral kidney involvement, treatment approaches should facilitate renal-sparing surgery.
  • About 2% of Wilms tumors have ureteral involvement.
  • The presence of gross hematuria, nonfunctioning kidney, or hydronephrosis suggests the tumor may extend into the ureter, and cystoscopy is recommended.
  • En bloc resection to avoid tumor spill is recommended.
  • The surgeon needs to be aware of the risk of intraoperative spill, especially in patients who have right-sided and large tumors, as noted in a study of cases of intraoperative spill among 1,131 patients.[2]

Renal-sparing surgery remains controversial and is not recommended, except for children with the following:[3]

  • Predisposition to bilateral tumors. Some children who are predisposed to bilateral tumors and who have very small tumors detected by ultrasound screening may be considered for renal-sparing surgery to preserve renal tissue.
  • A solitary kidney.
  • Horseshoe kidney. Wilms tumor arising in a horseshoe kidney is rare, and accurate preoperative diagnosis is important for planning the operative approach. Primary resection is possible in most cases. Inoperable cases can usually be resected after chemotherapy.
  • Wilms tumor in infants with Denys-Drash or Frasier syndrome (to delay the need for dialysis).
  • The use of renal-sparing surgery for bilateral tumors is under investigation.
  • Renal-sparing surgery does not appear to be feasible in most patients at the time of diagnosis because of the location of the tumor within the kidney, even in those with very low risk.
  • In North America, renal-sparing surgery (partial nephrectomy) of unilateral Wilms tumor following administration of chemotherapy to shrink the tumor mass is considered investigational.
  • Hilar and periaortic lymph node sampling is appropriate even if the nodes appear normal.
  • Furthermore, any suspicious node basin is sampled.
  • Margins of resection, residual tumor, and any suspicious node basins are marked with titanium clips.
  • Wilms tumor rarely invades adjacent organs; therefore, resection of contiguous organs is rarely indicated.
  • There is an increased incidence of complications occurring in more extensive resections that involve removal of additional organs beyond the diaphragm and adrenal gland.
  • This has led to the recommendation in current COG protocols that these patients should be considered for initial biopsy, neoadjuvant chemotherapy, and then secondary resection.
  • Primary resection of liver metastasis is not recommended.

The SIOP approach :

  • In this approach chemotherapy is given to reduce the size of the tumor.
  • This makes surgical excision easier.
  • By this approach the risk of tumor spillage is drastically reduced.[4]
  • By doing this the complications of local radiation therapy can also be decreased.[5]

References

  1. National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/publications/pdq
  2. Veylon R (November 1978). "[Man and the environment. 6th congress of the International Society for Research on Diseases of Civilization and the Environment, Brussels, 6-9 September 1978]". Nouv Presse Med (in French). 7 (41): 3788, 3791. PMID 32528.
  3. Veylon R, Borthwick R, Mackenzie CP (November 1978). "[Man and the environment. 6th congress of the International Society for Research on Diseases of Civilization and the Environment, Brussels, 6-9 September 1978]". Nouv Presse Med (in French). 7 (41): 3788, 3791. PMID 32528.
  4. Fuchs J, Kienecker K, Furtwängler R, Warmann SW, Bürger D, Thürhoff JW, Hager J, Graf N (April 2009). "Surgical aspects in the treatment of patients with unilateral wilms tumor: a report from the SIOP 93-01/German Society of Pediatric Oncology and Hematology". Ann. Surg. 249 (4): 666–71. doi:10.1097/SLA.0b013e31819ed92b. PMID 19300220.
  5. D'Angio GJ (December 2003). "Pre- or post-operative treatment for Wilms tumor? Who, what, when, where, how, why--and which". Med. Pediatr. Oncol. 41 (6): 545–9. doi:10.1002/mpo.10395. PMID 14595712.

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