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2017 ; 8
(1
): 4-8
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Management of Bilateral Wilms Tumor: Our Experience
#MMPMID28127175
Raghunath Bv
; Jadhav V
; Shankar G
; Narendrababu M
; Ramesh S
Indian J Surg Oncol
2017[Mar]; 8
(1
): 4-8
PMID28127175
show ga
Management of bilateral Wilms' tumor is particularly challenging, considering the
chances of recurrence and long-term renal function for affected patients.
Aggressive surgical resection to prevent recurrence must be balanced with the
desire to preserve renal function. We evaluated our experience in the management
of bilateral Wilms' tumor stressing the challenges encountered in decision making
and the role of nephron sparing surgery. We had four children presenting with
bilateral Wilms' tumor. All of them were appropriately staged and given standard
chemotherapy as per NWTS-5 guidelines. Tumors were considered to have a 'good'
response to chemotherapy if sufficient tumor shrinkage was observed so that renal
hilum was seen free of tumor and vice versa. Nephron-sparing surgery was
considered in all and was performed when feasible, followed by completion
adjuvant chemotherapy. All patients were followed up with serial ultrasound scans
(3-6 monthly) and CECT abdomen (yearly once). Blood urea and serum creatinine,
hypertension, and proteinuria were assessed during follow-up visits. All four
children received neo-adjuvant chemotherapy as per NWTS-5 guidelines. The first
child had poor response to chemotherapy and was considered for left radical and
right partial nephrectomy. However, patient attenders refused any surgical
intervention and the child was taken home. The second child had a 'good' response
on left side and was planned for left partial and right radical
nephroureterectomy based on pre-operative imaging analysis. However,
intra-operatively, bilateral partial nephrectomy with good margins was feasible.
The third child though, showed 'poor' response to pre-op chemotherapy and
warranted bilateral nephroureterectomy, right partial and left radical
nephroureterectomy was feasible. However, in the fourth child, we were not able
to perform nephron sparing surgery and left nephroureterectomy with right tumor
biopsy was done. Following this, child was started on 2nd-line chemotherapy, now
awaiting right partial nephrectomy. The second child is on follow up for
1.5 years, doing well. However, the third child expired 1.5 years following
surgery due to recurrence (lung metastasis). Management of bilateral Wilm's tumor
is challenging and nephron-sparing surgery should be considered in all patients
having bilateral Wilm's tumor with favorable histology, even if pre-operative
imaging studies suggest that the lesions are unresectable.
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