|Year : 2015 | Volume
| Issue : 2 | Page : 134-135
Neoadjuvant chemotherapy in neonatal Wilms' tumor
Ramesh K Reddy, Lavanya Kannaiyan, Srinivas Srirampur, Gulam Mohammed Irfan, Subba Rao
Department of Pediatric Surgery, Niloufer Hospital, Hyderabad, India
|Date of Web Publication||12-Jun-2015|
Dr. Ramesh K Reddy
Plot no. 36, Snehapuri Colony, Nacharam, Hyderabad - 500 076
Source of Support: None, Conflict of Interest: None
Neonatal presentation of Wilms' tumor is rare with an incidence of 0.16%. The treatment protocol has to be tailored according to the need. Most studies recommend nephroureterectomy followed by chemotherapy. We present our experience with a baby who came with stage 2 Wilms' tumor in the neonatal period and was treated using neoadjuvant chemotherapy.
Keywords: Neonate, Wilms′ tumor, neoadjuvant chemotherapy
|How to cite this article:|
Reddy RK, Kannaiyan L, Srirampur S, Irfan GM, Rao S. Neoadjuvant chemotherapy in neonatal Wilms' tumor. J NTR Univ Health Sci 2015;4:134-5
| Introduction|| |
Wilms' tumor is the most common abdominal malignancy in infancy, after neuroblastoma. The treatment protocols are well established in national Wilms tumour study (NWTS) and International Society of Pediatric Oncology (SIOP) protocols. Neonatal presentation of Wilms' tumor is rare. The current NWTS and SIOP protocols suggest primary nephrouretectomy followed by chemotherapy with dose reduction. Our patient presented with a large Wilms' tumor which was treated by nephroureterectomy following reduction in size of the lesion, with neoadjuvant chemotherapy tailored for the case.
| Case Report|| |
A 20-day-old baby was brought for a large abdominal mass. Upon investigation it was found to be a renal mass measuring 72 × 71 × 91 mm, crossing the midline. It was not invading the vessels. A trucut needle biopsy revealed a triphasic Wilms' tumor [Figure 1]. In view of the size of the mass and concerns regarding spillage during surgery, neoadjuvant chemotherapy was decided. Baby received 4 cycles of chemotherapy with Actinomycin-D and Vincristine at half the calculated dose. Chemotherapy was well tolerated and there was significant reduction in the size of the lesion. The child underwent nephroureterectomy. Postoperative chemotherapy was continued for 24 weeks [Figure 2]. Radiotherapy was not administered. At follow-up, after 1 year, the child was thriving well without any recurrence.
|Figure 1: CT abdomen showing large left renal tumor with local infiltration|
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| Discussion|| |
Neonatal renal tumors constitute 7% of all neonatally detected tumors.  Congenital mesenchymal nephroblastoma is the most common of these tumors. Also, 15%  of the patients are diagnosed antenatally. Some are associated with Beckwith-Wiedemann syndrome More Details or WAGR syndrome or Denys-Drash syndrome. , It has been observed that even aggressive tumors in neonates have a better prognosis than a presentation at an older age. 
Ritchey et al.  studied the management of Wilms' tumors presenting in the neonatal period. They studied 15 newborns. All underwent nephroureterectomy. Ten received adjuvant chemotherapy with 50% dose reduction (as recommended by NWTS-3 protocol). Among the five babies who did not receive adjuvant chemotherapy or radiotherapy, one developed recurrence. NWTS-5 recommends primary nephroureterectomy followed by radiation and chemotherapy in all children. In infants less than 1 year of age, 50% dose reduction is recommended due to increased incidence of toxicity. , Radiation is avoided due to long-term sequelae.  Stage I patients are treated with surgery alone. Stage II and above are treated with chemotherapy regimen depending on the extent of the lesion and loss of heterozygosity (LOH) at markers. Neoadjuvant chemotherapy is recommended in tumors associated with syndromes, unilateral kidney tumors, or bilateral tumors.  Patients with nephrogenic rests need close follow-up to detect metachronus tumors.  The event-free survival in neonatal Wilms' is 86% and overall survival is 93%. 
According to the SIOP protocol, Powis  recommended nephrectomy in patients of less than 6 months of age with unilateral renal tumors. In those patients with inoperable or bilateral tumors, neoadjuvant chemotherapy and partial nephrectomy are recommended. A 50% dose reduction of chemotherapy is recommended in infants less than 6 months of age.
In our patient, the tumor appeared to be non-resectable due to massive size crossing the midline. Neoadjuvant chemotherapy was given (DD-4A) for 4 cycles. There was a marked reduction in size of the tumor and nephroureterectomy was done. The child completed the remaining course of the chemotherapy. Patient was able to tolerate the chemotherapy without adverse effects.
In conclusion, neonatal Wilms' tumor can be successfully treated with a combination of surgery and neoadjuvant chemotherapy where indicated.
| References|| |
Ritchey ML, Azizkhan RG, Beckwith JB, Hrabovsky EE, Haase GM. Neonatal Wilms' Tumour. J Pediatr Surg 1995;30:856-9.
Morgan E, Baum E, Breslow N, Takashima J, D'Angio G. Chemotherapy-related toxicity in infants treated according to the Second National Wilms' Tumor Study. J Clin Oncol 1988;6:51-5.
Corn BW, Goldwein JW, Evans I, D'Angio GJ. Outcomes in low-risk babies treated with half-dose chemotherapy according to the Third National Wilms' Tumor Study. J Clin Oncol 1992;10:1305-9.
Evans AE, Norkool P, Evans I, Breslow N, D'Angio GJ. Late effects of treatment for Wilms' tumor: A report from the National Wilms' Tumor Study Group. Cancer 1991;67:331-6.
Powis M. Neonatal renal Tumours. Early Hum Dev 2010;86:607-12.
[Figure 1], [Figure 2]