Year : 2009 | Volume
: 20 | Issue : 1 | Page : 124--126
Renal cell carcinoma in a child
Mehdi Salehipour1, Alireza Rasekhi2, Mohammad Vasei3, Abbas Hasanpour1,
1 Department of Urology, Shiraz University of Medical Sciences, Shiraz, Iran
2 Department of Radiology, Shiraz University of Medical Sciences, Shiraz, Iran
3 Department of Pathology, Shiraz University of Medical Sciences, Shiraz, Iran
Department of Urology, Faghihi Hospital, Zand Ave, Shiraz
Renal cell carcinoma is a rare disease in children and adolescent. Less than 2% of cases occur in childhood and no treatment protocols exist among urologists and oncologists for management. We present a case of renal cell carcinoma in a child with uncommon presentation.
|How to cite this article:|
Salehipour M, Rasekhi A, Vasei M, Hasanpour A. Renal cell carcinoma in a child.Saudi J Kidney Dis Transpl 2009;20:124-126
|How to cite this URL:|
Salehipour M, Rasekhi A, Vasei M, Hasanpour A. Renal cell carcinoma in a child. Saudi J Kidney Dis Transpl [serial online] 2009 [cited 2021 Aug 5 ];20:124-126
Available from: https://www.sjkdt.org/text.asp?2009/20/1/124/44719
Renal cell carcinoma (RCC) is the most common primary malignancy of the kidney in adults, and is rarely encountered in children. This tumor has a different clinical presentation in children compared with adults. The incidence of RCC in childhood is estimated as 0.1% to 0.3% of all neoplasms and 1.8% to 6.3% of all malignant renal tumors, while the incidence of Wilms' tumor is 58% to 87%. ,,, Proper therapy has not been defined for pediatric RCC yet. Surgery is the mainstay of the treatment and results in cure when the tumor is localized and completely resectable, but the effects of adjuvant therapy including chemotherapy, radiotherapy, and immunotherapy are unclear. 
Here we report a child who presented with RCC with fever.
A 3-year-old girl presented with fever 15 days prior to admission. The patient's medical history and physical examination were unremarkable. Urinalysis disclosed micro-hematuria (1015 RBC/HPF), pyuria (7-10 WBC/HPF) and urine culture was negative. Results of blood chemistry and liver function tests were normal. Compelete blood count revealed microcytic hypochromic anemia with elevated erythrocyte sedimentation rate (ESR) (1 hr =128mm and in 2 hr =145mm).
Abdominal ultrasonography (U.S) and computed tomography (CT)-scan showed a large heterogenous mass in the lower and mid-pole of the right kidney with enhancement [Figure 1]. Left kidney was normal without hydronephrosis or other abnormality. Other radiologic findings were normal. Abdominal exploration revealed no evidence of metastases, and right radical nephrectomy with regional lymphadenctomy was performed. Histopathologic examination disclosed that most of the tumor cells were large polygonal with relatively sharp cell borders and abundant pale acidophilic granular cytoplasm. The nuclei were mostly large with irregular borders and occasional unclear halo. Prominent eosinophilic nucleoli were easily observed in many cells [Figure 2]. Necrosis and mitosis were frequent. Immunohistochemical examination showed co-expression of vimentin and cytokeratin in the cytoplasm of the tumor cells. The histopathologic and immunohistological features were compatible with renal cell carcinoma granular variant with nuclear grade 2.
Postoperative course was uneventful. After operation, the patient's fever subsided and she was discharged on the 4th postoperative day. Last follow-up eight months later, she was well and without any evidence of recurrence and metastases.
RCC is extremely rare malignancy in children. RCC accountes for 1.4% of all renal tumors in patients younger those 4 years, 15.2% in patients aged 5 to 9 years and 52.6% in patients aged 10 to 15 years. 
RCC exhibits a 2:1 male predominance in adults, but in pediatric group no clear pattern exists. Differentiation between RCC and Wilm's tumor preoperatively is difficult. Wilm's tumor is diagnosed mainly in children less than 5 years and mostly presenting around 3 years, whereas RCC presents around the age of 8 to 9 years in most series. ,, Tumor calcification on plain x-ray is more common in RCC (approximately 25% of cases) than in Wilm's tumor (approximately 5%). 
The most common symptom in pediatric RCC is flank or abdominal pain, gross hematuria, and an abdominal mass. ,,
Although paraneoplastic signs are infrequently documented in children, but Selle et al reported a high frequency of general symptoms (42.5%) such as fever (22.5%), nausea/vomiting (17.5%), pallor (10%), malaise (10%), and weight loss (5%).  Our patient also presented with fever and high ESR similar to Selle's study.
Pediatric RCC not only shows a different morphologic spectrum, but also differs genetically from RCC in adult. Clear cell carcinoma is less frequent in younger age groups. VHL alteration are also uncommon.  In adults, RCC with nonpapillary histology is associated with translocation or terminal deletion of the short arm of chromosome  , but this chromosomal abnormality is uncommon in children with RCC.  On the other hand, papillary carcinomas and rare subtypes are relatively more frequent in the pediatric.  In contrast to adult RCC, a much larger proportion of RCC in the children would still be categorized as unclassified (24% in Bruder's study and 16% Selle's study as opposed to 5% in adult). ,
Incidence of metastatic disease, commonly to lung and bones, in children with RCC is similar to adults with half of the patients presenting with metastatic disease at the time of diagnosis. 
Radical nephrectomy is the treatment of choice for any stage of RCC. Post-operatively, radiotherapy and chemotherapy have been used in patients with advanced stages. ,
Although immunotherapy with interferon or interleukin has been reported for advanced cases, the beneficial effect of these treatment options are uncertain in children.  The overall prognosis in children with RCC appears to be similar to adults  and the most important prognostic factor is tumor staging.  Five years survival rate for patient with stage I is higher than 90% and 50-80% for patients with stage II and III and only 9% for patients with stage IV. , Our patient developed a low-stage and low grade RCC with favorable prognostic factors.
In conclusion, experience with pediatric RCC is limited and further studies are necessary to develop a standard therapeutic strategy.
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