Renal cell carcinoma in children

Abstract

Data from four pediatric hospitals concerning 20 children treated for renal cell carcinoma (RCC) from 1964-1978 were reviewed. Median age of the patients (pts) was 11.8 years (range, 14 months-19 years). Twelve were male and eight female; 17 were white and three black. Most patients presented with pain and hematuria with or without a palpable mass. An intrarenal tumor was detected at IV urography (17 pts), arteriography (2 pts), or at surgery (1 pt). Treatment consisted of nephrectomy in 15 pts, renal biopsy (4 pts), or no surgery (1 pt), followed by chemotherapy (5 pts), radiation therapy (1 pt), or both (7 pts). Ten pts died of distant metastases at a median of one year (range, 0.2 to two years) after diagnosis. The other 10 pts (50%) survive free of relapse at a median of 4 years (range, two to ten years) from diagnosis. Proportions surviving free of recurrent disease two or more years by National Wilms' Tumor Study (NWTS) Group were 5/5 in Group I, 3/7 in Group II, 1/3 in Group III, and 1/5 in Group IV; by age at diagnosis, 6/6 in those under 11 years old and 4/14 in those 11 or older; and by type of surgery, 10/15 who had nephrectomy and 0/5 with limited or no surgery. The data indicate that radiation and chemotherapy had only minor if any influences on relapse-free survival. We conclude that (1) RCC in children is similar to its counterpart in adults; (2) RCC has a worse prognosis than Wilms' tumor except for the earliest stage; (3) nephrectomy alone is adequate treatment for Group I RCC, and (4) young age (less than 11 years old) may be prognostically favorable.

Treatment of childhood renal cell carcinoma with lymph node metastasis: Two cases and a review of literature

“…Regional recurrence or metastasis can occur even in Stage I or II cases and over 5 years after tumor resection [4,19]. A much more favorable outcome is expected in young patients, especially those under 10 years of age [3][4][5][6][7][8]12]. Less than 30% of patients with lymph node metastasis survive 5 years [4], and nodal involvement occurs in 10-20% of adults [18] but in 30-40% of children with RCC [4,18].…”

“…Accurate staging is determined by histologic examination of the regional lymph node. Lymphadenectomy is considered effective for pediatric patients to prevent recurrence or metastasis, because risk of nodal involvement is generally higher in children than in adults [4,18] and patients with lymph node involvement have a significantly increased risk of developing distant metastases and a relatively increased risk of local recurrence [3,11,[18][19][20][21].…”

“…RCC has been reported at a frequency of 1-7% in pediatric renal malignant tumors, the second most common tumor after Wilms tumor, which accounts for approximately 85% [2][3][4][5][6][7][8][9]12]. Age of occurrence is usually greater in patients with RCC than in patients with Wilms tumor (around 80% of Wilms tumors occur in children under 3 years of age).…”

“…Though prognostic factors include patient age [3,4], tumor size [18,19], histologic pattern [6,9], vascular invasion [1,2,6], or chromosomal abnormality [14,15], the most important prognostic determinator is anatomical extent of the tumor, i.e., pathologic stage [2][3][4][5][6][7][8][13][14][15][16][17][18][19]. Two staging systems are mainly used, the Robson [26] and pTNM [18] systems.…”

“…Routine test showed normal liver function, but slight leucocytosis was observed (white cell count, WBC, 11,800/mm 3 ), increased C-reactive protein (CRP, 6+), and elevated erythrocyte sedimentation rate (ESR, 74 mm/hr). Symptoms disappeared within 2 days.…”