Renal Cell Carcinoma in Children: The Detroit Experience

Abstract

Although renal cell carcinoma in childhood often presents at an advanced stage, the prognosis for those with isolated regional lymph node involvement appears to be encouraging.

Adult Xp11 Translocation Renal Cell Carcinoma Diagnosed by Cytogenetics and Immunohistochemistry

“…Generally, nodal metastases, even in the absence of distant metastases, portend ominous prognosis in RCC patients (16). The characteristics of our two cases with nodal involvement (cases 1 and 2) were similar to those of certain pediatric RCC cases with isolated lymph node involvement in which prolonged survival was reported (10). Very recently, Geller et al (14) also reported that nodal disease without distant metastasis (TxN1-3M0) has a favorable short-term prognosis after surgery alone in Xp11 translocation RCC patients.…”

Chromophobe Cell Renal Carcinoma in Childhood

“…Similarly only 0.3% to 1.3% of all RCC present during childhood. 5 The suggests a clinical similarity between adult and pediatric RCC except that in children the sex ratio is equal, minor trauma frequently participates in the presentation (as observed in our patient), and there is increased survival of children with isolated lymph node involvement.…”

Renal-cell carcinoma in children: A different disorder from its adult counterpart?

“…It occurs primarily in middle-aged to elderly adults, with less than 1% of cases occurring below the age of 20 years [3,9]. Speculation exists as to whether RCC in children represents a different entity from its adult counterpart.…”

“…Previous retrospective studies of RCC in children [2,3,5,[15][16][17][18][19][20][21][22][23] collectively show a mean age at presentation of 9-11 years; a lack of a male predominance; a 30-60% incidence of pain, mass, and hematuria, with virtual absence of the triad of all three; and a 50-70% overall survival, with a dismal prognosis for metastatic disease. Our results were similar, but in addition, we found a higher frequency of paraneoplastic phenomena (31%); two patients with polycythemia, four with hypertension, one with fever, and one with weight loss.…”

“…In contrast, RCC in children is rare, representing less than 0.1% of all malignant tumors and only 2-6% of all renal tumors [2]. In children, RCC presents at a mean age of 9-10 years, with an equal male-to-female ratio [3]. Furthermore, RCC in adults is correlated with several environmental factors (cigarette smoking, obesity, analgesic abuse, exposure to asbestos or tanning products, long-term dialysis) [4], whereas in children no such correlation has been found [5].…”