Renal cell carcinoma in children: a clinicopathologic study.
OBJECTIVE
To identify the prognostic factors, treatment, and outcome of children affected by renal cell carcinoma (RCC).


METHODS
The series included 41 patients (18 males and 23 females) with a median age of 124 months observed at the 11 Italian Association for Pediatric Hematology and Oncology centers from January 1973 to January 2001. Clinical data, surgical notes, pathologic findings, and summaries of therapy were taken from the charts.


RESULTS
Seven (17%) of the 41 patients had a papillary histology, and 34 (82.4%) had nonpapillary histology. Eighteen patients (43.9%) had stage I, one patient (2.4%) had stage II, two patients (4.8%) had stage IIIA, 10 patients (24.3%) had stage IIIB, and nine patients (21.9%) had stage IV disease. One patient had a bilateral involvement at diagnosis. Seven patients experienced disease recurrence. Lung and liver were the most common distant lesions and usually were fatal. In this study, the major factor influencing the prognosis was the stage. Event-free survival at 20 years was 53.5% for all patients. Overall survival at 20 years was 54.9% for all patients.


CONCLUSIONS
RCC is a rare disease in children and adolescents. This neoplasm has a different clinical presentation in children compared with adults but the same outcome. In our experience, patients with localized disease could be cured by nephrectomy alone. Prospective studies in a larger number of patients are needed to confirm radiation therapy and biologic response modifiers as effective adjunct therapy in RCC stage III. The alternative therapy seems warranted in patients with advanced disease.
Indolfi
Paolo
P
Pediatric Oncology Service-Pediatric Department II, University of Napoli, Napoli, Italy. paolo.indolfi@unina2.it
Terenziani
Monica
M
Casale
Fiorina
F
Carli
Modesto
M
Bisogno
Gianni
G
Schiavetti
Amalia
A
Mancini
Antonia
A
Rondelli
Roberto
R
Pession
Andrea
A
Jenkner
Alessandro
A
Pierani
Paolo
P
Tamaro
Paolo
P
De Bernardi
Bruno
B
Ferrari
Andrea
A
Santoro
Nicola
N
Giuliano
Maria
M
Cecchetto
Giovanni
G
Piva
Luigi
L
Surico
Gianmarco
G
Di Tullio
M Teresa
MT
D000293:Adolescent; D002292:Carcinoma, Renal Cell / Q000473:pathology* / Q000532:radiotherapy / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007680:Kidney Neoplasms / Q000473:pathology* / Q000532:radiotherapy / Q000601:surgery; D008297:Male; D009362:Neoplasm Metastasis; D009364:Neoplasm Recurrence, Local; D009367:Neoplasm Staging; D009392:Nephrectomy; D011379:Prognosis; D012307:Risk Factors; D016896:Treatment Outcome