Metastatic renal cell carcinoma in children and adolescents: A 30-year unsuccessful story

Paolo Indolfi, Filippo Spreafico, Paola Collini, Giovanni Cecchetto, Fiorina Casale, Monica Terenziani, Amalia Schiavetti, Paolo Pierani, Luigi Piva, Daniela Cuzzubbo, Maria D. De Pasquale, Elvira Pota, Alessandro Inserra, Gianni Bisogno

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

BACKGROUND: Because of the rare occurrence of renal cell carcinoma (RCC) among children very little is known about this malignancy in pediatric age. We aimed adding knowledge on the clinical characteristics and outcome of metastatic (m) RCC in children and adolescents. PATIENTS AND METHODS: The series included 14 stage 4 RCC patients with a median age at diagnosis of 155.5 months, observed at the Italian Pediatric Hematology and Oncology Association (AIEOP) centers from January 1973 to November 2010. We were able to reevaluate histopatology of 11 out of the 14 patients and perform immunostaining for TFE3 in 9 out of the 11 patients. RESULTS: Of the 14 patients under study, 5 (3 girls) had a translocation morphology TFE+ RCC, 2 were reassigned as papillary type 1 or 2, respectively, 2 tumor specimens with primary clear cell histology had confirmed the initial histologic diagnosis, and 2-whose biopsy specimen was insufficient-had the diagnosis of RCC not further specified with subtyping. In the remaining 3 cases, the initial diagnosis of clear cell carcinoma was left. Overall, 6 patients received chemotherapy, 9 immunotherapy, and 2 adjuvant antiangiogenic therapy. Overall, 11 patients (78.5%) never achieved complete remission and died from progressive disease 1 to 16 months after diagnosis (median overall survival 5.5 mo). Three patients, 2 of whom received adjuvant antiangiogenic therapy, relapsed to lung at 3, 6, and 8 months after diagnosis, and died 18, 32, and 33 months after diagnosis, respectively. CONCLUSIONS: Despite their possibly different biology, childhood and adult mRCC seems to be sharing comparable outcomes. Because of the very low incidence of mRCC (about 20%) in children and adolescents, an international pediatric cooperation to address biological studies and assess the novel targeted approaches is needed.

Original languageEnglish
JournalJournal of Pediatric Hematology/Oncology
Volume34
Issue number7
DOIs
Publication statusPublished - Oct 2012

Fingerprint

Renal Cell Carcinoma
Pediatrics
International Cooperation
Polytetrafluoroethylene
Hematology
Immunotherapy
Neoplasms
Histology
Carcinoma
Biopsy
Drug Therapy
Lung
Survival
Incidence
Therapeutics

Keywords

  • metastatic renal cell carcinoma
  • pediatric
  • rare cancer
  • targeted therapy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Oncology
  • Hematology

Cite this

Metastatic renal cell carcinoma in children and adolescents : A 30-year unsuccessful story. / Indolfi, Paolo; Spreafico, Filippo; Collini, Paola; Cecchetto, Giovanni; Casale, Fiorina; Terenziani, Monica; Schiavetti, Amalia; Pierani, Paolo; Piva, Luigi; Cuzzubbo, Daniela; De Pasquale, Maria D.; Pota, Elvira; Inserra, Alessandro; Bisogno, Gianni.

In: Journal of Pediatric Hematology/Oncology, Vol. 34, No. 7, 10.2012.

Research output: Contribution to journalArticle

Indolfi, Paolo ; Spreafico, Filippo ; Collini, Paola ; Cecchetto, Giovanni ; Casale, Fiorina ; Terenziani, Monica ; Schiavetti, Amalia ; Pierani, Paolo ; Piva, Luigi ; Cuzzubbo, Daniela ; De Pasquale, Maria D. ; Pota, Elvira ; Inserra, Alessandro ; Bisogno, Gianni. / Metastatic renal cell carcinoma in children and adolescents : A 30-year unsuccessful story. In: Journal of Pediatric Hematology/Oncology. 2012 ; Vol. 34, No. 7.
@article{fca67aea8fd140978f69e9df1451c10a,
title = "Metastatic renal cell carcinoma in children and adolescents: A 30-year unsuccessful story",
abstract = "BACKGROUND: Because of the rare occurrence of renal cell carcinoma (RCC) among children very little is known about this malignancy in pediatric age. We aimed adding knowledge on the clinical characteristics and outcome of metastatic (m) RCC in children and adolescents. PATIENTS AND METHODS: The series included 14 stage 4 RCC patients with a median age at diagnosis of 155.5 months, observed at the Italian Pediatric Hematology and Oncology Association (AIEOP) centers from January 1973 to November 2010. We were able to reevaluate histopatology of 11 out of the 14 patients and perform immunostaining for TFE3 in 9 out of the 11 patients. RESULTS: Of the 14 patients under study, 5 (3 girls) had a translocation morphology TFE+ RCC, 2 were reassigned as papillary type 1 or 2, respectively, 2 tumor specimens with primary clear cell histology had confirmed the initial histologic diagnosis, and 2-whose biopsy specimen was insufficient-had the diagnosis of RCC not further specified with subtyping. In the remaining 3 cases, the initial diagnosis of clear cell carcinoma was left. Overall, 6 patients received chemotherapy, 9 immunotherapy, and 2 adjuvant antiangiogenic therapy. Overall, 11 patients (78.5{\%}) never achieved complete remission and died from progressive disease 1 to 16 months after diagnosis (median overall survival 5.5 mo). Three patients, 2 of whom received adjuvant antiangiogenic therapy, relapsed to lung at 3, 6, and 8 months after diagnosis, and died 18, 32, and 33 months after diagnosis, respectively. CONCLUSIONS: Despite their possibly different biology, childhood and adult mRCC seems to be sharing comparable outcomes. Because of the very low incidence of mRCC (about 20{\%}) in children and adolescents, an international pediatric cooperation to address biological studies and assess the novel targeted approaches is needed.",
keywords = "metastatic renal cell carcinoma, pediatric, rare cancer, targeted therapy",
author = "Paolo Indolfi and Filippo Spreafico and Paola Collini and Giovanni Cecchetto and Fiorina Casale and Monica Terenziani and Amalia Schiavetti and Paolo Pierani and Luigi Piva and Daniela Cuzzubbo and {De Pasquale}, {Maria D.} and Elvira Pota and Alessandro Inserra and Gianni Bisogno",
year = "2012",
month = "10",
doi = "10.1097/MPH.0b013e318267fb12",
language = "English",
volume = "34",
journal = "Journal of Pediatric Hematology/Oncology",
issn = "1077-4114",
publisher = "Lippincott Williams and Wilkins",
number = "7",

}

TY - JOUR

T1 - Metastatic renal cell carcinoma in children and adolescents

T2 - A 30-year unsuccessful story

AU - Indolfi, Paolo

AU - Spreafico, Filippo

AU - Collini, Paola

AU - Cecchetto, Giovanni

AU - Casale, Fiorina

AU - Terenziani, Monica

AU - Schiavetti, Amalia

AU - Pierani, Paolo

AU - Piva, Luigi

AU - Cuzzubbo, Daniela

AU - De Pasquale, Maria D.

AU - Pota, Elvira

AU - Inserra, Alessandro

AU - Bisogno, Gianni

PY - 2012/10

Y1 - 2012/10

N2 - BACKGROUND: Because of the rare occurrence of renal cell carcinoma (RCC) among children very little is known about this malignancy in pediatric age. We aimed adding knowledge on the clinical characteristics and outcome of metastatic (m) RCC in children and adolescents. PATIENTS AND METHODS: The series included 14 stage 4 RCC patients with a median age at diagnosis of 155.5 months, observed at the Italian Pediatric Hematology and Oncology Association (AIEOP) centers from January 1973 to November 2010. We were able to reevaluate histopatology of 11 out of the 14 patients and perform immunostaining for TFE3 in 9 out of the 11 patients. RESULTS: Of the 14 patients under study, 5 (3 girls) had a translocation morphology TFE+ RCC, 2 were reassigned as papillary type 1 or 2, respectively, 2 tumor specimens with primary clear cell histology had confirmed the initial histologic diagnosis, and 2-whose biopsy specimen was insufficient-had the diagnosis of RCC not further specified with subtyping. In the remaining 3 cases, the initial diagnosis of clear cell carcinoma was left. Overall, 6 patients received chemotherapy, 9 immunotherapy, and 2 adjuvant antiangiogenic therapy. Overall, 11 patients (78.5%) never achieved complete remission and died from progressive disease 1 to 16 months after diagnosis (median overall survival 5.5 mo). Three patients, 2 of whom received adjuvant antiangiogenic therapy, relapsed to lung at 3, 6, and 8 months after diagnosis, and died 18, 32, and 33 months after diagnosis, respectively. CONCLUSIONS: Despite their possibly different biology, childhood and adult mRCC seems to be sharing comparable outcomes. Because of the very low incidence of mRCC (about 20%) in children and adolescents, an international pediatric cooperation to address biological studies and assess the novel targeted approaches is needed.

AB - BACKGROUND: Because of the rare occurrence of renal cell carcinoma (RCC) among children very little is known about this malignancy in pediatric age. We aimed adding knowledge on the clinical characteristics and outcome of metastatic (m) RCC in children and adolescents. PATIENTS AND METHODS: The series included 14 stage 4 RCC patients with a median age at diagnosis of 155.5 months, observed at the Italian Pediatric Hematology and Oncology Association (AIEOP) centers from January 1973 to November 2010. We were able to reevaluate histopatology of 11 out of the 14 patients and perform immunostaining for TFE3 in 9 out of the 11 patients. RESULTS: Of the 14 patients under study, 5 (3 girls) had a translocation morphology TFE+ RCC, 2 were reassigned as papillary type 1 or 2, respectively, 2 tumor specimens with primary clear cell histology had confirmed the initial histologic diagnosis, and 2-whose biopsy specimen was insufficient-had the diagnosis of RCC not further specified with subtyping. In the remaining 3 cases, the initial diagnosis of clear cell carcinoma was left. Overall, 6 patients received chemotherapy, 9 immunotherapy, and 2 adjuvant antiangiogenic therapy. Overall, 11 patients (78.5%) never achieved complete remission and died from progressive disease 1 to 16 months after diagnosis (median overall survival 5.5 mo). Three patients, 2 of whom received adjuvant antiangiogenic therapy, relapsed to lung at 3, 6, and 8 months after diagnosis, and died 18, 32, and 33 months after diagnosis, respectively. CONCLUSIONS: Despite their possibly different biology, childhood and adult mRCC seems to be sharing comparable outcomes. Because of the very low incidence of mRCC (about 20%) in children and adolescents, an international pediatric cooperation to address biological studies and assess the novel targeted approaches is needed.

KW - metastatic renal cell carcinoma

KW - pediatric

KW - rare cancer

KW - targeted therapy

UR - http://www.scopus.com/inward/record.url?scp=84866952437&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84866952437&partnerID=8YFLogxK

U2 - 10.1097/MPH.0b013e318267fb12

DO - 10.1097/MPH.0b013e318267fb12

M3 - Article

C2 - 23007345

AN - SCOPUS:84866952437

VL - 34

JO - Journal of Pediatric Hematology/Oncology

JF - Journal of Pediatric Hematology/Oncology

SN - 1077-4114

IS - 7

ER -