Factors possibly affecting prognosis in children with Wilms' tumor diagnosed before 24 months of age

A report from the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) Wilms Tumor Working Group

Paolo D'Angelo, Andrea Di Cataldo, Monica Terenziani, Gianni Bisogno, Paola Collini, Martina Di Martino, Fraia Melchionda, Clara Mosa, Marilina Nantron, Daniela Perotti, Giuseppe Puccio, Annalisa Serra, Serena Catania, Filippo Spreafico, AIEOP Wilms Tumor Working Group

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Abstract

BACKGROUND: Children with Wilms' tumor (WT) aged under 24 months (infants) have a better prognosis than older patients. Our aim was to study the epidemiology of this age group, with focus on the modality of diagnosis, tumor size, and association with malformations/syndromes, seeking to understand if any of these factors might be related to prognosis.

PATIENTS AND METHODS: Infants diagnosed with WT between 2003 and February 2010 were evaluated. A query form was used to collect data on the modality of WT diagnosis (symptomatic or incidental), tumor volume, maximum diameter, site, and stage.

RESULTS: Data were collected for 117 of 124 WT infants registered. Twenty-four cases had an incidental diagnosis (ID) of renal mass, usually arising from an abdominal ultrasound performed for other reasons, and 93 had been diagnosed based on clinical signs/symptoms. The incidental cohort displayed unifocal disease, mean tumor diameter 5.52 cm, mean tumor volume 84.30 ml, and 14 patients showed associated malformations. Symptomatic patients had mean maximum tumor diameter of 10.18 cm, mean tumor volume of 451.18 ml, and six had associated malformations.

CONCLUSIONS: Our study showed that 20% of the infants had an ID of WT; they had a relatively smaller nonmetastatic tumor and a higher rate of malformations than infants of the symptomatically diagnosed group, but we did not detect any difference in age at diagnosis between the two groups. Conversely, we found a significant difference in the 5-year event-free survival rate (P = 0.018) between infants under 1 year (96%), more frequently associated with congenital malformations, and infants 1-2 years (80%).

Original languageEnglish
JournalPediatric Blood and Cancer
Volume64
Issue number12
DOIs
Publication statusPublished - Dec 2017

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Wilms Tumor
Tumor Burden
Neoplasms
Disease-Free Survival
Signs and Symptoms
Epidemiology
Survival Rate
Age Groups
Kidney

Keywords

  • Age Factors
  • Congenital Abnormalities
  • Female
  • Humans
  • Infant
  • Kidney Neoplasms
  • Male
  • Prognosis
  • Retrospective Studies
  • Wilms Tumor
  • Journal Article

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Factors possibly affecting prognosis in children with Wilms' tumor diagnosed before 24 months of age : A report from the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) Wilms Tumor Working Group. / D'Angelo, Paolo; Di Cataldo, Andrea; Terenziani, Monica; Bisogno, Gianni; Collini, Paola; Di Martino, Martina; Melchionda, Fraia; Mosa, Clara; Nantron, Marilina; Perotti, Daniela; Puccio, Giuseppe; Serra, Annalisa; Catania, Serena; Spreafico, Filippo; AIEOP Wilms Tumor Working Group.

In: Pediatric Blood and Cancer, Vol. 64, No. 12, 12.2017.

Research output: Contribution to journalArticle

D'Angelo, P, Di Cataldo, A, Terenziani, M, Bisogno, G, Collini, P, Di Martino, M, Melchionda, F, Mosa, C, Nantron, M, Perotti, D, Puccio, G, Serra, A, Catania, S, Spreafico, F & AIEOP Wilms Tumor Working Group 2017, 'Factors possibly affecting prognosis in children with Wilms' tumor diagnosed before 24 months of age: A report from the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) Wilms Tumor Working Group', Pediatric Blood and Cancer, vol. 64, no. 12. https://doi.org/10.1002/pbc.26644
D'Angelo, Paolo ; Di Cataldo, Andrea ; Terenziani, Monica ; Bisogno, Gianni ; Collini, Paola ; Di Martino, Martina ; Melchionda, Fraia ; Mosa, Clara ; Nantron, Marilina ; Perotti, Daniela ; Puccio, Giuseppe ; Serra, Annalisa ; Catania, Serena ; Spreafico, Filippo ; AIEOP Wilms Tumor Working Group. / Factors possibly affecting prognosis in children with Wilms' tumor diagnosed before 24 months of age : A report from the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) Wilms Tumor Working Group. In: Pediatric Blood and Cancer. 2017 ; Vol. 64, No. 12.
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abstract = "BACKGROUND: Children with Wilms' tumor (WT) aged under 24 months (infants) have a better prognosis than older patients. Our aim was to study the epidemiology of this age group, with focus on the modality of diagnosis, tumor size, and association with malformations/syndromes, seeking to understand if any of these factors might be related to prognosis.PATIENTS AND METHODS: Infants diagnosed with WT between 2003 and February 2010 were evaluated. A query form was used to collect data on the modality of WT diagnosis (symptomatic or incidental), tumor volume, maximum diameter, site, and stage.RESULTS: Data were collected for 117 of 124 WT infants registered. Twenty-four cases had an incidental diagnosis (ID) of renal mass, usually arising from an abdominal ultrasound performed for other reasons, and 93 had been diagnosed based on clinical signs/symptoms. The incidental cohort displayed unifocal disease, mean tumor diameter 5.52 cm, mean tumor volume 84.30 ml, and 14 patients showed associated malformations. Symptomatic patients had mean maximum tumor diameter of 10.18 cm, mean tumor volume of 451.18 ml, and six had associated malformations.CONCLUSIONS: Our study showed that 20{\%} of the infants had an ID of WT; they had a relatively smaller nonmetastatic tumor and a higher rate of malformations than infants of the symptomatically diagnosed group, but we did not detect any difference in age at diagnosis between the two groups. Conversely, we found a significant difference in the 5-year event-free survival rate (P = 0.018) between infants under 1 year (96{\%}), more frequently associated with congenital malformations, and infants 1-2 years (80{\%}).",
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T1 - Factors possibly affecting prognosis in children with Wilms' tumor diagnosed before 24 months of age

T2 - A report from the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) Wilms Tumor Working Group

AU - D'Angelo, Paolo

AU - Di Cataldo, Andrea

AU - Terenziani, Monica

AU - Bisogno, Gianni

AU - Collini, Paola

AU - Di Martino, Martina

AU - Melchionda, Fraia

AU - Mosa, Clara

AU - Nantron, Marilina

AU - Perotti, Daniela

AU - Puccio, Giuseppe

AU - Serra, Annalisa

AU - Catania, Serena

AU - Spreafico, Filippo

AU - AIEOP Wilms Tumor Working Group

N1 - © 2017 Wiley Periodicals, Inc.

PY - 2017/12

Y1 - 2017/12

N2 - BACKGROUND: Children with Wilms' tumor (WT) aged under 24 months (infants) have a better prognosis than older patients. Our aim was to study the epidemiology of this age group, with focus on the modality of diagnosis, tumor size, and association with malformations/syndromes, seeking to understand if any of these factors might be related to prognosis.PATIENTS AND METHODS: Infants diagnosed with WT between 2003 and February 2010 were evaluated. A query form was used to collect data on the modality of WT diagnosis (symptomatic or incidental), tumor volume, maximum diameter, site, and stage.RESULTS: Data were collected for 117 of 124 WT infants registered. Twenty-four cases had an incidental diagnosis (ID) of renal mass, usually arising from an abdominal ultrasound performed for other reasons, and 93 had been diagnosed based on clinical signs/symptoms. The incidental cohort displayed unifocal disease, mean tumor diameter 5.52 cm, mean tumor volume 84.30 ml, and 14 patients showed associated malformations. Symptomatic patients had mean maximum tumor diameter of 10.18 cm, mean tumor volume of 451.18 ml, and six had associated malformations.CONCLUSIONS: Our study showed that 20% of the infants had an ID of WT; they had a relatively smaller nonmetastatic tumor and a higher rate of malformations than infants of the symptomatically diagnosed group, but we did not detect any difference in age at diagnosis between the two groups. Conversely, we found a significant difference in the 5-year event-free survival rate (P = 0.018) between infants under 1 year (96%), more frequently associated with congenital malformations, and infants 1-2 years (80%).

AB - BACKGROUND: Children with Wilms' tumor (WT) aged under 24 months (infants) have a better prognosis than older patients. Our aim was to study the epidemiology of this age group, with focus on the modality of diagnosis, tumor size, and association with malformations/syndromes, seeking to understand if any of these factors might be related to prognosis.PATIENTS AND METHODS: Infants diagnosed with WT between 2003 and February 2010 were evaluated. A query form was used to collect data on the modality of WT diagnosis (symptomatic or incidental), tumor volume, maximum diameter, site, and stage.RESULTS: Data were collected for 117 of 124 WT infants registered. Twenty-four cases had an incidental diagnosis (ID) of renal mass, usually arising from an abdominal ultrasound performed for other reasons, and 93 had been diagnosed based on clinical signs/symptoms. The incidental cohort displayed unifocal disease, mean tumor diameter 5.52 cm, mean tumor volume 84.30 ml, and 14 patients showed associated malformations. Symptomatic patients had mean maximum tumor diameter of 10.18 cm, mean tumor volume of 451.18 ml, and six had associated malformations.CONCLUSIONS: Our study showed that 20% of the infants had an ID of WT; they had a relatively smaller nonmetastatic tumor and a higher rate of malformations than infants of the symptomatically diagnosed group, but we did not detect any difference in age at diagnosis between the two groups. Conversely, we found a significant difference in the 5-year event-free survival rate (P = 0.018) between infants under 1 year (96%), more frequently associated with congenital malformations, and infants 1-2 years (80%).

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KW - Humans

KW - Infant

KW - Kidney Neoplasms

KW - Male

KW - Prognosis

KW - Retrospective Studies

KW - Wilms Tumor

KW - Journal Article

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DO - 10.1002/pbc.26644

M3 - Article

VL - 64

JO - Pediatric Blood and Cancer

JF - Pediatric Blood and Cancer

SN - 1545-5009

IS - 12

ER -