20 Citations (Scopus)

Abstract

Costello Syndrome is a specific MCA/MR syndrome mainly characterized by dysmorphic facial features, peculiar biphasic growth pattern, motor and mental retardation, ectodermal anomalies involving skin and nails, and age dependent development of nasal and perianal papillomata. Heart malformations and/or hypertrophic cardiomyopathy are frequently observed. We report a 4-year-old girl with Costello syndrome who developed an intrathoracic ganglioneuroblastoma. In previous reports two patients with ectodermal tumours have been described, a ganglioneuroblastoma of the adrenal gland and an epithelioma. This third report suggests that neural crest neoplasia may be a significant risk factor for children with Costello syndrome. (C) 2000 Lippincott Williams and Wilkins.

Original languageEnglish
Pages (from-to)265-268
Number of pages4
JournalClinical Dysmorphology
Volume9
Issue number4
Publication statusPublished - 2000

Fingerprint

Costello Syndrome
Ganglioneuroblastoma
Neoplasms
Congenital Heart Defects
Neural Crest
Hypertrophic Cardiomyopathy
Papilloma
Adrenal Glands
Nails
Nose
Intellectual Disability
Carcinoma
Skin
Growth

Keywords

  • Costello syndrome
  • Malignancy
  • Neuroblastoma

ASJC Scopus subject areas

  • Anatomy
  • Genetics(clinical)
  • Pediatrics, Perinatology, and Child Health

Cite this

Costello syndrome : A cancer predisposing syndrome? / Moroni, I.; Bedeschi, F.; Luksch, R.; Casanova, M.; D'Incerti, L.; Uziel, G.; Selicorni, A.

In: Clinical Dysmorphology, Vol. 9, No. 4, 2000, p. 265-268.

Research output: Contribution to journalArticle

@article{64de4718c35e485ca8f77fbe629717ec,
title = "Costello syndrome: A cancer predisposing syndrome?",
abstract = "Costello Syndrome is a specific MCA/MR syndrome mainly characterized by dysmorphic facial features, peculiar biphasic growth pattern, motor and mental retardation, ectodermal anomalies involving skin and nails, and age dependent development of nasal and perianal papillomata. Heart malformations and/or hypertrophic cardiomyopathy are frequently observed. We report a 4-year-old girl with Costello syndrome who developed an intrathoracic ganglioneuroblastoma. In previous reports two patients with ectodermal tumours have been described, a ganglioneuroblastoma of the adrenal gland and an epithelioma. This third report suggests that neural crest neoplasia may be a significant risk factor for children with Costello syndrome. (C) 2000 Lippincott Williams and Wilkins.",
keywords = "Costello syndrome, Malignancy, Neuroblastoma",
author = "I. Moroni and F. Bedeschi and R. Luksch and M. Casanova and L. D'Incerti and G. Uziel and A. Selicorni",
year = "2000",
language = "English",
volume = "9",
pages = "265--268",
journal = "Clinical Dysmorphology",
issn = "0962-8827",
publisher = "Lippincott Williams and Wilkins",
number = "4",

}

TY - JOUR

T1 - Costello syndrome

T2 - A cancer predisposing syndrome?

AU - Moroni, I.

AU - Bedeschi, F.

AU - Luksch, R.

AU - Casanova, M.

AU - D'Incerti, L.

AU - Uziel, G.

AU - Selicorni, A.

PY - 2000

Y1 - 2000

N2 - Costello Syndrome is a specific MCA/MR syndrome mainly characterized by dysmorphic facial features, peculiar biphasic growth pattern, motor and mental retardation, ectodermal anomalies involving skin and nails, and age dependent development of nasal and perianal papillomata. Heart malformations and/or hypertrophic cardiomyopathy are frequently observed. We report a 4-year-old girl with Costello syndrome who developed an intrathoracic ganglioneuroblastoma. In previous reports two patients with ectodermal tumours have been described, a ganglioneuroblastoma of the adrenal gland and an epithelioma. This third report suggests that neural crest neoplasia may be a significant risk factor for children with Costello syndrome. (C) 2000 Lippincott Williams and Wilkins.

AB - Costello Syndrome is a specific MCA/MR syndrome mainly characterized by dysmorphic facial features, peculiar biphasic growth pattern, motor and mental retardation, ectodermal anomalies involving skin and nails, and age dependent development of nasal and perianal papillomata. Heart malformations and/or hypertrophic cardiomyopathy are frequently observed. We report a 4-year-old girl with Costello syndrome who developed an intrathoracic ganglioneuroblastoma. In previous reports two patients with ectodermal tumours have been described, a ganglioneuroblastoma of the adrenal gland and an epithelioma. This third report suggests that neural crest neoplasia may be a significant risk factor for children with Costello syndrome. (C) 2000 Lippincott Williams and Wilkins.

KW - Costello syndrome

KW - Malignancy

KW - Neuroblastoma

UR - http://www.scopus.com/inward/record.url?scp=0033804358&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0033804358&partnerID=8YFLogxK

M3 - Article

VL - 9

SP - 265

EP - 268

JO - Clinical Dysmorphology

JF - Clinical Dysmorphology

SN - 0962-8827

IS - 4

ER -