1. Antenatal manifestation of congenital pancreatoblastoma in a fetus with Beckwith-Wiedemann syndrome
- Author
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G. P. e. l. i. z. z. o., . G. Conoscenti, Vesce, Fortunato, Guerrini, . P., Cavazzini AUTHOR CORRECTIONS: Two of the author names in the above paper, . L., published in the April issue of Prenatal Diagnosis, The correct names are Fortunato Vesce, were supplied e. r. r. o. n. e. o. u. s. l. y., and The authors would like to apologise for any inconvenience caused, Pietro G. u. e. r. r. i. n. i.
- Subjects
congenital, hereditary, and neonatal diseases and abnormalities ,Pediatrics ,medicine.medical_specialty ,Pathology ,Pancreatic disease ,Beckwith-Wiedemann Syndrome ,Pregnancy, High-Risk ,Pancreatoblastoma ,Beckwith–Wiedemann syndrome ,Prenatal diagnosis ,Ultrasonography, Prenatal ,Pregnancy ,Neoplasms ,medicine ,complications/pathology/ultrasonography ,Humans ,Prenatal ,Cyst ,Tomography ,Genetics (clinical) ,Ultrasonography ,Fetus ,business.industry ,Infant, Newborn ,Obstetrics and Gynecology ,Infant ,complications/surgery/ultrasonography ,Neoplasms, Germ Cell and Embryonal ,Middle Aged ,medicine.disease ,complications/pathology/ultrasonography, Female, Humans, Infant ,Newborn, Maternal Age, Middle Aged, Neoplasms ,Germ Cell and Embryonal ,complications/surgery/ultrasonography, Pancreatic Neoplasms ,complications/surgery/ultrasonography, Pregnancy, Pregnancy ,High-Risk, Tomography ,X-Ray Computed, Treatment Outcome, Ultrasonography ,Newborn ,Uniparental disomy ,X-Ray Computed ,Pancreatic Neoplasms ,medicine.anatomical_structure ,Treatment Outcome ,Female ,High-Risk ,Pancreas ,business ,Tomography, X-Ray Computed ,Maternal Age - Abstract
Antenatal detection of an isolated abdominal cyst was found to be a pancreatoblastoma in a female fetus with Beckwith-Wiedemann syndrome. Prenatal and post-natal features and management of this very rare tumour are discussed. Molecular investigation disclosed a mosaic paternal 11p15 uniparental disomy in the tumoral cells. The prognosis of a congenital pancreatoblastoma is good if complete surgical excision is achieved. However, the association with Beckwith-Wiedemann syndrome requires a prolonged follow-up because of the increased risk of developing malignant tumours.
- Published
- 2003