[Intraductal breast papillomas in patients with Costello syndrome].
Costello syndrome is a multisystemic congenital disorder with a very low prevalence. The pathogenesis remains unclear and predisposes to the development of tumors of ectodermal origin. Diagnosis is clinical, based on findings of mental and growth retardation and a characteristic phenotype. We report the case of a patient with Costello syndrome who was referred to our unit with a suspected diagnosis of intraductal papilloma based on the presence of various episodes of nipple discharge. Postoperative histopathological study confirmed the diagnosis of multiple intraductal papilloma. We review the literature on the topic and discuss the advisability of aggressive surgical therapy, given the predisposition of these patients to develop both benign and malignant tumors.
Arpa
Eva
E
Servicio de Obstetricia y Ginecología, Hospital Virgen del Camino, Pamplona, Navarra, España. eva.arpa@gmail.com
Domínguez-Cunchillos
Fernando
F
Martínez-Montero
Isabel
I
De Miguel
Concepción
C
Moras
Natalia
N
D000328:Adult; D001706:Biopsy; D001943:Breast Neoplasms / Q000150:complications* / Q000473:pathology* / Q000601:surgery; D002892:Chromosomes, Human, Pair 22 / Q000235:genetics; D004547:Elastic Tissue / Q000378:metabolism*; D005260:Female; D006801:Humans; D008607:Intellectual Disability / Q000150:complications*; D007818:Laryngeal Diseases / Q000150:complications* / Q000235:genetics / Q000378:metabolism*; D008408:Mastectomy; D018300:Papilloma, Intraductal / Q000150:complications* / Q000473:pathology* / Q000601:surgery; D010608:Pharyngeal Diseases / Q000150:complications* / Q000235:genetics / Q000378:metabolism*; D010641:Phenotype; D017354:Point Mutation / Q000235:genetics; D013577:Syndrome