Lineage-Independent Tumors in Bilateral Neuroblastoma.
N Engl J Med
; 383(19): 1860-1865, 2020 11 05.
Article
en En
| MEDLINE
| ID: mdl-33211929
ABSTRACT
Childhood tumors that occur synchronously in different anatomical sites usually represent metastatic disease. However, such tumors can be independent neoplasms. We investigated whether cases of bilateral neuroblastoma represented independent tumors in two children with pathogenic germline mutations by genotyping somatic mutations shared between tumors and blood. Our results suggested that in both children, the lineages that had given rise to the tumors had segregated within the first cell divisions of the zygote, without being preceded by a common premalignant clone. In one patient, the tumors had parallel evolution, including distinct second hits in SMARCA4, a putative predisposition gene for neuroblastoma. These findings portray cases of bilateral neuroblastoma as having independent lesions mediated by a germline predisposition. (Funded by Children with Cancer UK and Wellcome.).
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Neoplasias de las Glándulas Suprarrenales
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Neoplasias Abdominales
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Neoplasias Primarias Múltiples
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Neuroblastoma
Límite:
Child, preschool
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Female
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Humans
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Male
Idioma:
En
Revista:
N Engl J Med
Año:
2020
Tipo del documento:
Article