Wilms tumor, pleuropulmonary blastoma, and DICER1: case report and literature review.
World J Surg Oncol
; 16(1): 164, 2018 Aug 10.
Article
in En
| MEDLINE
| ID: mdl-30097050
ABSTRACT
BACKGROUND:
Pleuroblastoma (PPB) is a rare pediatric tumor which, in 30% of cases, is associated with cystic nephroma. It has been recently linked to the DICER1 mutation as part of a predisposition syndrome for various tumors. However, if DICER 1 anomalies have been reported in patients with Wilms tumor (WT), to date, no cases of PPB, WT, and DICER1 mutations have been reported in the same patient. CASE PRESENTATION We report the case of a 3-year-old patient, initially managed for metastatic WT. During his clinical course, the diagnosis of a PPB was made after detecting the DICER1 mutation and subsequent management was therefore modified.CONCLUSION:
This case highlights that in case of simultaneous discovery of a renal tumor and a pulmonary lesion in a child, the DICER 1 mutations should be looked for as these could help adapt management and schedule the surgical procedures.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Pulmonary Blastoma
/
Wilms Tumor
/
Ribonuclease III
/
DEAD-box RNA Helicases
/
Kidney Neoplasms
/
Lung Neoplasms
Type of study:
Prognostic_studies
Limits:
Child, preschool
/
Female
/
Humans
Language:
En
Journal:
World J Surg Oncol
Year:
2018
Document type:
Article
Affiliation country:
France