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Wilms tumor with Mulibrey Nanism: A case report and review of literature

Authors :
Karthik Upasana
Dhwanee Thakkar
Dheeraj Gautam
Manvinder Singh Sachdev
Anjali Yadav
Rohit Kapoor
Veena Raghunathan
Maninder Singh Dhaliwal
Kartikeya Bhargava
Sandhya Nair
Jaiprakash Sharma
Neha Rastogi
Satya Prakash Yadav
Source :
Cancer Reports, Vol 5, Iss 5, Pp n/a-n/a (2022)
Publication Year :
2022
Publisher :
Wiley, 2022.

Abstract

Abstract Background Mulibrey‐Nanism (Muscle‐liver‐brain‐eye Nanism = dwarfism; MUL) is a rare genetic syndrome. The underlying TRIM37 mutation predisposes these children to develop tumors frequently. In the largest published series of MUL, 8% patients were reported to develop Wilms tumor (WT). The published literature lacks data regarding the best treatment protocol and outcome of this cohort of children with WT and MUL. We report here a 2‐year‐old boy with WT and MUL and present a review of literature on WT in MUL. Case Our patient had associated cardiac problems of atrial septal defect, atrial flutter and an episode of sudden cardiac arrest. We managed him successfully with chemotherapy, surgery and multi‐speciality care. He is alive and in remission at follow‐up of 6 months. Conclusion A total of 14 cases (including present case) of WT have been reported in MUL and treatment details were available for six cases. They were managed primarily with surgery, chemotherapy with/without radiotherapy, and all achieved remission. The outcome data is available only for two cases, one has been followed up till 15 years post treatment for WT and other is our patient.

Details

Language :
English
ISSN :
25738348
Volume :
5
Issue :
5
Database :
Directory of Open Access Journals
Journal :
Cancer Reports
Publication Type :
Academic Journal
Accession number :
edsdoj.2ec838b354554bf4a08ff1cbbe70baf6
Document Type :
article
Full Text :
https://doi.org/10.1002/cnr2.1512