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

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...

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Published inCancer reports Vol. 5; no. 5; pp. e1512 - n/a
Main Authors Upasana, Karthik, Thakkar, Dhwanee, Gautam, Dheeraj, Sachdev, Manvinder Singh, Yadav, Anjali, Kapoor, Rohit, Raghunathan, Veena, Dhaliwal, Maninder Singh, Bhargava, Kartikeya, Nair, Sandhya, Sharma, Jaiprakash, Rastogi, Neha, Yadav, Satya Prakash
Format Journal Article
LanguageEnglish
Published United States John Wiley & Sons, Inc 01.05.2022
John Wiley and Sons Inc
Wiley
Subjects
Abdomen
atrial flutter
atrial septal defect
Biopsy
Case Report
Case Reports
Chemotherapy
COVID-19
Genes
Genetic testing
Genomes
Liver
Magnetic resonance imaging
Metastasis
Mulibrey Nanism
Mutation
Proteins
Signal transduction
Tomography
Wilms tumor
Mulibrey Nanism
atrial septal defect
atrial flutter
Wilms tumor
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Summary: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.
Bibliography:ObjectType-Case Study-2
SourceType-Scholarly Journals-1
ObjectType-Review-3
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ObjectType-Report-1
ISSN:2573-8348
2573-8348
DOI:10.1002/cnr2.1512