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Wilms tumor with Mulibrey Nanism: A case report and review of literature.
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.
  • Upasana K; Pediatric Hematology Oncology and Bone Marrow Transplant Unit, Cancer Institute, Medanta The Medicity Hospital, Gurgaon, Haryana, India.
  • Thakkar D; Pediatric Hematology Oncology and Bone Marrow Transplant Unit, Cancer Institute, Medanta The Medicity Hospital, Gurgaon, Haryana, India.
  • Gautam D; Department of Pathology, Medanta The Medicity, Gurgaon, Haryana, India.
  • Sachdev MS; Department of Pediatric Cardiology, Medanta The Medicity, Gurgaon, Haryana, India.
  • Yadav A; Pediatric Hematology Oncology and Bone Marrow Transplant Unit, Cancer Institute, Medanta The Medicity Hospital, Gurgaon, Haryana, India.
  • Kapoor R; Pediatric Hematology Oncology and Bone Marrow Transplant Unit, Cancer Institute, Medanta The Medicity Hospital, Gurgaon, Haryana, India.
  • Raghunathan V; Pediatric Intensive Care Unit, Department of Pediatrics, Medanta The Medicity, Gurgaon, Haryana, India.
  • Dhaliwal MS; Pediatric Intensive Care Unit, Department of Pediatrics, Medanta The Medicity, Gurgaon, Haryana, India.
  • Bhargava K; Department of Electrophysiology and Pacing, Heart Institute, Medanta The Medicity, Gurgaon, Haryana, India.
  • Nair S; MedGenome Labs Ltd., Bangalore, Karnataka, India.
  • Sharma J; Division of Radiology, Medanta The Medicity, Gurgaon, Haryana, India.
  • Rastogi N; Pediatric Hematology Oncology and Bone Marrow Transplant Unit, Cancer Institute, Medanta The Medicity Hospital, Gurgaon, Haryana, India.
  • Yadav SP; Pediatric Hematology Oncology and Bone Marrow Transplant Unit, Cancer Institute, Medanta The Medicity Hospital, Gurgaon, Haryana, India.
Cancer Rep (Hoboken) ; 5(5): e1512, 2022 05.
Article in English | MEDLINE | ID: covidwho-1913776
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.
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Full text: Available Collection: International databases Database: MEDLINE Main subject: Wilms Tumor / Mulibrey Nanism / Kidney Neoplasms Type of study: Case report / Cohort study / Diagnostic study / Observational study / Prognostic study Topics: Long Covid Limits: Child / Child, preschool / Humans / Male Language: English Journal: Cancer Rep (Hoboken) Year: 2022 Document Type: Article Affiliation country: Cnr2.1512

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Full text: Available Collection: International databases Database: MEDLINE Main subject: Wilms Tumor / Mulibrey Nanism / Kidney Neoplasms Type of study: Case report / Cohort study / Diagnostic study / Observational study / Prognostic study Topics: Long Covid Limits: Child / Child, preschool / Humans / Male Language: English Journal: Cancer Rep (Hoboken) Year: 2022 Document Type: Article Affiliation country: Cnr2.1512