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.
Subject(s)
Kidney Neoplasms , Mulibrey Nanism , Wilms Tumor , Child , Child, Preschool , Humans , Kidney Neoplasms/diagnosis , Kidney Neoplasms/therapy , Male , Mulibrey Nanism/complications , Mulibrey Nanism/genetics , Mulibrey Nanism/pathology , Nuclear Proteins/genetics , Tripartite Motif Proteins , Ubiquitin-Protein Ligases , Wilms Tumor/complications , Wilms Tumor/diagnosis , Wilms Tumor/therapyABSTRACT
Abstract Background and aims Multidisciplinary team (MDT) meetings provide a regular, structured meeting of a core group of professionals to provide expert decision-making in individual patient care. We describe our experiences in establishing a virtual pediatric oncology MDT between twinning partners in Cameroon (4 hospitals) and the UK. Methods Monthly video conferencing MDT meetings were established in March 2019, initially using Google meet then using Zoom platforms. Case details were circulated using formal proformas. Standard operating procedures were outlined for the MDT conduct. Results There have been 269 discussions of 201 patients in 21 meetings. Patients have been varied in age (up to 24 years). Eighty-seven (43.3%) patients had NHL, 16 (8.0%) had Wilms tumour, 20 (10.0%) had Acute Lymphoblastic Leukaemia, 19 (9.5%) had Retinoblastoma and 59 (29.4%) had other malignancies. Four of the patients did not have a malignancy. The majority of patients had stage 3 (51, 45.1%) or stage 4 (59, 52.2%). Nine patients had relapsed disease. Core team members reported positive benefits from the MDT, including defining goals of care, shared decision making, professional education and team-building. Conclusions Virtual MDT meetings between geographically dispersed teams are possible and have proved particularly valuable during the COVID-19 pandemic when in-person visits are not permitted. Cancer treatment cannot be done in isolation; the regular MDT meetings have paved the way for informed care through regular consultancy.
Subject(s)
Precursor Cell Lymphoblastic Leukemia-Lymphoma , Wilms Tumor , Neoplasms , COVID-19 , RetinoblastomaABSTRACT
Since December 2019, severe acute respiratory syndrome coronavirus 2 infection has spread worldwide. We all are concerned about immunocompromised children, especially hematologic and oncologic pediatric patients. We want to share our experience with 2 pediatric cancer patients with severe acute respiratory syndrome coronavirus 2 infection. Both presented mild disease and good outcome. No respiratory symptoms were identified, but both developed diarrhea, one probably secondary to lopinavir/ritonavir. Pediatric cancer patients may have milder disease than adults, but larger studies are needed to make conclusions.