Clinical prognostic factors for pediatric extra-abdominal desmoid tumor: analyses of 66 patients at a single institution

Ning, Bo; Na, Jian; Ma, Rong

doi:10.1186/s12957-018-1536-x

Cited by 5 publications

(2 citation statements)

References 34 publications

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“…There are also sporadic mutations related to DT ( 2 ). In the paediatric population, DT is associated with more gene mutations and a higher gene mutation rate than in adult cases, described in the literature the mutation rate of three exons of CTTNNB1 ( 3 ). DT can be related to local trauma, and surgery is associated with 28% of DT cases ( 4 ).…”

Section: Discussionmentioning

confidence: 99%

Desmoid tumour of the chest wall in paediatric post-operatory of heart transplant

Filho

Nakahira²,

Schmidt³

et al. 2022

Front. Pediatr.

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We will report a case of a desmoid tumour (DT), which developed at the surgical site of the pacemaker after a late childhood heart transplant. Patients with idiopathic dilated cardiomyopathy followed up in the paediatric cardiology service. It evolved with the dissociation of ventricular rhythm caused by severe heart failure, which led to the implantation of a cardiac resynchronization device prior to heart transplantation. The progression to end-stage heart disease culminated in a heart transplant at 12 years old. One year after the transplant, at the age of 13 years, he presented a progressively growing mass on the generator site of the resynchronization device. The initial decision was to remove the device. During the removal surgery, there was no haematoma or fluid collection. However, there was a progression of the lesion. The lesion was biopsied with the anatomopathological diagnosis of a DT. Resection surgery happened 4 months after the start of the mass growth. At that time, the tumour reached 20 cm in diameter. The lesion infiltrated the pectoralis major muscle and this muscle was resected partially en bloc with the lesion. The defect had primary closure. The patient evolved without postoperative complications and was discharged on the 14th postoperative day. The surgical specimen came with negative circumferential margins. However, the deep margin was microscopically positive. Due to deep involvement, the patient underwent adjuvant radiotherapy. Currently, the patient is under clinical follow-up and has no evidence of tumour recurrence. DT is a rare tumour, with unpredictable courses. Surgery can be considered in the progression of lesions. Treatment is justified by long survival after a heart transplant and in DT patients. DT is a differential diagnosis to be considered in progressive growth lesions.

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Section: Discussionmentioning

confidence: 99%

Desmoid tumour of the chest wall in paediatric post-operatory of heart transplant

Filho

Nakahira²,

Schmidt³

et al. 2022

Front. Pediatr.

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“…Thirty-six (55%) patients had local recurrence with a median follow-up of 6.6 years. Radiotherapy decreased the local recurrence rate and should be considered [ 7 ]. Since surgery is less frequently undertaken for DTs in the latest guideline, the level of evidence for adjuvant radiotherapy is low, except for patients with difficult treatment recurrent disease.…”

Section: Introductionmentioning

confidence: 99%

Clinical Prognostic Factors and Integrated Multi-Omics Studies Identify Potential Novel Therapeutic Targets for Pediatric Desmoid Tumor

Ning

Huang

Zhu³

et al. 2022

Biol Proced Online

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Background Desmoid tumor (DT), also known as desmoid-type fibromatosis (DTF) or aggressive fibromatosis (AF) is a rare mesenchymal tumor affecting both children and adults. It is non-metastasis but infiltrative, growing with a high recurrence rate to even cause serious health problems. This study investigates the biology of desmoid tumors through integrated multi-omics studies. Methods We systematically investigated the clinical data of 98 extra-abdominal cases in our pediatric institute and identified some critical clinical prognostic factors. Moreover, our integrated multi-omics studies (Whole Exome Sequencing, RNA sequencing, and untargeted metabolomics profiling) in the paired PDT tumor/matched normal tissues identified more novel mutations, and potential prognostic markers and therapeutic targets for PDTs. Results The top mutation genes, such as CTNNB1 (p.T41A and p.S45F) and MUC4 (p.T3775T, p.S3450S, etc.), were observed with a mutation in more than 40% of PDT patients. We also identified a panel of genes that are classed as the FDA-approved drug targets or Wnt/β-catenin signaling pathway-related genes. The integrated analysis identified pathways and key genes/metabolites that may be important for developing potential treatment of PDTs. We also successfully established six primary PDT cell lines for future studies. Conclusions These studies may promote the development of novel drugs and therapeutic strategies for PDTs.

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Musculoskeletal Disorders

Mak

Wong

2023

Medical Radiology

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Clinical prognostic factors for pediatric extra-abdominal desmoid tumor: analyses of 66 patients at a single institution

Cited by 5 publications

References 34 publications

Desmoid tumour of the chest wall in paediatric post-operatory of heart transplant

Desmoid tumour of the chest wall in paediatric post-operatory of heart transplant

Clinical Prognostic Factors and Integrated Multi-Omics Studies Identify Potential Novel Therapeutic Targets for Pediatric Desmoid Tumor

Musculoskeletal Disorders

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