Background and Aims

Chest wall tumours are rare in children and there is varied literature concerning the management and outcomes of these heterogenous lesions. This current study report highlights the accumulating experience of surgical management with particular focus on those requiring chest wall resection and body wall reconstruction.

Methods

All index patients (<18yrs) with chest wall tumours attending a single UK CCLG oncology center during 2001 - 2021 were identified using electronic patient records (EPR) and a pathology database registry. Data were analysed and reported with regard demographics, radiology investigative studies, operative surgery and multidisciplinary oncology management.

Results

Over a 20 year period, 28 pediatric patients with chest wall tumours were identified, aged 4 days - 18 years (13M : 15F). A variety of pathological lesions were confirmed histologically with primitive neuro-ectodermal tumour (PNET) being the most frequent neoplasm (25%). Other chest wall diagnoses included lipoblastomas, lymphangiomas, sarcomas, hamartomas amongst other diagnoses which were rare. Presenting clinical features included a palpable lump (74%) and chest pain (13%). Ten patients (36%) required rib resection(s), four (14%) of these cases subsequently requiring artificial prosthetic patch insertion for chest wall reconstruction. A single patient had a latissimus dorsi muscle flap with 12 (43%) cases also receiving adjuvant multimodal chemotherapy / radiotherapy . Surgical approaches were tailored to each individual patient depending on the size and anatomical location of the lesion. The most common morbidity(s) documented were wound seromas (11%) in the short term and scoliosis (7%) on longer term follow up.

Conclusions

Whilst the pathology of chest wall tumours are varied, a significant number of these lesions are malignant and require complete surgical resection (R0) where possible. Chest wall reconstruction following tumour excision remains a challenge particularly in those patients where rib resection(s) are required and an individualised approach is thus highly recommended.

Background and Aims

To describe the clinical characteristics of children and adolescents with ES / PNET in the chest wall, and to evaluate the result of multidisciplinary management, and its relationship with the t (11,22) (q24q12) translocation.

Methods

Patients with ES / PNET treated at the Federico Gómez Children's Hospital (HIMFG in Mexico City, Mexico) and Children´s Hospital Dr Renan Esquivel (in Panama City, Panama) from 01/01/2001 to 04/30/2017.

Review of medical records, molecular biology studies (RT-PCR determination of chromosomal translocation EWS-FLI and EWS –ERG) on tissue samples previously obtained for pathological diagnosis.

Results

Thirteen patients were analyzed. There were 8 girls and 5 boys; the mean age was 13 ± 3.19 years. Location of the tumor: rib cage 5, scapula 5, and thoracic vertebrae 3. Pulmonary metastases were present at diagnosis in 8 cases. The tumor size ranged from 3 cm to 20 cm. The operations included 4 scapulectomies, 5 thoracotomies with chest wall resections, 1 clamshell thoracotomy, 2 paravertebral resections, and 1 laminectomy were performed. EWS-FLI 1 was identified in 10 patients, and EWS –ERG in 1. Autologous stem hematopoietic cell rescue was used in 2 cases. The overall survival rate was 60% after 2 years of diagnosis.

Conclusions

The absence of metastasis at diagnosis gave 80% survival at 2 years of diagnosis. The presence of EWS-FLI or EWS –ERG was possible to determine by RT-PCR from two different centers. Further multicenter research is necessary for the identification of prognostic factors and to treat accordingly.