|Year : 2023 | Volume
| Issue : 1 | Page : 18-24
Thoracic tumors in children and their long-term outcomes: A single-center experience
Neehar Patil1, Padmalatha S Kadamba1, Manjiri Somashekhar1, Jeevak Shetty1, Somashekar A Rama2
1 Department of Paediatric Surgery, Ramaiah Medical College and Hospital, Bengaluru, Karnataka, India
2 Department of Paediatrics, Ramaiah Medical College and Hospital, Bengaluru, Karnataka, India
|Date of Submission||01-Jun-2022|
|Date of Decision||25-Aug-2022|
|Date of Acceptance||08-Oct-2022|
|Date of Web Publication||10-Jan-2023|
Padmalatha S Kadamba
Department of Paediatric Surgery, Ramaiah Medical College and Hospital, Bengaluru, Karnataka
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Aims: This study aims to review our experience in children with thoracic tumors managed by different surgical approaches, and to evaluate their long-term outcomes in relation to their functional status and quality of life. Subjects and Methods: This is a retrospective study (2011–2021). Children <18 years with tumors of the thorax (lung, mediastinum, and thoracic cage) were included. All included were diagnosed, managed, and followed up based on a departmental protocol. Children alive were followed up annually to monitor the development of chest wall/spinal deformities and assessed regarding their quality of life (Lansky play-performance scale) and pulmonary functions. Information regarding their demography, clinical presentations, diagnosis, treatment administered, outcome, and follow-up details were collated and analyzed. Results: Twenty-two children with thoracic tumors were included (2011–2021). Of which, 6/22 are benign and 16/22 are malignant lesions. About 14/22 children are alive on a regular follow-up until 2021, with a mean follow-up of 6 years (benign) and 6.25 years (malignant). About 3/22 children with malignant tumors requiring thoracotomy with rib resections developed scoliosis with a severely restrictive pattern on pulmonary functions, having a mild-to-moderate restriction of play on quality of life grading. Conclusions: Early follow-up of children who have undergone various surgical approaches for thoracic tumors based on the quality of life assessment and pulmonary function tests helps in planning early intervention if needed, especially in those who have undergone thoracotomy with rib resections, thereby improving their long-term functional status.
Keywords: Children, Lansky play performance scale, pulmonary function test, thoracic tumors
|How to cite this article:|
Patil N, Kadamba PS, Somashekhar M, Shetty J, Rama SA. Thoracic tumors in children and their long-term outcomes: A single-center experience. J Indian Assoc Pediatr Surg 2023;28:18-24
|How to cite this URL:|
Patil N, Kadamba PS, Somashekhar M, Shetty J, Rama SA. Thoracic tumors in children and their long-term outcomes: A single-center experience. J Indian Assoc Pediatr Surg [serial online] 2023 [cited 2023 Feb 2];28:18-24. Available from: https://www.jiaps.com/text.asp?2023/28/1/18/367390
| Introduction|| |
A wide spectrum of tumors of the thorax may originate from the lungs, mediastinum, and thoracic cage. Sound knowledge of their anatomical site with their clinical and radiographical presentations, helps us to infer a differential diagnosis and formulate guidelines for their surgical management. There are lesser-known facts in the published literature regarding the impact of various surgical approaches on thoracic tumors in relation to their quality of life and lung functions. These facts tend to be important, especially in children undergoing tumor resections involving rib resections which have an impact on the growing skeleton, thus resulting in deformities. These deformities might have consequences on their pulmonary functional status in turn affecting their quality of life which is a growing concern among the increasing numbers of long-term survivors of childhood thoracic tumors in recent years.
We have aimed to review our experience in different surgical approaches (thoracoscopic, thoracotomy without rib resections, and thoracotomy with rib resections) in the management of children with thoracic tumors and to evaluate the long-term outcomes of these approaches.
| Subjects and Methods|| |
This is a single-center, retrospective study conducted in the department of pediatric surgery, at a tertiary care hospital over 10 years (2011–2021). All children <18 years of age who were diagnosed with thoracic tumors based on the departmental protocol as depicted in [Figure 1] were included. Of these, tumors of the lung, mediastinum, and thoracic cage (ribs, intercostal muscles, nerves, and scapula) were included and those with cardiac tumors were excluded.
|Figure 1: Departmental protocol for the diagnosis and management of children with suspected thoracic lesions|
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All children with a clinical suspicion of a thoracic lesion underwent a systematic clinical and radiological assessment to identify the exact anatomical location, the extent of the tumor, and the presence of metastases. [Figure 2] depicts the tumors diagnosed at different anatomical locations of the thorax. The treatment of the thoracic tumors depended on the nature and the extent of the tumor, i.e., biopsy followed by adjuvant therapy or upfront surgical resections. The surgical approaches followed were either thoracoscopic surgeries, thoracotomy without rib resections, or thoracotomy with rib resections. The case files were reviewed and information regarding their demography, clinical presentations, diagnosis, treatment administered, and outcomes were collated and analyzed.
|Figure 2: Contrast enhanced computed tomography images depicting malignant tumours at various anatomical locations (a) Encircled area depicting a pleuropulmonary blastoma of the left lung, arrow showing the area of necrosis. (b) Encircled area depicting a neuroblastoma of the posterior mediastinum with destruction of the vertebrae. (c) Encircled area depicting a right chest wall Ewing's sarcoma, infiltrating the adjacent lung|
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All children were followed up annually, and the last follow-up details (2021) of those who were alive were collated and analyzed. They were assessed based on their general well-being and quality of life using the Lansky play-performance scale which was graded as the normal range of play (80–100), mild-to-moderate restriction of play (50–70), and moderate-to-severe restriction of play (0–40). These children were graded individually based on a questionnaire filled out by the parent or the child themselves. Their functional status was assessed by documenting any skeletal deformities (spine/chest wall) and by an objective evaluation of their lung capacity using pulmonary function tests which were performed by a pediatric pulmonologist. The pulmonary functions were graded as per the American Thoracic Society Grades for the severity of a pulmonary function test abnormality, based on the forced expiratory volume in 1 s, i.e., >70: normal pattern, 35–69: mild-to-moderate restrictive pattern, and <35 severely restrictive patterns. Based on this pulmonary function evaluation and the severity of the deformity, children requiring skeletal deformity corrections were managed by a specialized pediatric orthopedic team to improve their functional status and their overall survival.
This study was approved by the institutional ethics committee, and informed verbal as well as written consent had been obtained from the parents of the children included in this study.
| Results|| |
There were 22 children who underwent thoracic surgeries for various thoracic tumors satisfying our inclusion criteria. Among these, six were diagnosed with benign tumors and 16 were diagnosed with malignant tumors. [Figure 3] depicts the thoracic tumors encountered by us during the 10 years. The mean age at presentation among benign and malignant tumors was 5 years (2–12 years) and 6.5 years (1 month–13 years), respectively. Two children with malignancies had antenatally detected lesions, i.e., one child who was operated on for a congenital cystic adenomatoid malformation in infancy later developed a pleuropulmonary blastoma and another child had a mediastinal lesion, i.e., neuroblastoma IV-S.
|Figure 3: Distribution of benign and malignant tumours amongst all children with thoracic tumours.|
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All six children with benign lesions underwent primary tumor excisions, i.e., thoracoscopic resections – three children and thoracotomy with tumor excision without rib resection – three children. Sixteen children diagnosed with malignant thoracic tumors underwent either a biopsy, neoadjuvant chemotherapy, or an upfront tumor resection. Three children with malignant lung lesions underwent thoracoscopic surgeries. Among the eight children with mediastinal tumors, three underwent thoracoscopic surgery, four underwent thoracotomy and tumor excision without rib resections, and one child underwent thoracotomy and tumor excision with rib resection (for composite pedicled intercostal muscle-rib graft for vertebral collapse). Among five children with chest wall tumors, four children underwent tumor excision by thoracotomy and rib resections and one child underwent tumor excision by thoracotomy and wide local excision without rib resections. The demographic, operative, and follow-up details of individual thoracic tumors, i.e., benign and malignant have been depicted in [Table 1] and [Table 2].
|Table 1: Demographic, operative, and follow-up details of children with benign thoracic tumors|
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|Table 2: Demographic, operative, and follow-up details of children with malignant thoracic tumors|
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All six children with benign lesions are alive and doing well with a mean follow-up of 6 years (3–9 years). Among 16 children with malignant lesions, six have succumbed during their disease, two have been lost to follow-up and the remaining eight children are on regular follow-up with a mean follow-up of 6.25 years (3–9 years). The 14 children (both benign and malignant) who are alive, have been on a regular follow-up until 2021. None of the children who underwent surgeries for benign tumors developed spinal deformities, whereas three children with malignant tumors who are alive and underwent thoracotomy and tumor excision with rib resections developed spinal deformities (scoliosis).
A pulmonary function test was performed at follow-up in all the 14 children alive; eight children showed a normal pattern, three children had a mild-to-moderate restrictive pattern, and three children had a severely restrictive pattern. We noticed a normal pattern among children who underwent thoracoscopic surgeries. Among those who had thoracotomies without rib resections for benign lesions, a normal pattern was seen, whereas a mild-to-moderate restrictive pattern was observed in children who underwent thoracotomies without rib resections for malignant tumors. A severely restrictive pattern was documented in children with spinal deformities which were seen in children who underwent thoracotomies with rib resections. The quality of life grading using the Lansky play-performance scale revealed that 11 children had a normal range of play and three children had a mild-to-moderate restriction of play which corresponded to the children with spinal deformities who depicted a severely restrictive pulmonary function.
| Discussion|| |
The incidence of childhood tumors has shown an increasing trend over the past 25 years and among these, tumors of the thorax account for a small percentage. Thoracic tumors in children appear in different sites of the thorax spanning a wide spectrum from benign-to-malignant tumors, and the surgical approach for their management depends on the anatomical site, extent, and pathology of tumor. There is limited data in the published literature on the long-term follow-up of children who have undergone various surgical approaches for thoracic tumors, to monitor the development of skeletal deformities and to assess the quality of life in relation to their pulmonary functions.,
We documented a 68% of overall male predominance, which could be compared to the studies by Gurney and Bondy. Persistent cough and dyspnea were the most common symptoms which could be attributed to the compressive effect of the lesion, leading to a restrictive lung condition. Two children had antenatally detected lesions, i.e., one child operated for a congenital cystic adenomatoid malformation in infancy and later developed a pleuropulmonary blastoma, and another child had a mediastinal lesion, i.e., neuroblastoma IV-S. The mortality rates among the mediastinal tumors were the least showing that they had a better prognosis, which could be comparable to the study performed by McLatchie and Young which documented a mortality rate of 14%.
Surgical approaches for the excision of tumors in the thorax could involve thoracoscopy, thoracotomy without rib resections, and thoracotomy with rib resections. These individual approaches result in a certain level of morbidity, especially in young children during their growing phase. Scoliosis is a well-known complication following thoracotomies and the severity is related to the extent of rib resections; in addition, irradiation of the thorax is a risk factor for secondary impairment of pulmonary functions restricting the lung capacity., We in our review detected scoliosis in three children who underwent rib resections (one – neuroblastoma who underwent rib resection for or composite pedicled intercostal muscle-rib graft for vertebral collapse, one – rib Ewing's sarcoma, and one – Ewing's sarcoma of the scapula). We documented a normal pattern on the pulmonary function tests among the children who underwent thoracoscopic surgeries. Three children who underwent thoracotomies without rib resections for benign lesions had a normal pattern on the pulmonary function tests, whereas a mild-to-moderate restrictive pattern was observed in three children who similarly underwent thoracotomies without rib resections for malignant tumors. This could be attributed to the pulmonary fibrosis caused secondary to the chemotherapy and radiotherapy administered to children with malignant tumors. The three children who developed scoliosis secondary to the rib resection surgeries developed a severe restrictive pattern on the pulmonary function tests. This documentation of spinal deformities helped us in guiding the parents toward further management for the correction of scoliosis, which would in turn help in improving their pulmonary function.
Children with cancer, often face considerable amounts of restriction in their day-to-day activities which are related to the physical impact of both the disease and the treatment modalities, often necessitating hospitalization or confinement to a bed at home. Recording these changes at follow-up can be useful in evaluating the efficacy of various surgical approaches, prognostication of their effects, and assessment of disease progression. We in our review adopted the Lansky play-performance scale which is commonly used in children based on play. The proposed scale is advantageous in its ease of administration, i.e., nonprofessional people can observe and assess the quality and type of a child's play by filling out a simple questionnaire, not requiring any complicated instrumentation. This scale has helped us in monitoring the quality of life in these children in conjunction with the pulmonary function evaluation, thus helping us to monitor and improve their overall quality of life. Of the 14 children at follow-up, 11 had a normal range of play and three had a mild-to-moderate restriction of play. The three children with mild-to-moderate restriction of play were the children with scoliosis and a severely restrictive pattern on pulmonary function evaluation. [Figure 4] depicts the follow-up images of children who have developed scoliosis with a severely restrictive pulmonary function, and children who have not developed scoliosis with a normal pulmonary function.
|Figure 4: Follow up images of children who underwent various surgeries for thoracic tumours. (a) Child who underwent thoracotomy and excision of tumour with ribs resection, for an Ewing's sarcoma of the thoracic cage shows presence of scoliosis, with a severely restrictive pulmonary function and mild to moderate range of play. (Case 12-Malignant tumours) (b) Child who underwent thoracotomy and excision of a mediastinal neuroblastoma with composite pedicled intercostal muscle-rib graft for vertebral collapse, shows presence of scoliosis, with a severely restrictive pulmonary function and mild to moderate range of play. (Case 8 -Malignant tumours) (c) Child who underwent a thoracotomy for excision of neuroblastoma of the mediastinum without rib resection, no evidence of scoliosis with a mild to moderate restriction in pulmonary function and normal range pf play. (Case 6- Malignant tumours) (d) Child who underwent a Thoracoscopic excision of a benign thymus, no evidence of a scoliosis with normal pulmonary function and normal range of play. (Case 1 – Benign tumours)|
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Although various authors have documented skeletal deformities following rib resection surgeries for chest wall tumors, our study is a comprehensive review of thoracic tumors arising from different anatomic locations with the impact of different surgical approaches on the long-term outcome, especially in the development of skeletal deformities and their effect on pulmonary functions. We acknowledge certain limitations of our study that this is a retrospective study with a limited sample size and it is a heterogeneous cohort. Our data collection is ongoing for a larger sample size with a longer-term follow-up.
| Conclusions|| |
We would like to conclude that early detection of the chest wall or spinal deformities in whom rib resections have been performed and their quality of life assessment with pulmonary function tests at follow-up are mandatory. These assessments help in planning early intervention, if needed, thereby improving their long-term functional status.
- There are lesser-known facts regarding the long-term outcomes of different surgical approaches for the management of thoracic tumors in relation to their quality of life and lung functions
- Quality of life assessment and pulmonary function tests at follow-up helps in planning early intervention if needed, especially in those who have undergone rib resections, thereby improving their long-term functional status.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]
[Table 1], [Table 2]