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Journal List > J Lung Cancer > v.8(2) > 1050699

An, Han, Ahn, Kim, and Choi: Primary Neoplasms of the Lung in Children and Adolescents: 22 Cases from a Single Institute

Abstract

Purpose

Primary lung tumors in children and adolescents are uncommon. We report here on the clinical presentations, the methods of treatment, the results and the outcomes of a series of children with these rare tumors.

Materials and Methods

We conducted a retrospective review on all the patients less than 19 years of age with primary lung tumors and who were treated at Samsung Medical Center between 1995 and 2009. Twenty two cases of primary lung tumors were reviewed and the clinical-pathological information concerning the tumor characteristics, the therapy and the follow-up was collected.

Results

The average age of the 10 male patients and 12 female patients was 11.5 years (range: 3 months to 18 years). Of the 22 primary lung tumors, 18.2% (4/22) were benign, 36.4% (8/22) were of borderline malignancy and 45.4% (10/22) were malignant. The pathologic types were inflammatory myofibroblastic tumor (6/22), mucoepidermoid carcinoma (4), pleuropulmonary blastoma (2), adenocarcinoma (1), alveolar soft part sarcoma (1), malignant lymphoma (1), leiomyoma (1), pulmonary leiomyomatous hamartoma (1), congenital pulmonary myofibroblastic tumor (1), Langerhans’ cell histiocytosis (1), neurilemmoma (1), sclerosing pneumocytoma (so-called sclerosing hemangioma) (1) and atypical carcinoid tumor (1). After surgery for the benign tumors, no postoperative recurrence was been observed during long-term follow-up. All the patients with the benign tumors were alive with no evidence of disease. For the patients with the tumors of borderline malignancy, one patient experienced recurrence, but there was no death from disease. For the group with malignant tumors, two patients died from untreatable metastatic disease and 8 patients survived their disease on the long-term follow-up.

Conclusion

The primary lung tumors during childhood and adolescence had different characteristics compared with their adult counterparts with respect to the incidence and prognosis. These facts must be considered when making the diagnosis and deciding on proper therapy.

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References

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Figures and Tables

jlc-8-103f1.tif
Fig. 1.
Primary lung tumors of children and adolescents. (A, B) An inflammatory myofibroblastic tumor of a 12-year-old girl (patient 22). The segmentectomy specimen shows a well-demarcated solid mass at the periphery. It consists of myofibroblastic spindle cells with infiltrative plasma cells, lymphocytes and eosinophils. (C, D) A low grade mucoepidermoid carcinoma of a 6-year-old girl (patient 11). The mass occludes the right main bronchus. The tumor reveals a predominant cystic pattern of intermediate cells and occasional mucous cells with minimal cellular pleomorphism. (E, F) A pleuropulmonary blastoma of a 14-year-old boy (patient 8). The CT scan reveals a huge mass in the right mid-hemithorax. The mass is composed of solid sheets of primitive anaplastic cells that exhibits severe atypia and multinucleation without an epithelial component. (G, H) An atypical carcinoid tumor of a 16-year-old girl (patient 20). Bronchoscopy reveals an obstructive endobronchial mass in the right bronchus. It shows ribbon-like or trabecular nests of monomorphic cells with rare mitosis (1/10 high powered fields).
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Table 1.
Primary Lung Neoplasms in Childhood and Adolescence: Samsung Medical Center, 1994∼2009
Patient No. Age at diagnosis Sex Pathologic diagnosis Location Diagnostic workup Surgical procedure Treatment Follow-up (month)/ Outcome Multiple lesions or lymph node metastasis
1 7 F Pleuropulmonary blastoma Left lower lobe CT, frozen diagnosis VAT's wedge resection Combined chemo- and radiotherapy after surgery 160/NED  
2 9 M Mucoepidermoid carcinoma, intermediate grade Trachea CT, bronchoscopic biopsy Tracheal segmentectomy Surgery only 115/NED  
3 13 M Adenocarcinoma, poorly differentiated Left upper lobe CT, bronchoscopic biopsy Lobectomy Combined chemo- and radiotherapy after surgery 16/DOD Lymph node metastasis
4 18 F Alveolar soft part sarcoma Both lung fields CT, frozen diagnosis VAT's wedge resection No further treatment 35/DOD Lung to lung metastasis
5 14 M Inflammatory myofibroblastic tumor Left bronchus CT, bronchoscopic biopsy Bronchial segmentectomy Surgery only 64/NED  
6 18 M Inflammatory myofibroblastic tumor Trachea CT, bronchoscopic biopsy Tracheal segmentectomy Surgery only 38/NED  
7 11 F Mucoepidermoid carcinoma, low grade Right bronchus CT, bronchoscopic biopsy Lobectomy Chemotherapy after surgery 78/NED  
8 14 M Pleuropulmonary blastoma Right middle lobe CT, Gun biopsy Lobectomy and chest wall excision Neoadjuvant chemotherapy after surgery 74/NED  
9 9 M Posttransplant lymphoproliferative disorder Both lung fields CT, frozen diagnosis VAT's wedge resection Chemotherapy 69/NED Multiple lesions in liver and adrenal gland
10 17 M Leiomyoma Right bronchus CT, bronchoscopic biopsy Bronchial removal Surgery only 40/NED    
11 6 F Mucoepidermoid carcinoma, low grade Right bronchus CT, frozen diagnosis Lobectomy Surgery only 35/NED  
12 13 F Pulmonary leiomyomatous hamartoma Left lower lobe CT, frozen diagnosis VAT's wedge resection Surgery only 4/NED  
13 4 M Inflammatory myofibroblastic tumor Left bronchus CT, frozen diagnosis Bronchial segmentectomy Surgery only 31/NED  
14 16 F Inflammatory myofibroblastic tumor Left lower lobe CT, frozen diagnosis Lobectomy Surgery and radiotherapy 19/Recur, 28/NED  
15 0 F congenital pulmonary myofibroblastic tumor Right middle and lower lobes CT, frozen diagnosis Lobectomy Surgery only 24/NED  
16 6 M Langerhans' cell histiocytosis Both lung fields CT, frozen diagnosis VAT's wedge resection Chemotherapy 40/NED Multiple lesions in lung
17 16 M Neurilemmoma Left bronchus CT, bronchoscopic biopsy Lobectomy Surgery only 1/NED  
18 10 F Inflammatory myofibroblastic tumor Right lower lobe CT, frozen diagnosis Lobectomy Surgery only 30/NED  
19 12 F Sclerosing pneumocytoma (so-called sclerosing hemangioma) Left upper lobe CT, frozen diagnosis Lobectomy Surgery only 15/NED  
20 16 F Atypical carcinoid tumor d Right bronchus CT, bronchoscopic biopsy Lobectomy after bronchoscopic removal Surgery only 19/NED  
21 13 F Mucoepidermoid carcinoma, low grade Right bronchus CT, bronchoscopic biopsy Lobectomy after bronchoscopic removal Surgery only 13/NED  
22 12 F Inflammatory myofibroblastic tumor Left upper lobe CT, frozen diagnosis VAT's segmentectomy Surgery only 12/NED  

CT: computed tomography, VAT: video assisted thoracotomy, NED: no evidence of disease, DOD: dead of disease.

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