J Lung Cancer.  2009 Dec;8(2):103-110. 10.6058/jlc.2009.8.2.103.

Primary Neoplasms of the Lung in Children and Adolescents: 22 Cases from a Single Institute

• An J
• Han J
• Ahn KM
• Kim J
• Choi YS

• KMID: 2199970
• DOI: http://doi.org/10.6058/jlc.2009.8.2.103

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.

Keyword

Lung; Neoplasms; Childhood; Adolescent

MeSH Terms

Adenocarcinoma
Adolescent
Adult
Carcinoid Tumor
Carcinoma, Mucoepidermoid
Child
Female
Follow-Up Studies
Hamartoma
Histiocytosis
Humans
Incidence
Leiomyoma
Lung
Lymphoma
Male
Myofibroblasts
Neurilemmoma
Prognosis
Pulmonary Blastoma
Recurrence
Retrospective Studies
Sarcoma, Alveolar Soft Part
Pulmonary Blastoma

Figure

• 

  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).

Reference

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