[Myofibroblastic tumor of the lung: clinical features and results in 9 children]. / Tumor miofibroblástico de pulmón: aspectos clínicos y resultados en 9 niños.

ABSTRACT

INTRODUCTION: Primary lung tumors in children are rare, being the myofibroblastic lung tumor (MLT) reported as the most common. Nevertheless the literature about it is scarce, and mostly consistent of case reports. The aim of this article is to describe the results obtained in a group of patients with this tumor treated in a tertiary pediatric hospital. METHODS: A single institution retrospective chart review was performed with institutional review board approval. All patients treated between 1990 and 2010 with diagnosis of MLT were included. Age, symptoms at presentation, surgical procedures, complications, histopathology and follow-up were reviewed. RESULTS: 9 patients were identified. The commonest symptoms at presentation were fever and cough (n=6), and seizures (n=2). Surgical treatment consisted in complete resection in 8 patients and partial resection in 1. To achieve this 3 patients underwent pneumonectomy and 6 lobectomy. Two of the patients with pneumonectomy required also a partial resection of the right atrium and both had cerebral lesions. The mean follow-up was 2.8 years (r0.16-12) and all the patients that remained in follow-up (66%) are alive at the moment. DISCUSSION: Complete surgical resection in MLT is possible, however, to achieve this complex procedures and a multidisciplinary approach might be needed. Although the potential of distant metastasis is not clearly reported, we think that it is a topic that must be carefully revised.