Endobronchial, Pulmonary and Liver Leiomyomata in a Child with Primary Immune Deficiency

Introduction

Leiomyoma of the lung is a rare benign tumor in children [8]. It is associated with immune suppression after organ transplantation and viral infections such as Eppstein-Barr virus (EBV) and HIV [4]. It is of mesodermal origin and it has been hypothesized that leiomyoma of the lung arises from the smooth muscles of the bronchial wall or artery [2]. Half of these tumors are parenchymal and the rest of the lesions are endobronchial or tracheal [5]. Symptoms include bronchial obstruction and atelectasis, and the lesions can be the cause of recurrent pulmonary infections, but they are otherwise coincidentally found [3] [7]. We present the first case of a child with a primary immune deficiency and EBV-negative serology and endobronchial, pulmonary and liver leiomyomata to emphasize the importance of planning before surgery and the necessity of investigating for associated leiomyomata.

References

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