Benign spindle cell tumor surrounding the bronchovascular st
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Neurofibromatosis type I (NF1) is an autosomal dominant disease with variable clinical manifestations that involve the dermatologic, neurologic, skeletal, and endocrine systems.

In this article, here presented a case of a spindle cell tumors encompassing the bronchovascular structures in a 14‐year‐old male with NF1. A 14‐year‐old male was referred to our hospital for an abnormal chest radiograph performed during a routine examination. He had no respiratory symptoms. Physical examination revealed more than six café‐au‐lait macules larger than 1.5 cm, and frecklings in the axillary and inguinal regions with four Lisch nodules on ophthalmologic examination. There was no family history of NF1. Results of arterial blood gas analysis were normal. NF1 whole gene deletion was detected on the genetic testing using a multiplex ligation‐dependent probe amplification.

The pulmonary function test showed minimal airway obstruction based on the decrease in flow rate at peak flow, and flow at 50% and 75% of the flow-volume curve, with forced vital capacity (FVC) of 90%, forced expiratory volume in 1 second (FEV1) of 80%, and FEV1/FVC ratio of 82%. Chest radiography and chest computed tomography (CT) showed nodular lesions surrounding the peri bronchovascular areas of the right upper lung fields, interstitial thickening, and paratracheal adenopathy (Figure 1A‐F). Pathologic examination of the transbronchial biopsy performed in the anterior segment of the right upper lobe revealed a benign spindle cell tumor, suggesting neurogenic origin (Figure 1G). The patient has been undergoing regular follow‐up to detect changes in the size of the chest mass, development of respiratory symptoms associated with the chest mass, and/or changes in pulmonary function.

Intrathoracic tumors reported in children include angiosarcoma differentiated from malignant peripheral nerve sheath tumor and malignant peripheral nerve sheath tumor in intrathoracic spine. Neurofibromas in the thorax, including the mediastinum, can transform into malignant tumors. Hence, some doctors recommend surgical resection of the intrathoracic neurogenic tumors, even if the tumor is benign. The patient has been under the close follow‐up to monitor any increase in tumor size since there were no changes in size in the preceding 2 years.

Source: https://onlinelibrary.wiley.com/doi/abs/10.1002/ppul.24769?af=R

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