Juvenile Bronchopulmonary Fibrosarcoma.

Primary bronchopulmonary fibrosarcoma is the second most common intrathoracic sarcoma after leiomyosarcoma.1

Because of the limited number of cases, there are only a few studies that describe the management of these tumors. Here, we present a case of juvenile bronchial fibrosarcoma that developed asynchronously at 2 separate sites in the right main bronchus (RMB). We discussed the benefits of rigid bronchoscopy and surgery toward the management of these tumors with a review of the literature.

A 13-year-old girl was admitted to our hospital in December 2013 with complaints of cough, hemoptysis, and dyspnea and SpO2 of 60%. On physical examination, the respiratory rate was 46/minute and the right chest was found to be silent on auscultation. The chest radiograph revealed complete opacification of the right lung. A computed tomography of the chest with contrast revealed a soft tissue lesion completely occluding the RMB.

A rigid bronchoscopy confirmed the presence of the lesion arising from the right upper lobe bronchus completely occluded the RMB. The distal RMB was fully patent. The endobronchial portion of lesion was removed using an endobronchial electrocautry. An immunohistochemical staining of the biopsy samples showed the presence of a juvenile bronchopulmonary fibrosarcoma. The mitotic index was >10 per 10 high-power fields. A symptomatic treatment was offered at this stage.

Three weeks later the patient developed hemoptysis and dyspnea. A repeat computed tomography of chest showed the tumor now infiltrating the right lower lobe bronchus (Fig. 1). A repeat bronchoscopy revealed regrowth of the tumor yet, this time originating from the right lower lobe bronchus while the right upper lobe bronchus was patent. Luminal patency was reestablished using endobronchial electrocautry.

FIGURE 1

Fibrosarcoma infiltrates the segmental airway of the right lower lobe. Arrow shows the tumor infiltrating the segmental airways of the right lower lobe bronchus.

Eventually the patient underwent successful pneumonectomy with médiastinal lymphadenectomy. The pneumonectomy specimen confirmed the 2 separate origins of the tumor (Fig. 2).

FIGURE 2

Pneumonectomy specimen shows the infiltration of the segmental airway of the RLLB. Arrow shows the macroscopic infiltration of the RLLB. RLLB indicates right lower lobe bronchus.

The juvenile bronchopulmonary fibrosarcoma is a rare tumor of the airways with <100 cases reported.2 This case is of interest as the tumors developed asynchronously at 2 separate locations. The site of origin could have been either the endobronchial tree or the lung parenchymal.3 This rare malignant tumor occurs in the large airways of younger individuals but tends to localize in the parenchyma in older individuals.3 Endobronchial fibrosarcoma has been shown to have a better diagnosis than inraparenchymal fibrosarcoma.3

We used rigid bronchoscopy accompanied by high-frequency electrocautery to resect the tumors. The combination of rigid bronchoscopy and electrocautery or laser is useful in establishing airway patency from the lesion. Kunst and colleagues have used rigid bronchoscopy with electrocautery to completely remove a tumor from the right mainstem bronchus that originated from the right upper lobe carina. The patient had no recurrence or metastasis and remained free of the disease for over 5 years.4

Surgery remains a mainstay treatment for fibrosarcoma. In our case, although we achieved a complete endobronchial resection of the tumor with rigid bronchoscopy and electrocautery, the appearance of tumor at a second location led us to use surgery as a curative option. Mitotic index is useful in deciding for surgical treatment and is of prognostic significance. Besides, it has been showed that tumors with 8 mitotic figures per 10 high-power fields behave aggressively.5 Our patient had a much higher mitotic index which also helped us choose the appropriate treatment modality. Our patient continues to do well without any evidence of recurrence.