Rare airway tumor in a previously healthy adolescent successfully treated with endoscopic resection and oral propranolol

Abstract

Airway tumors are rare in children. Pyogenic granuloma (PG), also known as lobular capillary hemangioma, is a benign vascular tumor usually found on the skin or in the oral cavity. Rarely, these lesions occur in the airway and cause significant hemoptysis. Most reported airway PGs have occurred in the trachea of adults. Here, we present a case of an adolescent female who presented with hemoptysis and was found to have a PG in the right lower lobe. Per institutional guidelines, this case report was exempt from institutional review board approval.

1 |. CASE

A 14-year-old female with a remote history of chronic cough presented to the emergency department with progressively worsening hemoptysis. She had visited her pediatrician and an outside emergency department 1 day before her presentation for similar symptoms. An extensive laboratory evaluation and a chest radiograph were unremarkable, and she was diagnosed with presumed viral bronchitis. The next day, she coughed several “palm-full” volumes of blood and came to our hospital for further evaluation. A computed tomography angiogram of the chest was performed (Figure 1) which revealed an inferior lobar bronchus filling defect and right middle and lower lobe consolidative opacities. Based on the imaging, it was unclear whether there was a mass present in the inferior lobar bronchus with possible vascular enhancement, or simply clotted blood (Figure 1). A flexible bronchoscopy was performed using a 4.2 mm flexible bronchoscope. A bronchial blocker was secured to the distal tip of the bronchoscope and positioned into the bronchus intermedius as a means of limiting the impact of a potential airway bleeding event. A large, organized clot was visualized in the bronchus intermedius. After the removal of the clot, a tumor was seen completely occluding the lateral basal segment of the right lower lobe (Figure 2A). The tumor was approximately 6–8 mm in diameter. The 2 mm endobronchial forceps were used to resect the tumor. Intermittent bleeding was effectively managed using 0.5 mL aliquots of topical oxymetazoline. Most of the tumor was removed (Figure 2B) and there was no bleeding visualized at the conclusion of the bronchoscopy. Histopathology (Figure 2C) revealed polypoid tissue composed of numerous arborizing congested thin-walled vessels in an edematous inflamed background, consistent with pyogenic granuloma (PG). Three days later, the patient experienced hemoptysis and a repeat bronchoscopy was performed which revealed a large clot in the bronchus intermedius that was removed using suction and endobronchial forceps. There was a small regrowth of the previously identified PG. Four 1 mL aliquots of oxymetazoline were sprayed onto the tumor surface and it was again resected using endobronchial forceps. Then, electrocautery was used to ablate the bronchial mucosa at the site of the debulked tumor. At the conclusion of the procedure, there was no further bleeding. Propranolol (0.5 mg/kg) was prescribed, and she was discharged from the hospital after 72 h of monitoring with no hemoptysis. Five days later, she returned to the emergency department after coughing up small dark clots. A third bronchoscopy was performed and there was no evidence of regrowth of the endobronchial tumor. Scant bloody secretions and several clots were identified throughout the right bronchi, which were removed using suction. She was then discharged home and completed a 4-week course of propranolol. As of 5 months after her initial hospitalization, she has had no recurrence of hemoptysis and a repeat computed tomography (CT) scan of the chest was normal.

FIGURE 1.

Computed tomography angiogram of the chest demonstrating a right inferior lobar filling defect (top image), and possible mass with questionable contrast enhancement (bottom image, yellow arrow).

FIGURE 2.

Bronchoscopy images (A) before and (B) after endobronchial tumor resection. (C) Pathology image (×54 magnification) of biopsy specimen showing polypoid tissue composed of numerous arborizing congested thin-walled vessels in an edematous inflamed background.

2 |. DISCUSSION

PG is an acquired benign vascular lesion that occurs on the skin and mucus membranes and appears as a pedunculated red papule that often bleeds easily. The name PG is a misnomer, as the lesion is not infectious; lobular capillary hemangioma is a more accurate term that is based on the tumor’s histopathology.¹ The underlying pathophysiology driving the development of PGs is unknown, although it is thought to be acquired. Some, but not all, patients have a history of local trauma, infection, or inflammation. Our patient had a remote history of chronic productive cough during early childhood, which may have represented recurrent bacterial bronchitis that resulted in the formation of the PG. Hormonal changes are thought to play a role, supported by the fact that PGs commonly occur in pregnant women.² Histopathology shows a polypoid mass with aggregates of thin-walled blood vessels in a background of inflamed granulomatous tissue.¹

Pediatric airway tumors are rare, but should be considered in the differential diagnosis of a child presenting with hemoptysis.³ To our knowledge, this is the most distal airway PG reported, and the youngest reported case of an airway PG in general. Qiu et al. summarized 12 airway PG cases reported in the literature. Eleven were found in the trachea, and one occurred in the bronchus intermedius. Most cases occurred in adults, apart from three adolescents (ages 15, 17, and 17 years). Tumor size ranged from 0.2 to 2.0 cm, and treatment modalities used included forceps excision, cryoexcision, laser excision, argon plasma coagulation, electrocautery, and brachytherapy. The prognosis was generally good, although tumor recurrence was observed in some, highlighting the need for continued observation.²

We successfully used endobronchial forceps to excise the PG and employed a combination of topical oxymetazoline and electrocautery to minimize airway bleeding. Given the experience of our pulmonary flexible bronchoscopy team managing airway tumors, we felt comfortable performing this resection ourselves. In other institutions, requisite expertise to resect airway tumors may lie within other specialties such as otolaryngology or pediatric surgery. In any case, preoperative planning and close collaboration with the anesthesiologist are of utmost importance in preparing for possible intraoperative complications such as bleeding when resecting airway tumors. Although the mass itself and therefore its vascularity was not visualized on the CT scan (likely because of the degree of surrounding blood clot), we knew that there would be a significant bleeding risk with resection. Therefore, we sprayed the mass with oxymetazoline to alleviate this risk. Postoperatively, we treated the patient with systemic propranolol (0.5 mg/kg) for 4 weeks. Propranolol is a well-established therapy to treat infantile hemangiomas, and although its exact mechanism in treating hemangiomas is unknown, possibilities include induction of vasoconstriction, inhibition of angiogenesis, and induction of apoptosis in endothelial cells.⁴ Several case reports describe the use of topical and/or oral beta blockers in the treatment of nonairway PGs.⁵ Given the underlying vascular histopathology and tumor regrowth, we elected to try this therapy on our patient and we suspect that it may have played a role in preventing further tumor regrowth.

3 |. CONCLUSION

Airway tumors should be considered in the differential diagnosis of a child with hemoptysis. PG is a benign vascular tumor that occurs commonly on the skin but is extremely rare in the airways, especially in children. There have been no prospective studies evaluating treatment, although several endoscopic techniques are described in case reports. Propranolol is a generally safe medication that is effective in treating infantile hemangiomas and may be worth considering to help prevent recurrence in high-risk PGs treated with surgical modalities.

DATA AVAILABILITY STATEMENT

The data that support the findings of this study are available from the corresponding author upon reasonable request.