Primary tracheal meningiomas treated with endoscopy: a case description

Introduction

Ectopic meningioma is a rare subtype of meningioma that develops outside the central nervous system, accounting for approximately 2% of all meningiomas. These tumors predominantly occur in the head and neck regions, including the orbit, skull, paranasal sinuses, nasal cavity, and oropharynx (1). Primary pulmonary meningiomas (PPMs), first described in 1982 (2), remain exceptionally uncommon, with fewer than 70 documented cases in the English-language literature (3,4). Although most reported cases have involved open thoracic surgical resection, we present the first documented case of primary ectopic meningioma arising in the upper trachea, notably managed via bronchoscopic resection.

Case presentation

A 21-year-old male presented with exertional chest tightness persisting for over 1 month, accompanied by intermittent paroxysmal cough. No dizziness, headache, or constitutional symptoms were reported. Past medical history included tonsillectomy but no personal or family history of smoking, tuberculosis, or malignancy. Physical examination revealed the following: a respiratory rate of 22 breaths/min, exertion-dependent inspiratory dyspnea, and audible inspiratory stridor at the pharyngeal region. In diagnostic imaging, contrast-enhanced thoracic computed tomography (CT) indicated a well-circumscribed 1.6 cm × 1.4 cm endoluminal mass in the upper segment of the trachea (Figure 1A,1B), causing significant airway narrowing (>70% lumen reduction). Spirometry was deferred due to compromised respiratory effort.

Figure 1.

Clinical imaging and pathological manifestations of the patient. (A,B) The CT scan revealed an endoluminal mass (see the black arrows) located in the upper segment of the trachea. (C) Bronchoscopy revealed the presence of a neoplasm in the upper trachea, accompanied by a severe narrowing of the lumen (see the black arrow). (D) HE staining indicated spindle-shaped cells organized in whorls under the intact ciliated columnar and squamous epithelium (original magnification 200×). (E-I) The findings for immunohistochemical staining were as follows: (E) EMA (+), (F) Ki-67(+) 1%, (G) PR (+), (H) SSTR2 (+), and (I) vimentin (+) (original magnification 200×). (J) Follow-up bronchoscopy revealed a polypoid granulomatous protrusion (see the black arrow) at the site 2 months after treatment. CT, computed tomography; HE, hematoxylin and eosin.

Interventional treatment was applied as follows: under general anesthesia with laryngeal mask airway (LMA) placement, evaluation with a bronchoscope (BF-1T260; Olympus, Tokyo, Japan) revealed a nearly obstructive pedunculated lesion arising from the 12 o’clock tracheal wall (Figure 1C). The firm, broad-based tumor was sequentially managed using forceps biopsy, resection with an electrosurgical snare (Cook Medical, Bloomington, IN, USA), cryoextraction (Beijing Kooland Technology Co., Ltd., Beijing, China), argon plasma coagulation (APC; ERBE Elektromedizin, Tübingen, Germany) for base ablation, and adjuvant cryotherapy cycles.

Regarding the pathologic findings, the resected specimen (1.6 cm × 1.4 cm × 1.0 cm) demonstrated spindle-shaped cells arranged in whorls beneath intact ciliated columnar and squamous epithelium, focal necrosis with cholesterol clefts, foam cell aggregates, and keratin cysts (Figure 1D). The immunohistochemical profile was as follows: positivity for EMA (diffuse), SSTR2, vimentin, PR (focal), CD34, SAM (focal), and Ki-67 (1%) and negativity for CK, CD5/6, desmin, GFAP, P63, SOX-10, and S-100 (Figure 1E-1I). These features confirmed a World Health Organization (WHO) grade I ectopic meningioma. Notably, abdominal CT and neuraxial magnetic resonance imaging (MRI) indicated no primary central nervous system lesions, excluding metastatic disease.

During follow-up, surveillance bronchoscopy performed 2 months after resection revealed a polypoid granulomatous protrusion at the site (Figure 1J). The lesion was excised using biopsy forceps under direct visualization, with subsequent APC applied to achieve hemostasis and mucosal cauterization. Histopathological analysis of the excised tissue demonstrated chronic inflammatory changes characterized by lymphocytic infiltration and fibroblastic proliferation, with no evidence of malignant cells. At 69 months of follow-up, the patient remained asymptomatic with a resolved cough and normal pulmonary function.

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient for publication of this article and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Discussion

Tracheobronchial tumors are rare clinical entities and are predominantly malignant. Squamous cell carcinoma and adenoid cystic carcinoma collectively account for 61.1% of reported cases (5). Benign tracheal neoplasms typically include squamous papillomas, chondromas, and granular cell tumors, among others (6). Notably, this case represents the first documented occurrence of primary tracheal ectopic meningioma in the English-language medical literature. The diagnostic challenge for benign tracheal tumors stems from their nonspecific symptomatology and delayed presentation, typically manifesting as dyspnea or chronic cough. Clinically significant airway obstruction occurs when tracheal diameter narrows beyond 50%, with exertional dyspnea emerging at critical thresholds below 8 mm (7). Our patient’s presentation with activity-induced chest tightness and intermittent paroxysmal cough aligns with this progression pattern. Advanced CT imaging modalities, particularly multiplanar reconstruction and virtual bronchoscopy, have enhanced the preoperative assessment of tracheal lesions (8). In this case, contrast-enhanced CT revealed a well-demarcated 1.6 cm × 1.4 cm proximal tracheal mass causing significant luminal compromise. Bronchoscopic evaluation remains the diagnostic gold standard, providing critical information regarding lesion morphology, vascularity, and degree of obstruction while enabling simultaneous histopathological sampling (9). However, practitioners must weigh the risk of biopsy-induced edema and hemorrhage against diagnostic urgency, particularly in high-grade stenoses. The American Thoracic Society guidelines recommend rigid bronchoscopy as the preferred approach for maintaining airway security during interventional procedures (10). Contemporary management strategies emphasize two primary objectives: immediate restoration of airway patency and definitive treatment addressing the underlying pathology (11). Emergent interventions are indicated when critical stenosis (>70% luminal occlusion) or hemorrhagic risk threatens respiratory compromise (11). LMA is the most widely used approach for fiberoptic bronchoscopy due to its low technical difficulty, unimpaired ventilation (without requiring high-frequency ventilation), and less stringent anesthesia depth requirements as compared to rigid bronchoscopy, which involves greater technical expertise, deeper anesthesia, increased risks of glottic edema and dental injury. However, it offers limited operational space, making it unsuitable for massive hemorrhage, emergency airway obstruction rescue, or the placement of silicone stents and T-tubes (12). Our treatment algorithm for LMA incorporated sequential bronchoscopic techniques: initial electrosurgical debulking, which achieved rapid lumen recanalization; APC for residual tumor ablation; and adjuvant cryotherapy, which mitigated recurrence risk through cytoreductive effects. During the procedure, the application of radial probe endobronchial ultrasound may help determine the origin of the lesion (intrinsic or extrinsic compression) and evaluate the depth of tracheobronchial wall invasion, thereby facilitating treatment planning and modification (13).

Immunohistochemical profiling plays a pivotal role in the diagnosis of meningioma, typically demonstrating strong vimentin and EMA positivity with variable PR expression (14,15). Our case exhibited characteristic immunoreactivity for vimentin, EMA, and focal PR positivity, complemented by a low Ki-67 proliferation index (1%), consistent with benign histology. These findings indicated conservative management over surgical resection. PPMs, often presenting radiologically as solitary nodules or micronodular clusters, remain diagnostic challenges (3). Although Masai et al. reported the first bronchoscopic diagnosis with endobronchial ultrasound-guided sheath (EBUS-GS) sampling of peripheral lesions (16), most reported cases (including endobronchial variants) require surgical confirmation (3,17). Cases reported by Fidan et al. exemplify the technical limitations of bronchoscopic biopsy, with thoracotomy being required for definitive diagnosis despite two prior endoscopic attempts (17). Our therapeutic approach mirrors Prasad et al.’s bronchoscopic management of main bronchus meningioma, albeit with notable distinctions (18). The substantial specimen size (16 mm × 14 mm × 10 mm) enabled definitive histopathological diagnosis, circumventing the diagnostic uncertainty common in smaller biopsies. Following recurrence at 2 months, repeat endoscopic intervention achieved durable remission without the further need for thoracic surgery, with the patient’s 69-month disease-free survival thus far representing a key clinical advancement.

Conclusions

This report presents the first documented case of primary tracheal ectopic meningioma successfully managed through bronchoscopic resection. Our experience suggests that endoscopic techniques may achieve definitive treatment in select airway meningiomas, and serial bronchoscopic surveillance enables early recurrence detection and minimally invasive retreatment.

Supplementary

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Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient for publication of this article and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.