Pitfalls in the management of subglottic paragangliomas at unusual location: a case report and literature review

Abstract

Background: Subglottic paragangliomas (PGs) are exceptionally rare and unpredictable, occasionally presenting at an atypical location. There are three different clinical forms of subglottic PGs: intraluminal (tracheal PGs), extraluminal (thyroid PGs) and the mixed type (both intraluminal and extraluminal, mixed-subglottic PGs). These tumors are usually misdiagnosed as other relatively common primary thyroid or laryngotracheal tumors, and the treatment is troublesome.

Case presentation: A 22-year-old male patient with subglottic PGs has been successively misdiagnosed as thyroid tumors and subglottic hemangiomas, and lastly underwent local extended lumpectomy and laryngotracheal reconstruction with a pedicled thoracoacromial artery perforator flap (PTAPF). The patient was decannulated successfully after the second-stage tracheal reconstruction with a local flap, and no evidence of local recurrence and distant metastasis of the tumor until now.

Conclusion: Subglottic PGs can be easily misdiagnosed as laryngotracheal or thyroid tumors when presented at an atypical location. It is essential for otolaryngologists and head and neck surgeons to remain vigilant against these tumors. If the tumor is not diagnosed or removed completely, patients may encounter a risk of lethal paroxysm, which is incredibly troublesome.

Keywords: Case report; Laryngeal paraganglioma; Subglottic paraganglioma; Thyroid paraganglioma; Tracheal paraganglioma.

Fig. 1

Preoperative imaging of the new reported case. A the smooth and sessile vascular tumor (labeled with asterisk) was seen in the subglottis and vascular proliferation in a pseudo angiomatosus (labeled with triangle) was captured on the tumor surface by flexible fiberoptic bronchoscope (the right image is a close view of the left image). B CT scans with plain (the left) and contrast-enhanced (the right) image showing the intraluminal and extraluminal involvement of left subglottic larynx (labeled with asterisk). The right lobe of the thyroid (labeled with arrow) was present while the left lobe and isthmus of thyroid were absent. C the demarcation line was not clearly recognized between the tumor (labeled with asterisk) and the thyroid (labeled with arrow) by plain CT scan (the left) and contrast-enhanced CT scan (the right) before thyroidectomy (7 years prior to this study)

Fig. 2

Operative imaging and pathological analysis of the new reported case. A intra-operative view of the tumor (labeled with asterisk) and the cricoid cartilage (labeled with arrow). B the gross specimen consisted of a 3.0- × 3.0- × 2.0-cm irregular, tan–pink mucosal lesion attached to the lower portion of the cricoid cartilage and the first three tracheal cartilages. C the chief cells (labeled with asterisk) show abundant and pale pink cytoplasm, and round nuclei with nucleoli. The peripheral sustentacular cells (labeled with arrow) are small and long with oval nuclei. Vascular sinusoids (labeled with triangle) are around the organoid pattern of “zellballen” (Hematoxylin–Eosin × 40), scale bar: 50 μm. D, E immunostaining analysis shows that chief cells are positive for chromogranin (D) and synaptophysin (E) in the cytoplasm (arrow, original magnification ×20), scale bar: 100 μm. F the peripheral sustentacular cells are outlined by S100 immunostaining (arrow, original magnification ×20). G intra-operative view of reconstruction of the defect with PTAPF (labeled with arrow), scale bar: 100 μm. H CT scans shows the reconstructed trachea with successful decannulation and no sign of tumor recurrence at the third year post-operatively

Fig. 3

The timeline of medical record of the new reported case