Ann Thorac Surg 2012;93:1717-1719. doi:10.1016/j.athoracsur.2011.09.053
© 2012 The Society of Thoracic Surgeons
Case Reports
Tracheal Paraganglioma: An Unusual Neoplasm of the Upper Airway
Mark T. Metzdorff, MDa,*,
Joseph C. Seaman, MDc,
David A. Opperman, MDa,
Jeffrey J. Goates, MDb,
Ali I. Musani, MDc
a Department of Surgery, Rose Medical Center, Denver, Colorado
b Department of Pathology, Rose Medical Center, Denver, Colorado
c Department of Medicine, Division of Interventional Pulmonology, National Jewish Health, Denver, Colorado
Accepted for publication September 19, 2011.
* Address correspondence to Dr Metzdorff, 1601 E 19th Ave, Ste 5000, Denver, CO 80218 (Email: mtmetz{at}teleport.com).
 |
Abstract
|
|---|
Paraganglioma of the trachea is a rare neoplasm, with fewer than 15 cases reported. A 40-year-old man presented with stridor and hemoptysis. Bronchoscopy demonstrated a tumor of the posterior trachea and biopsy initially suggested typical carcinoid. The patient underwent surgical resection uneventfully and made a good recovery. Final pathology disclosed the tumor to be a paraganglioma based on immunohistology. The pathophysiology and treatment of this tumor are discussed.
|
Introduction
|
|---|
Paraganglioma is an unusual histology for tracheal neoplasms, with fewer than 15 cases reported in the literature. We recently encountered a typical presentation of this rare entity and share our experience in its diagnosis and treatment.
A 40-year-old man presented with a 1-year history of hemoptysis and increasing stridor. He was initially seen by a pulmonologist and thoracic surgeon at an outlying hospital and underwent bronchoscopy with attempted biopsy of an upper tracheal tumor, but the procedure was terminated due to excessive bleeding, and no diagnosis was made. He was referred to our center, where another attempt at biopsy was made by our interventional pulmonologist after careful preparation with thoracic surgical consultation and availability of rigid bronchoscopy with laser coagulation. The tumor was seen to be a fleshy, glistening, sessile vascular mass based on the membranous trachea near the second tracheal ring (Fig 1). Although significant bleeding was again encountered, a satisfactory specimen was obtained and hemostasis was obtained with the aid of the Nd:YAG laser. The initial pathological interpretation was typical carcinoid, based on the histological appearance as well as reactivity for chromogranin, synaptophysin, and CD56. Because of the subglottic location of the tumor, otorhinolaryngology consultation was requested and a high-resolution computed tomography (CT) scan of the neck was obtained (Fig 2).
View larger version (89K):
[in this window]
[in a new window]
|
Fig 2. Computerized tomographic appearance of tracheal tumor, (A) axial (tumor between arrows) and (B) sagittal views.
|
|
The patient was electively taken to the operating room, where intraoperative bronchoscopy confirmed the location of the tumor and aided in locating the margins for tracheal resection. Through a low cervical incision, the trachea was initially divided distal to the tumor and airway management converted from a laryngeal mask airway to on-the-field distal intubation. A tracheal resection involving the second and third tracheal rings was performed, and intraoperative frozen section examination confirmed negative surgical margins. The trachea was reconstructed with end-to-end anastomosis. The patient awoke with no evidence of recurrent laryngeal nerve injury, made an uneventful recovery, and was discharged on the third postoperative day. Two weeks later, fiberoptic laryngoscopy demonstrated good healing of the anastomosis.
The final pathology report revealed the tumor to be a 1.4-cm paraganglioma with negative surgical margins (Fig 3). The new diagnosis was supported by additional immunoperoxidase stain examinations as follows: AE1/AE3, negative; CAM 5.2, negative; S-100, positive (focal); MOC31, negative; and CD99, negative.
View larger version (81K):
[in this window]
[in a new window]
|
Fig 3. Histological appearance of the tracheal tumor. (A) Hematoxylin and eosin stain, original magnification x200, showing well-defined nests of tumor cells ("zellballen") surrounded by fibrovascular septa. (B) S-100 stain, original magnification x400, showing tumor cell nests surrounded by S-100–positive sustentacular cells.
|
|
|
Comment
|
|---|
Paragangliomas are members of the extensive and diffuse category of neuroendocrine tumors. They are nearly always benign neoplasms that arise from extra-adrenal chromaffin cells derived from the neural crest. They are related to pheochromocytomas but rarely secrete catecholamines in amounts significant to cause symptoms. They may occur in any location where chromaffin cells migrate or are found embryologically. They are typically found in the neck, mediastinum, and abdomen and most commonly in association with the cranial nerves [1].
Tracheal paraganglioma was first described in 1956 [2]. Review of the literature yields only about 11 cases of purely tracheal paraganglioma [3], with a propensity for the tumor to arise from the membranous trachea, as occurred in our case. Typically very vascular tumors, tracheal paragangliomas usually present with hemoptysis, and biopsy may be fraught with difficulty because of bleeding. If preoperative biopsy is entertained, optimal diagnostic management should include involvement of a thoracic surgeon and availability of complex airway management techniques including rigid bronchoscopy. A case can be made for proceeding directly to surgery, as the principles of treatment (excision with adequate margin and primary reconstruction) are the same with all tracheal neoplasms. However, we believe that preoperative knowledge of tumor type may be an advantage in dealing with more aggressive tumors, where further workup or a larger margin may be required.
Treatment is surgical excision, and several approaches have been described, including tracheal resection, preoperative embolization with subsequent resection [4], and endotracheal dissection through tracheofissure followed by cauterization of the tumor bed [5]. Preresection tracheostomy was used in at least 2 cases [5, 6], but this may not be necessary with modern surgical and anesthetic techniques in a typical case. A case treated with laser ablation because of location at the carina was complicated by subsequent tracheomalacia, which required tracheal stenting, followed by radiation therapy [7]. Simple excision with minimal adequate margin and primary reconstruction was the most commonly used and most successful surgical technique in the reported cases and can be recommended as optimal therapy.
With attention to surgical technique and adequate excision, complications are rare and prognosis is good. Although long-term follow-up is lacking in most of the reports, one of the reported cases describes recurrence 9 months after initial treatment [8]. The recurrence was probably due to the tumor being removed endoscopically and was subsequently controlled with laser resection. Not all paragangliomas behave benignly, however, and clinicians should maintain an index of suspicion for recurrence if symptoms warrant.
|
Acknowledgments
|
|---|
Images of the radiographs in Figure 2 were provided by Craig Kornbluth, MD, of the Department of Radiology, Rose Medical Center.
|
References
|
|---|
- Kantorovich V, King KS, Pacak K. SDH-related pheochromocytoma and paraganglioma Best Pract Res Clin Endocrinol Metab 2010;24:415-424.[Medline]
- McCall JW, Karam FK. Chemodectoma of the trachea AMA Arch Otolaryngol 1958;67:372-373.[Medline]
- Batioglu-Karaaltin A, Karaaltin MV, Esozlu I, Cansiz H. Tracheal paraganglioma: A case report Kulak Burun Bogaz Ihtis Derg 2011;21:229-233.[Medline]
- Michaelson PG, Fowler CB, Brennan J. Tracheal paraglioma presenting with acute airway obstruction Otolaryngol Head Neck Surg 2005;132:661-662.[Medline]
- George M, Ayyappan P, Cherian R, Kurien K. Tracheal paraganglioma: A rare vascular neoplasm AJR Am J Roentgenol 2006;187:231-234.
- Maisel R, Schmidt D, Pambuccian S. Subglottic laryngeal paraganglioma Laryngoscope 2003;113:401-405.[Medline]
- Sing TMYS, Wong KP, Young N, Despas P. Chemodectoma of the trachea Thorax 1996;51:341-342.[Abstract/Free Full Text]
- Jones TM, Alderson D, Sheard JD, Swift AC. Tracheal paraganglioma: A diagnostic dilemma culminating in a complex airway management problem J Laryngol Otol 2001;115:747-749.[Medline]
Top
Abstract
Introduction
