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Laryngeal paraganglioma: report of an unusual entity.

Abstract

Paragangliomas are rare in the larynx. When they do occur there, the most common subsite is the supraglottic compartment. Unlike other neuroendocrine tumors of the larynx, laryngeal paragangliomas are three times as common in women as in men. Although a preoperative biopsy is often performed to establish the diagnosis, this procedure carries a considerable risk of bleeding, which may necessitate a tracheotomy to secure the airway. Immunohistochemical staining is useful in the differential diagnosis to distinguish a paraganglioma from other neuroendocrine tumors. Computed tomography and/or magnetic resonance imaging with preoperative angiography and possible embolization are important to obtain prior to treatment. Surgical excision is the treatment of choice.

Introduction

Paraganglia are clusters of neuroendocrine cells that are connected to the autonomic nervous system and dispersed throughout the body. These cells act as chemoreceptors, and they secrete catecholamines. (1) The largest collection of these cells resides in the adrenal medulla. Tumors of the adrenal medulla are called pheochromocytomas, while tumors that arise from the extra-adrenal paraganglia are called paragangliomas. Paragangliomas are generally benign and highly vascular neoplasms that arise from the neural-crest-derived paraganglia of the autonomic nervous system. (1,2)

Paragangliomas of the head and neck are rare, accounting for about 0.6% of all head and neck tumors and about 0.03% of all tumors. (3) The most common of the paragangliomas of the head and neck are, in descending order of prevalence, carotid body paragangliomas, jugulotympanic paragangliomas, and vagal paragangliomas. (1,2) Laryngeal involvement, which was first reported by Blanchard and Saunders (4) in 1955, is rare. When laryngeal paragangliomas have occurred, they have been more common in women than in men (female-to-male ratio: 3:1), unlike other neuroendocrine tumors of the larynx. (2,5-9) The mean age at diagnosis is 44 years (range: 14 to 80). (6)

The larynx contains two paired paraganglia, superior and inferior, which are located along the course of the superior and recurrent laryngeal nerves, respectively. (5,6,8) Laryngeal paragangliomas almost always (>90% of cases) arise from the superior paraganglia--more precisely, from the supraglottic larynx. (5,6,9) They are usually located in the area of the right aryepiglottic fold and the false vocal fold. (2,6) These tumors are rarely functional; only about 2.9 to 3.6% of all head and neck paragangliomas hypersecrete catecholamines. (6,10) Paragangliomas occur both sporadically and as a part of familial syndromes. The prevalence of multiple tumors is 10 to 20% among sporadic cases, and it may reach 80% in familial cases?

In this article, we present a case of laryngeal paraganglioma in the supraglottic compartment of a male patient.

Case report

A 34-year-old man was referred to our clinic for evaluation of hoarseness that had slowly progressed over the previous 5 years. Endoscopic examination detected a smooth submucosal right-sided mass that involved the aryepiglottic fold, false vocal fold, and true vocal fold (figure 1). Vocal fold mobility was somewhat restricted on the right because of the presence of the mass; mobility on the left was unaffected. No palpable cervical lymphadenopathy was found. Laboratory testing revealed that all biochemical values, including the hemoglobin concentration, were in the normal range.

[FIGURE 1 OMITTED]

Computed tomography (CT) with contrast demonstrated a hyperdense mass that extended from the right aryepiglottic fold to the right vocal fold (figure 2). The mass had obliterated the paraglottic space and distorted the thyroid cartilage. The tumor did not extend to the subglottic compartment.

An endoscopic incisional biopsy was performed with general anesthesia. In response to severe intraoperative bleeding, a tracheotomy was performed to secure the airway. The mass was found to be filling the right side of the larynx. It had a rather smooth, lobulated appearance. After the specimen was taken, the profuse bleeding was successfully controlled by electrocautery. On histopathologic examination, the mass was diagnosed as a paraganglioma.

Surgical resection with preoperative embolization was planned. Angiography revealed that the mass was highly vascularized, chiefly from the superior thyroid artery. Embolization of the tumor was performed without any complication. On the third day following embolization, surgical intervention was carried out. The larynx was entered via a laryngofissure approach. Intraoperatively, the mass was found to fill the right ventricle, and it extended to the thyroid cartilage without invading it. The superior thyroid artery and vein were ligated, and the mass was elevated from the inner perichondrium of the thyroid cartilage. The mucosa covering the mass was also elevated, and after complete skeletonization, the mass was excised in toto. Intraoperative blood loss was minimal. The left vocal fold and ventricular fold were left intact. The endolaryngeal mucosa was reconstructed with simple sutures, and the laryngeal framework was closed appropriately.

[FIGURE 2 OMITTED]

During recovery, the patient was allowed to resume oral intake on postoperative day 3, and he was decannulated on day 5. No postoperative complications were noted. Throughout 3-plus years of follow-up, the patient remained well and exhibited no evidence of recurrence.

On gross examination, the tumor was grey and tan. Histologic examination identified nests of large polygonal to spindle-shaped cells with round nuclei and prominent pale-pink cytoplasm (figure 3). The nests were surrounded by scant stroma containing a delicate vascular network. The nuclei were mildly pleomorphic. No mitotic activity was present. On immunohistologic analysis, sustentacular cells stained strongly positive with S-100. The findings confirmed the diagnosis of a laryngeal paraganglioma.

Discussion

The nature of symptoms of a laryngeal paraganglioma depends on the size and location of the tumor. Symptoms may include dyspnea, dysphagia, hoarseness, a foreign-body sensation, and stridor. (5) Tumor spread may lead to vocal fold fixation. (9) Most of these tumors originate in the supraglottic larynx as a submucosal mass. (6) They are rarely associated with necrosis and vascular invasion, and they are generally benign; malignancy occurs in less than 2% of cases. (5,9) A recurrence rate of 17% has been reported. (11) Local recurrence may be a sign of malignancy. (8)

[FIGURE 3 OMITTED]

Contrast-enhanced CT and, if necessary, magnetic resonance imaging (MRI) should be included in the standard workup of all laryngeal masses. (2) MRI may be considered the imaging modality of choice. (5) Also, preoperative angiography may define the size and location of the lesion and its blood supply; it should be used in combination with embolization to decrease the blood supply of the paraganglioma. (2) In addition, angiography may detect a synchronous tumor. (8) Still, angiography in these cases remains a matter of discussion in otolaryngology, (5) and paragangliomas can be adequately assessed without it.

Laryngeal paragangliomas are generally diagnosed histologically prior to definitive treatment. Preoperative biopsies are recommended by some authors, (2) even though the result is that the patient must undergo at least two invasive procedures. Because laryngeal paragangliomas are covered with an intact mucosa, a deep biopsy may be required. (12) In view of the highly vascularized nature of these lesions, bleeding may be bothersome during biopsy. In our patient, bleeding was so severe that a tracheotomy became necessary to secure the airway. Indeed, excessive bleeding during a biopsy of a submucosal mass may be regarded as a hint that the tumor is a laryngeal paraganglioma. If the initial imaging findings provide sufficient clues as to the probable diagnosis of a paraganglioma (or any other vascular tumor), then a biopsy can be omitted and direct surgery following angiography and embolization may be more appropriate.

Some tumors maybe large enough to herniate through the thyrohyoid membrane and present as a neck mass. (5) The differential diagnosis of laryngeal paraganglioma includes carcinoid tumor, malignant melanoma, medullary thyroid carcinoma, and small-cell neuroendocrine carcinoma. (2,5) Immunohistochemical staining may be useful in distinguishing among the possibilities. (6) S-100 testing is particularly necessary to establish the correct diagnosis.

The treatment of choice in laryngeal paragangliomas is surgical resection. (6) Surgical excision methods include supraglottic or total laryngectomy and endoscopic resection. (2,9,13) However, open surgery is associated with better rates of disease control. (5) In our patient, we preferred simple excision via a laryngofissure approach. Most of these tumors can be excised completely. (13) The superior thyroid artery is the primary vessel that supplies these tumors. (2) Embolization and ligation of this vessel may help the surgeon obtain a relatively blood-free surgical field. Radiotherapy and chemotherapy are considered ineffective in the treatment of laryngeal paragangliomas. (9)

Because laryngeal paragangliomas are rarely malignant and do not metastasize to the cervical lymph nodes, elective neck dissection is not indicated. (5)

References

(1.) Boedeker CC, Ridder GJ, Schipper J. Paragangliomas of the head and neck: Diagnosis and treatment. Fam Cancer 2005;4(1):55-9.

(2.) Sesterhenn AM, Folz BJ, Lippert BM, et al. Laser surgical treatment of laryngeal paraganglioma. J Laryngol Otol 2003;117(8):641-6.

(3.) Lee JH, Barich F, Karnell LH, et al for the American College of Surgeons Commission on Cancer and the American Cancer Society. National Cancer Data Base report on malignant paragangliomas of the head and neck. Cancer 2002;94(3):730-7.

(4.) Blanchard CL, Saunders WH. Chemodectoma of the larynx; case report. AMA Arch Otolaryngol 1955;61(4):472-4.

(5.) Myssiorek D, Rinaldo A, Barnes L, Ferlito A. Laryngeal paraganglioma: An updated critical review. Acta Otolaryngol 2004; 124(9):995-9.

(6.) Del Gaudio JM, Muller S. Diagnosis and treatment of supraglottic laryngeal paraganglioma: Report of a case. Head Neck 2004;26 (1):94-8.

(7.) Stanley RJ, Scheithauer BW, Weiland LH, Neel HB III. Neural and neuroendocrine tumors of the larynx. Ann Otol Rhinol Laryngol 1987;96(6):630-8.

(8.) Ozunlu A, Dundar A, Satar B, Gunhan O. Laryngeal paraganglioma. A review and report of a single case. J Laryngol Otol 1996;110(6): 519-26.

(9.) Gupta S, Pathak KA, Sanghvi V. Transventricular paraganglioma of the larynx. Eur Arch Otorhinolaryngol 2003;260(7):358-60.

(10.) Erickson D, Kudva YC, Ebersold MJ, et al. Benign paragangliomas: Clinical presentation and treatment outcomes in 236 patients. J Clin Endocrinol. Metab 2001;86(11):5210-16.

(11.) Ferlito A, Barnes L, Rinaldo A, et al. A review of neuroendocrine neoplasms of the larynx: Update on diagnosis and treatment. J Laryngol Otol 1998;112(9):827-34.

(12.) Werner JA, Hansmann ML, Lippert BM, Rudert H. Laryngeal paraganglioma and pregnancy. ORL J Otorhinolaryngol Relat Spec 1992;54(3):163-7.

(13.) Young WF Jr. Paragangliomas: Clinical overview. Ann N Y Acad Sci 2006;1073:21-9.

Asim Kaytaz, MD; Emin Karaman, MD; Deniz Tuna Edizer, MD; Yusuf Haciyev, MD; Buge Oz, MD

From the Department of Otorhinolaryngology (Dr. Kaytaz, Dr. Karaman, Dr. Edizer, and Dr. Haciyev) and the Department of Pathology (Dr. Oz), Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.

Corresponding author: Deniz Tuna Edizer, MD, Department of Otorhinolaryngology, Istanbul Universitesi Cerrahpasa Tip Fakultesi KBB ABD, Istanbu1 34099, Turkey. E-mail: deniztunaedizer@yahoo. com
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Title Annotation:ORIGINAL ARTICLE
Author:Kaytaz, Asim; Karaman, Emin; Edizer, Deniz Tuna; Haciyev, Yusuf; Oz, Buge
Publication:Ear, Nose and Throat Journal
Article Type:Clinical report
Geographic Code:7TURK
Date:Nov 1, 2010
Words:1782
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