Laryngeal Paraganglioma: An Updated Critical Review

Abstract

Laryngeal paragangliomas are rare submucosal lesions that arise from paraganglion cells located in the false vocal fold and subglottic larynx. To date, 76 recognized cases have been reported in the world literature. Symptoms arise when the lesions become large enough to impair function. Supraglottic paragangliomas cause hoarseness and deglutition disorders, whereas subglottic tumors become symptomatic when they obstruct the airway. Evaluation of these tumors includes obtaining a complete history. Familial paragangliomas and hypertension should be sought but are rarely, if ever, associated with laryngeal paragangliomas. MRI can detect these lesions and permit characterization of the vascularity of the lesion. Adding 111In pentetreotide scanning can distinguish neuroendocrine tumors from other submucosal laryngeal lesions, making the preoperative diagnosis clearer and obviating the need for biopsy. The biggest dilemma regarding laryngeal paragangliomas is making the correct pathologic distinction between paraganglioma, typical carcinoid, atypical carcinoid and medullary thyroid cancer. Immunohistochemical markers, supplementing standard histopathologic evaluation, can distinguish paragangliomas from the aforementioned tumors. This distinction is critical as the prognosis for treated paragangliomas is excellent compared to that for other neuroendocrine neoplasms. Almost all alleged malignant paragangliomas of the larynx are in reality atypical carcinoid tumors that have been misdiagnosed. Treatment should always comprise excision. Thyrotomy has the best chance of achieving a sustained cure without damaging phonation or deglutition. Laser excision has been used successfully but there is no great experience with this modality. Surgery is preferable to radiation for paragangliomas in all locations but especially so in the larynx, due to issues such as swelling, airway protection and destruction of cartilage. With increased clinical suspicion and the use of modern imaging techniques, laryngeal paragangliomas should be routinely diagnosed and treated without loss of laryngeal functions.

Laryngeal Paraganglioma: A Rare Clinical Entity Managed by Supraselective Embolization and Lateral Pharygotomy

“…Ferlito et al [1,2,34] suggested the existence of other atypical carcinoid tumors as high as 25 % which were earlier thought as metastasis. They reviewed 65 cases over 35 years and found the mean age to be 44 years, mean duration of symptoms to be 26 months and most of the tumors were excised surgically [1,2,34].…”

“…Digital subtraction angiography also allows the determination of carotid artery invasion and the performance of balloon occlusion studies to assess collateral cerebral circulation in carotid body paragangliomas [10,33]. Usually superior thyroid artery is the primary vessel that supplies these tumors, so embolization creates a bloodless surgical field [34]. Preoperative embolization done by superselective catheterization of the supplying branches and transarterial embolization with particulate agents dramatically improves the operative field [35].…”

“…Malignant paragangliomas are reported in 2 % in some series while pathologic findings cannot predict clinical and biological behavior of the tumor [1,2,34]. Rare cases of distant metastasis is reported while neck nodes spread are rarely reported and so selective neck node clearance is not warranted [1,2].…”

“…They reviewed 65 cases over 35 years and found the mean age to be 44 years, mean duration of symptoms to be 26 months and most of the tumors were excised surgically [1,2,34]. They reported no role of radiation or chemotherapy [5].…”

Primary neuroendocrine tumors of the ear, nose and throat: A report of three cases and a review of the literature

“…Laryngeal paragangliomas are almost always benign and should be treated accordingly. Partial laryngectomy is preferable to radiation as a cure is usually achieved without loss of laryngeal function (15). Laser surgery is not widely used due to the vascular nature of these tumors (13).…”