Eur J Cardiothorac Surg 2004;25:655-657
© 2004 Elsevier Science NL
Inferior laryngeal paraganglioma presenting as plunging goiter
Olgun Kadir Aribasa*,
Fikret Kanatb,
Mustafa Cihat Avundukc
a Department of Thoracic Surgery, Meram Medical School of Selcuk University, 42080 Meram, Konya, Turkey
b Department of Chest Diseases, Meram Medical School of Selcuk University, Konya, Turkey
c Department of Pathology, Meram Medical School of Selcuk University, Konya, Turkey
Received 2 September 2003;
received in revised form 30 December 2003;
accepted 2 January 2004.
* Corresponding author. Address: Selcuk Universitesi Meram Tip Fakultesi Hastanesi, Gogus Cerrahisi Anabilim Dali, 42080 Meram–Konya, Turkey. Tel.: +90-332-3232600/1844; fax: +90-332-3243730
e-mail: olgun{at}selcuk.edu.tr
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Abstract
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Paragangliomas are uncommon slow-growing neuroendocrine tumors that may arise from the extra-adrenal paraganglia. Paragangliomas of the inferior and superior laryngeal paraganglia are known as laryngeal paraganglioma. Inferior laryngeal paraganglioma, which is also called subglottic paraganglioma, is very seldomly observed. To our knowledge only 24 patients with subglottic paraganglioma have been found. We present a 77-year-old male patient who has been previously followed-up for cervical goiter, which has a progressive enlargement into the mediastinum, causing severe tracheal obstruction. The tumor was completely and easily resected via median sternotomy with collar incision and finally diagnosed as inferior laryngeal paraganglioma. The present case is the first subglottic laryngeal paraganglioma descending into the visceral compartment of the mediastinum in the literature.
Key Words: Paraganglioma • Laryngeal paraganglioma • Inferior laryngeal paraganglioma • Subglottic paraganglioma • Plunging goiter • Mediastinum
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1. Introduction
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Paragangliomas are neuroendocrine, slow-growing, highly vascular tumors. They arise from the extra-adrenal paraganglia of the autonomic nervous system. They may be present in different locations. Paragangliomas arising from the inferior and superior laryngeal paraganglion are called laryngeal paraganglioma, which may be either supraglottic, glottic or subglottic [1]. Subglottic paragangliomas originate from the inferior laryngeal paraganglion and they are extremely rare [1,2]. We have found only a total of 24 patients with paraganglioma derived from the inferior laryngeal paraganglia [3].
We report a patient with paraganglioma originating from the inferior laryngeal paraganglion, who has been followed for goiter for 10 years.
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2. Case
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A 77-year-old male patient who had been followed-up for goiter for 10 years was admitted with an attack of severe dyspnea. On physical examination he appeared lethargic, the trachea was displaced to right, and bilaterally diminished respiratory sounds and inspiratory stridor were heard on auscultation. He had an immobile mass to the left of neck. The patient had mild leukocytosis (12,100/mm3). Erythrocyte sedimentation rate was 90 mm/h. Blood biochemical examination was normal. Thyroid function tests were within normal limits. Chest roentgenogram revealed a mass of upper mediastinum narrowing the trachea and compressing it to the right (Fig. 1A)
. Chest CT scan disclosed a 13 cm diameter, anterior and upper mediastinal mass descending into the aortopulmonary window, and causing displacement of the trachea to right, narrowing the lumen approximately by 80% and making appearance of saber-sheath trachea (Fig. 1B). Since the patient had severe respiratory insufficiency (arterial blood gas; pH 7.26, PaO2 38 mmHg and PaCO2 56 mmHg) he was intubated and supported with mechanical ventilation. Later he underwent immediate surgery. Trachea was examined via rigid bronchoscopy just before the operation and we observed that the trachea was severely narrowed beginning from nearly 2 cm distal to the vocal cords ending 3 cm proximal to the carina. The tracheal mucosa was intact. Via cervical transverse incision with median sternotomy, an encapsulated, highly vascularized, and lobulated huge mass measuring 13x11x8 cm3 in size and originating from the level of the left lobe of thyroid gland was totally excised (Fig. 2A)
. Cuboidal cells, which have abundant granular cytoplasm and separated by vascularized fibrous septa, are detected microscopically. Bizarre nuclei and vascular invasion are not seen. Tumor cells expressed neuron-specific enolase and chromogranin immunohistochemically (Fig. 2B). The postoperative period was uneventful. The patient has been followed-up for about 2 years. Clinical and radiological examination of the patient was completely normal 2 years after the operation (Fig. 1C and D).
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Fig. 1. (A) Chest roentgenogram showing a mass of upper mediastinum narrowing the trachea and compressing it to the right (arrows). (B) Chest CT scan disclosing a 13 cm diameter, anterior and upper mediastinal mass (asterix) causing displacement of the trachea to right, narrowing lumen approximately 80% and making appearance of saber-sheath trachea (arrowhead) and lying towards the aortopulmonary window. (C) Chest roentgenogram taken 2 years after the operation showing only a mild tracheal displacement to right. (D) Chest CT scan 2 years after the operation.
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Fig. 2. (A) Encapsulated, highly vascularized, and lobulated, resected huge mass measuring 13x11x8 cm3 and the cut surface of the tumor. (B) Abundant granular cytoplasm are seen immunohistochemically (chromogranin x40).
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3. Discussion
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Paragangliomas, tumors of the extra-adrenal paraganglionic system, are unusual neoplasms that originate from the paraganglions, which are the minute macroscopic and microscopic cell groups associated with the autonomic nervous system [4]. They are described in many locations and classified according to their anatomical site of origin and the presence of their functional activity. In the head and neck region, paragangliomas are associated with the carotid body, the vagus nerve, the middle ear or jugulotympanic paraganglia, and the superior and inferior laryngeal paraganglia [5].
Laryngeal paragangliomas, which are extremely rare, derive from superior or inferior laryngeal paraganglia [1,2]. The superior laryngeal paraganglia are situated near the superior edge of the thyroid cartilage, and the inferior laryngeal paraganglia are usually found between the thyroid cartilage and the first tracheal ring, occasionally within the capsule of the thyroid gland. [6]. Majority of the laryngeal paragangliomas are supraglottic (82%); 12% are subglottic and 3% are glottic [1]. Ferlito [2] reorganized the collection of laryngeal paragangliomas by Barnes [1] and after addition of new cases, he noted that only 15% of the laryngeal paragangliomas were subglottic, which were believed to arise from the inferior laryngeal paraganglia [2]. Recently a patient with transventricular paraganglioma with fixed hemilarynx has been reported [7].
Laryngeal paragangliomas are more common in women, and in the fourth and sixth decades of life. They also show right side predominance [2]. Our patient was a 77-year-old male and the tumor descended to the mediastinum from the left side of the neck.
Although paragangliomas are regarded as benign tumors, they may cause significant problems because of their location. No morphologic criteria have been described for separation of the benign from the malignant paraganglioma. They are accepted malignant if a distant metastasis is present [8]. They may present with symptoms related to the compression of adjacent structures if they are nonfunctional. The most common presenting symptoms are hoarseness, throat pain, dysphagia, dyspnea, hemoptysis, and airway obstruction. However, there may be headache, diaphoresis, palpitations, and other symptoms of a hyperadrenergic state, such as hypertension, paroxysms, and cardiac, gastrointestinal and metabolic manifestations, headache, hypertension, which are typical of a pheochromocitoma. These symptoms are less apparent even in a functional paraganglioma and rarely present in a malignant paraganglioma [9]. Our patient had been admitted to the emergency service with respiratory insufficiency due to a severe tracheal obstruction and he was immediately intubated with great effort. When he was interrogated after the operation we were informed that he suffered from progressive dyspnea for the last few years.
Paragangliomas are known to be radioresistant, thus, surgical resection is the treatment of choice. Since these tumors are highly vascular and often adhere to vital structures some authors advocate preoperative radiotherapy or embolization to reduce the vascularity [8]. However, these tumors are generally diagnosed after surgical removal, therefore the above means are difficult to perform in most of the cases. Chemotherapy is recommended for the unresectable or recurrent tumor [8,10]. For our patient we performed median sternotomy with collar incision. We observed a huge, brownish, solid, encapsulated, highly vascularized and lobulated mass, displacing the thyroid and trachea extremely to right and vascular structures to anterior, descending to the level of aortic arch, and having a blood supply from the left inferior thyroid artery. The tumor was completely resected without a complication.
In conclusion our case has shown that some of the patients followed for goiter for long term might have subglottic laryngeal paraganglioma. This diagnosis should also be considered especially in patients considered to have a huge goiter-compressing trachea. To our knowledge this case is the first subglottic laryngeal paraganglioma descending into the visceral compartment of the mediastinum.
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References
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Abstract
1. Introduction
