|Year : 2020 | Volume
| Issue : 2 | Page : 53-56
Aditya Jain, Satish Nair, Deeksha Thakur, Ashish S Shah
Department of ENT-HNS, Apollo Hospitals, Bengaluru, Karnataka, India
|Date of Submission||30-Nov-2019|
|Date of Acceptance||07-Jan-2021|
|Date of Web Publication||19-Feb-2021|
Dr. Aditya Jain
Department of ENT-HNS, Apollo Hospitals, 154/11, Bannerghatta Road, Bengaluru - 560 076, Karnataka
Source of Support: None, Conflict of Interest: None
Paragangliomas are rare benign neuroendocrine tumors that originate from the neural crest-derived cells. Paragangliomas in the head and neck are typically found in the region of the carotid body, jugular bulb, and along the 9th and 10th cranial nerves. Laryngeal paragangliomas are rare tumors and are found along the superior laryngeal nerve and recurrent laryngeal nerve. Hoarseness or dysphagia is the most common presenting symptom, and surgical excision is the treatment of choice. A 58-year-old female presented with complaints of progressive difficulty in breathing, especially on lying down with difficulty in swallowing for 1 year. Examination revealed a smooth mass lesion involving the laryngeal aspect of the epiglottis, left pharyngoepiglottic fold, and left aryepiglottic fold. Left endolarynx could not be visualized. Imaging showed a well-defined highly enhancing lesion with multiple feeders from superior thyroid, pharyngeal, and esophageal vasculature. The presumptive diagnosis was a vascular lesion or glomus tumor. The tumor was operated by lateral pharyngotomy approach with a temporary tracheostomy without preoperative embolization. Final histopathology confirmed it to be paraganglioma. The paragangliomas of the larynx are rare neuroendocrine tumors. Complete surgical excision of tumor with preservation of neurovascular structures gives an excellent prognosis.
Keywords: Glomus laryngicum, lateral pharyngotomy, paraganglioma larynx, paraganglioma
|How to cite this article:|
Jain A, Nair S, Thakur D, Shah AS. Glomus laryngicum. Ann Indian Acad Otorhinolaryngol Head Neck Surg 2020;4:53-6
| Introduction|| |
Paragangliomas are rare benign neuroendocrine tumors that originate from the neural crest-derived cells., Paragangliomas account for about 0.6% of all head-and-neck tumors and 0.03% of all neoplasms and are found typically in the region of the carotid body, jugular body, and along the 9th and 10th cranial nerves. Laryngeal paragangliomas are rare tumors and are found along the superior laryngeal nerve and recurrent laryngeal nerves. Hoarseness, dysphagia, and breathing difficulty are the most common presenting symptoms, and surgical excision with or without preoperative embolization is the treatment of choice.,
| Case Report|| |
A 58-year-old female presented with complaints of progressive difficulty in breathing aggravated on lying down with difficulty in swallowing for 1 year. Laryngoscopic examination revealed a smooth mass lesion involving the laryngeal aspect of the epiglottis, left pharyngoepiglottic fold, and left aryepiglottic fold. Left endolarynx could not be visualized [Figure 1].
|Figure 1: Laryngoscopic examination showing a smooth mass lesion in the left supraglottic region|
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Imaging showed a 34 mm × 24 mm, well-defined highly enhancing lesion with multiple feeders from superior thyroid, pharyngeal, and esophageal vasculature at the level of C2–C5. The presumptive diagnosis was a vascular lesion or glomus laryngicum [Figure 2].
|Figure 2: Imaging showing enhancing lesion involving the left supraglottic region|
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The tumor was operated by lateral pharyngotomy approach without preoperative embolization [Figure 3]. The patient was tracheostomized intraoperatively and decanulated on the 5th postoperative day and started oral feeds on the 10th postoperative day. Postoperative examination showed no residual lesion [Figure 4].
Final histopathology revealed a tumor composed of cells arranged in lobules and cord and nests with zellballen pattern separated by delicate sinusoids. The tumor cells were regular round and uniform having round and central vesicular nucleus with nucleoli and abundant acidophilic granular cytoplasm. The nest of tumor cells was surrounded by sustentacular cells. All the features were consistent with paraganglioma [Figure 5].
| Discussion|| |
The paraganglionic system comprises the adrenal medulla and the extra-adrenal paraganglia arising from the neural crest cells. The adrenal medulla gives rise to pheochromocytomas whereas the extra-adrenal gives rise to paragangliomas. The extra-adrenal paraganglia are collections of specialized neuroendocrine cells closely associated with large blood vessels, cranial nerves, autonomic nerves, and ganglia. Extra-adrenal paragangliomas may be found anywhere from the upper neck to the pelvic floor.
In the head and neck, paragangliomas are mostly found in the region of the carotid body, jugular body, along the glossopharyngeal nerve and its tympanic branch, and the vagus nerve. Laryngeal paragangliomas are rare tumors, and the differential diagnosis includes typical carcinoid, atypical carcinoid, small-cell neuroendocrine carcinoma, malignant melanoma, and medullary carcinoma of the thyroid gland.
The larynx has two groups of paraganglia; the superior paraganglia, located in the false vocal cord, are associated with the superior laryngeal nerve and artery. The inferior paraganglia, located near the lateral margin of the cricoid cartilage in the cricotracheal membrane, are associated with the recurrent laryngeal nerve.
The most common age of presentation is 4th–6th decade with female preponderance (3:1). Paragangliomas can occur in familial autosomal dominant traits or multicentric forms, but generally, laryngeal paragangliomas are not observed in these patients.
Functional activity is seen in a few cases (1%–3%). Malignant paragangliomas are reported in 2% in some series.
On imaging, paraganglioma appears as a homogeneous, hypervascular, well-defined soft-tissue mass. A heterogeneous enhancement may be observed if hemorrhage or thrombosis has occurred. Angiography and preoperative embolization are useful in minimizing bleeding and injury to neurovascular structures., In our case, we managed without any preoperative embolization.
The sites of distribution are supraglottic (82%), subglottic (15%), and glottic (1%). Their blood supply is mostly provided by the superior laryngeal artery, a branch of the superior thyroid artery. In our case, the lesion was supraglottic, and there were multiple feeders from superior thyroid artery, pharyngeal vessels, and esophageal vasculature.
Histologically, they are highly vascularized tumors composed of two cell types: chief cells and sustentacular cells. Chief cells are polygonal with inconspicuous nuclei and eosinophilic cytoplasm. Chief cells are packed in a characteristic alveolar or “zellballen” pattern. Scattered spindled sustentacular cells with basophilic cytoplasm are found at the perimeters of the cell balls.
Immunohistochemically, the chief cells express all neuroendocrine markers such as chromogranin and synaptophysin but do not stain with epithelial markers (cytokeratin, carcinoembryonic antigen, and epithelial membrane antigen). Sustentacular cells are stained by antibodies to S-100 protein and glial fibrillary acid protein. Chief cells are S-100 protein and glial fibrillary acid protein negative.,
Open surgical resection via a transcervical or laryngofissure approach is the most commonly used as these provide better results with lower recurrence rates. Other treatment techniques include transoral robotic surgery, endoscopic removal, and microlaryngoscopy with laser excision. Endoscopic techniques are successful with smaller lesions but have been associated with higher rates of recurrence., There is no role of radiotherapy or chemotherapy for organ preservation..In our case, the visualization of the tumor was not achieved for transoral resection, and the open approach with a temporary tracheostomy was performed.
A recurrence rate of 17% is reported in superior laryngeal paragangliomas and 4% in inferior laryngeal paragangliomas. Hence, a close follow-up is warranted, especially in subglottic cases where it can lead to sudden respiratory obstruction.,
| Conclusion|| |
Paragangliomas of the larynx are rare neuroendocrine tumors. Complete surgical excision of tumor with preservation of neurovascular structures gives an excellent prognosis.
Informed consent was obtained from the patient for the publication of the case report.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Ferlito A, Rosai J. Terminology and classification of neuroendocrine neoplasms of the larynx. ORL J Otorhinolaryngol Relat Spec 1991;53:185-7.
Barnes L. Paraganglioma of the larynx: A critical review of the literature. ORL J Otorhinolaryngol Relat Spec 1991;53:220-34.
Chen J. Paraganglioma. Chin J Pathol 2006;35:494-6.
Myssiorek D, Rinaldo A, Barnes L, Ferlito A. Laryngeal paraganglioma: An updated critical review. Acta Otolaryngol 2004;124:995-9.
Sanders KW, Abreo F, Rivera E, Stucker FJ, Nathan CO. A diagnostic and therapeutic approach to paragangliomas of the larynx. Arch Otolaryngol Head Neck Surg 2001;127:565-9.
Lack E. Tumors of the Adrenal Glands and Extraadrenal Paraganglia. (Afip Atlas of Tumor Pathology Series 4). 3rd
ed., Vol. 8. Maryland, USA: American Registry of Pathology (January 2007); 2007.
Myssiorek D, Halaas Y, Silver C. Laryngeal and sinonasal paragangliomas. Otolaryngol Clin North Am 2001;34:971-82.
Barnes L, Tse LL, Hunt JL, Michaels L. Tumours of paraganglionic system: Introduction. In: Barnes L, Eveson JW, Reichart P, Sidransky D, editors. World Health Organization Classification of Tumours. Pathology and Genetics of Head and Neck Tumours. Lyon: IARC Press; 2005. p. 362-3.
Rubin AD, Cheng SS, Bradford CR. Laryngeal paraganglioma in a patient with multiple head and neck paragangliomas. Otolaryngol Head Neck Surg 2005;132:520-2.
Ferlito A, Barnes L, Rinaldo A, Gnepp DR, Milroy CM. A review of neuroendocrine neoplasms of the larynx: Update on diagnosis and treatment. J Laryngol Otol 1998;112:827-34
Tikkakaoski T, Luotonen J, Leinonen S, Siniluoto T, Heikkila O, Paivansalo M, et al
. Preoperative embolization in the management of neck paragangliomas. Laryngoscope 1997;107:821-6.
Naik SM, Shenoy AM, Chavan P, Patil A, Gupta S. Laryngeal paraganglioma: A rare clinical entity managed by supraselective embolization and lateral pharygotomy. Indian J Otolaryngol Head Neck Surg 2013;65:95-104.
Ferlito A, Barnes L, Wenig BM. Identification, classification, treatment, and prognosis of laryngeal paraganglioma. Review of the literature and eight new cases. Ann Otol Rhinol Laryngol 1994;103:525-36.
Smolarz JR, Hanna EY, Williams MD, Kupferman ME. Paraganglioma of the endolarynx: A rare tumor in an uncommon location: Head Neck Oncol 2010;2:2.
Hinojar AG, Prieto JR, Munoz E, Hinojar AA. Relapsing paraganglioma of the inferior laryngeal paraganglion: Case report and review of the literature. Head Neck 2002;24:95-102.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]