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Year : 2008  |  Volume : 51  |  Issue : 1  |  Page : 63-64
Small-cell neuroendocrine tumor of larynx: A rare presentation

1 Department of Pathology, GSVM Medical College, Kanpur, Uttar Pradesh, India
2 Department of Otorhinolaryngology, GSVM Medical College, Kanpur, Uttar Pradesh, India

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Neuroendocrine tumors of the larynx are extremely rare and represent <1% of all primary laryngeal tumors. We report a case of a 55-year-old man who presented to us with laryngeal tumor, histopathological evaluation of which revealed it to be a neuroendocrine tumor. Diagnosis is based mostly on light microscopy and may be supported by special histochemical stains in some instances.

Keywords: Malignancy, neck, gastrointestinal tract, chromogranin A

How to cite this article:
Dhingra M, Agarwal A, Kaushik S, Singh S N. Small-cell neuroendocrine tumor of larynx: A rare presentation. Indian J Pathol Microbiol 2008;51:63-4

How to cite this URL:
Dhingra M, Agarwal A, Kaushik S, Singh S N. Small-cell neuroendocrine tumor of larynx: A rare presentation. Indian J Pathol Microbiol [serial online] 2008 [cited 2022 May 18];51:63-4. Available from: https://www.ijpmonline.org/text.asp?2008/51/1/63/40401

   Introduction Top

Gastrointestinal tract is the commonest site for the occurrence of neuroendocrine tumors. However, larynx is an extremely uncommon site for these tumors and the diagnosis can be established only by histopathology.

   Case History Top

A 55-year-old man, a known smoker for more than 30 years, presented with unexplained weight loss of about 10 kg over 2 months and change in voice for 2 weeks. Suspecting malignancy, an indirect laryngoscopy was performed which revealed a growth in the supraglottic area involving arytenoid and part of left aryepiglottic fold. Chest X-ray was normal. Light microscopy of the biopsy from the growth revealed small round to oval cells with large vesicular nucleus and prominent nucleolus [Figure - 1]. These cells were highly suspicious of metastatic small round cell tumor/neuroendocrine tumor. Immunohistochemistry confirmed neuroendocrine origin by chromogranin (CgA) positivity [Figure - 2]. Evaluation of gastrointestinal tract for any evidence of tumor origin was negative. Later, the patient developed ipsilateral cervical lymph node enlargement, fine needle aspiration cytology of which also confirmed small cell origin.

   Discussion Top

Our patient presented with the features suggestive of small laryngeal tumor with cervical lymph node involvement. Histopathological evaluation revealed a malignancy that has been rarely described at this site. [1] Neuroendocrine tumors primarily affect the gastrointestinal tract. They are classified according to their site of origin and may be gastric carcinoids or pancreatic neuroendocrine tumors.

Neuroendocrine tumor of larynx is an extremely rare disease entity characterized by pathological heterogeneity. The World Health Organization categorizes these tumors as typical carcinoids, atypical carcinoids, small-cell neuroendocrine carcinomas, and paragangliomas. However according to a recent report, [2] the classification proposed by Wening and Gnepp [3] of well differentiated, moderately differentiated, and poorly differentiated tumors is more uniform and helpful for prognostication and treatment of such patients. [2],[3] Small-cell neuroendocrine carcinoids are further categorized as oat-cell, intermediate-cell, or combined-cell types. Light microscopy is usually diagnostic [4] but in some instances must be supported by immunohistochemistry which reveals positivity for either epithelial (cytokeratin) or neuroendocrine markers (CgA and neuron-specific enolase). [4] Current diagnosis and staging of neuroendocrine tumors are significantly improved by the introduction of the CgA assay in plasma or serum as a tumor marker, and by the use of somatostatin receptor scintigraphy (SRS) for tumor localization. The SRS proved to be more sensitive than CgA, with equivalent specificity. The plasma CgA level is related to tumor secretory activity. [5] Paragangliomas are the only laryngeal tumors with female preponderance. [4] They are generally seen as vascular submucosal masses in supraglottis or even in subglottis. Typical carcinoids are very rare. However, surgery is generally curative and lymph node dissection is not required because metastasis is rare. Atypical carcinoids form the most common form of nonsquamous cell carcinoma of larynx. Surgery with lymph node metastasis is required because of high likelihood of metastasis.

Small-cell neuroendocrine tumors of larynx are still rare. These are aggressive neoplasms characterized by diffuse early metastasis. Chemotherapy and radiotherapy are the best form of palliation. [6] Surgery for this tumor has been disappointing and is reserved for cases of local relapse with no evidence of metastasis. Small-cell neuroendocrine carcinomas are not cured by local excision, and these patients have the worst prognosis (5-year survival of 5%). [7]

   References Top

1.Procopio G, Ricotta R, Fusi A, Celio L De Dosso S, Catena L, et al . Neuroendocrine tumors of the larynx: A clinical report and literature review. Tumori 2006;92:72-5.  Back to cited text no. 1    
2.Hamid O, Fiky LE, Arab LEE, Beltagy YE, Amin R. Small cell carcinoma of the larynx: a case report. Otolaryngol Head Neck Surg 2005;133:1-3.  Back to cited text no. 2    
3.Wening BM, Gnepp DR. The spectrum of neuroendocrine carcinomas of the larynx. Semin Diag Pathol 1989;6:329-50.  Back to cited text no. 3    
4.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.  Back to cited text no. 4  [PUBMED]  
5.Moisa II. Neuroendocrine tumors of the larynx. Head Neck 1991;13:498-508.  Back to cited text no. 5  [PUBMED]  
6.Cimitan M, Buonadonna A, Cannizzaro R, Canzonieri V, Borsatti E, Ruffo R. Somatostatin receptor scintigraphy versus chromogranin A assay in the management of patients with neuroendocrine tumors of different types: clinical role. Ann Oncol 2003;14:1135-41.  Back to cited text no. 6    
7.Cuzzourt JC, Pezold JC, Dunn CW. Typical carcinoid tumor of the larynx occurring with otalgia: a case report. Ear Nose Throat J 2002;81:40-3.  Back to cited text no. 7  [PUBMED]  

Correspondence Address:
M Dhingra
Department of Pathology, GSVM Medical College, Kanpur, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.40401

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  [Figure - 1], [Figure - 2]

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