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Table of Contents
Year : 2019  |  Volume : 6  |  Issue : 1  |  Page : 30-32

Primary small-cell carcinoma of the glottic larynx: A case report from Northern Pakistan

Department of Oncology, Atomic Energy Cancer Hospital, NORI, Islamabad, Pakistan

Date of Submission18-Apr-2018
Date of Decision07-Sep-2018
Date of Acceptance16-Sep-2018
Date of Web Publication1-Mar-2019

Correspondence Address:
Dr. Hadia Fatima
Department of Oncology, Atomic Energy Cancer Hospital, NORI, Islamabad
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JCRP.JCRP_2_18

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Extrapulmonary neuroendocrine small-cell carcinoma is a rare tumor accounting for only 2.5%–4% of all small-cell carcinomas, and during the past 30 years, only 160 cases of primary small-cell carcinoma of the larynx have been reported worldwide. Most previously reported cases were metastatic small-cell carcinomas. Herein, we report the case of a 51-year-old male who was treated at the Atomic Energy Cancer Hospital, NORI, Islamabad, Pakistan for small-cell carcinoma of the larynx.

Keywords: Cisplatin, etoposide, larynx, small-cell carcinoma

How to cite this article:
Awan K, Mahmood H, Fatima H, Arif S, Faheem M. Primary small-cell carcinoma of the glottic larynx: A case report from Northern Pakistan. J Cancer Res Pract 2019;6:30-2

How to cite this URL:
Awan K, Mahmood H, Fatima H, Arif S, Faheem M. Primary small-cell carcinoma of the glottic larynx: A case report from Northern Pakistan. J Cancer Res Pract [serial online] 2019 [cited 2022 Oct 3];6:30-2. Available from: https://www.ejcrp.org/text.asp?2019/6/1/30/253245

  Introduction Top

Approximately, 99% of the laryngeal tumors are squamous-cell carcinomas.[1] Laryngeal tumors are mostly found in the region of the glottis or true vocal cords. A neuroendocrine tumor of the larynx was first described in 1969 by Goldman et al.,[2] and neuroendocrine tumors in the form of atypical carcinoids have since been identified as common nonsquamous cancers that arise from the larynx. Primary Laryngeal small cell carcinoma (LSCC) accounts for <0.5% of all laryngeal neoplasms.[3] Herein, we describe a case of localized primary small-cell carcinoma (LSCC) that was diagnosed on histopathology supported by immunohistochemistry. The patient was treated with a combination of external beam radiotherapy and chemotherapy, with an excellent response to the treatment.

  Case Report Top

A 51-year-old male patient with hypertension which was controlled by medication and no family history of any malignancy presented with the complaints of hoarseness of voice for the past 8 months associated with an occasional cough but not accompanied with bloodtinged discharge. His swallowing and dietary habits were normal. The patient had smoked a hukka and cigarettes for the past 20 years. There was no palpable cervical lymphadenopathy. A fiber-optic endoscopic examination revealed a left vocal cord lesion involving almost the whole-vocal cord. Vocal cord mobility was intact. Multiple biopsies were taken from the growth, which showed the features of small-cell cancer, including a high nuclear-to-cytoplasmic ratio with scant cytoplasm, round-to-oval cells, and a large number of mitotic figures [Figure 1]. Immunohistochemistry was positive for cytokeratin, synaptophysin, and CAM 5.2 [Figure 2].
Figure 1: Biopsy showing features of small-cell carcinoma

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Figure 2: Immunohistochemical stain positive for synaptophysin

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A computed tomography (CT) scan with contrast from the base of his skull to the clavicles showed subtle thickening at the anterior part of his left vocal cord, but the other findings were unremarkable [Figure 3]. His CT scan of the chest, abdomen, and bone was normal [Figure 4], and hence the disease was nonmetastatic. The diagnosis of primary small cell of the larynx was confirmed. The stage was T1aN0M0 (Stage 1a).
Figure 3: Pretreatment computed tomography scan of the neck showing left vocal cord irregularity and thickening

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Figure 4: Prechemotherapy computed tomography scan of the chest showing normal findings

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He was treated with external beam radiotherapy (55 Gy with 2.75 Gy/fraction in 20 fractions) followed by six cycles of chemotherapy (cisplatin 75 mg/m2 on D1 and etoposide 100 mg/m2 D1–d3). Six months after completing treatment, there was no evidence of local or regional recurrence in a postradiotherapy CT scan.

  Discussion Top

Laryngeal cancers are mostly squamous-cell carcinomas. Laryngeal small-cell neuroendocrine tumors are a rare malignancy with aggressive clinical behavior. Neuroendocrine small-cell carcinomas are usually observed in males (3:1 male-to-female ratio) with a median age at diagnosis ranging from 50 to 70 years.[4] Our patient was male and 51 years old.

Heavy cigarette smoking is the most important risk factor involved in the etiology of such tumors.[5] In addition to cigarette smoking, our case also had a history of hukka smoking. Common presenting symptoms include dysphonia, dysphagia, or in some cases a neck mass.[1] Our patient presented with dysphonia and hoarseness of voice.

Although this rare tumor has been reported in various locations in the larynx, the supraglottic region is the most frequently involved site.[4] In our patient, the site of origin was the left vocal cord. Primary small-cell carcinoma of the larynx usually presents as locally advanced or metastatic disease.[6] Our patient presented with localized disease in his left vocal cord.

Small-cell carcinoma may be immunoreactive for cytokeratins and for general neuroendocrine markers, including chromogranin, CD 56, and synaptophysin.[6],[7] In our case, immunohistochemical studies were positive for cytokeratin, synaptophysin, and CAM 5.2.

Systemic chemotherapy should be used to treat laryngeal small cell carcinoma, as there is a possibility of occult metastasis in cases presenting with localized disease. Moreover, the surgery should be avoided because of the poor prognosis and the morbidity associated with surgery of the larynx.[8],[9] Hence, organ preservation with radiotherapy combined with adjuvant or concurrent chemotherapy should be the prime objective in the treatment of laryngeal small cell carcinoma.[10] Our patient was treated with external beam radiotherapy 55 Gy with 2.75 Gy/fraction for 20 days followed by adjuvant chemotherapy with cisplatin 75 mg/m2 D1 and etoposide 100 mg/m2 D1–D3 in 3 weekly protocols for six cycles.

The prognosis of patients with primary laryngeal small cell carcinoma is considered to be poor with a 5-year survival rate of only 5%.[8] Although patients with localized early-stage disease can have prolonged survival, but there is a possibility of treatment resistance.[8] Our patient had a complete response to the treatment, and he has been free from disease for the 6-month follow-up visit.

  Conclusion and Recommendation Top

A diagnosis of early-stage primary small-cell carcinoma of the larynx is extremely rare, and histopathologists should keep in mind of this rare diagnosis when evaluating the laryngeal carcinomas. Treatment of localized laryngeal small cell carcinoma with external beam radiotherapy and chemotherapy can result in a satisfactory response and can be considered to be the standard treatment for early-stage laryngeal small cell carcinomas.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Jaiswal VR, Hoang MP. Primary combined squamous and small cell carcinoma of the larynx: A case report and review of the literature. Arch Pathol Lab Med 2004;128:1279-82.  Back to cited text no. 1
Goldman NC, Hood CI, Singleton GT. Carcinoid of the larynx. Arch Otolaryngol 1969;90:64-7.  Back to cited text no. 2
Gnepp DR. Small cell neuroendocrine carcinoma of the larynx. A critical review of the literature. ORL J Otorhinolaryngol Relat Spec 1991;53:210-9.  Back to cited text no. 3
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. 4
Ferlito A, Rinaldo A. Primary and secondary small cell neuroendocrine carcinoma of the larynx: A review. Head Neck 2008;30:518-24.  Back to cited text no. 5
Ferlito A, Silver CE, Bradford CR, Rinaldo A. Neuroendocrine neoplasms of the larynx: An overview. Head Neck 2009;31:1634-46.  Back to cited text no. 6
Mikić A, Zvrko E, Trivić A, Stefanović D, Golubović M. Small cell neuroendocrine tumor of the larynx – a small case series. Coll Antropol 2012;36 Suppl 2:201-4.  Back to cited text no. 7
Renner G. Small cell carcinoma of the head and neck: A review. Semin Oncol 2007;34:3-14.  Back to cited text no. 8
Pointer KB, Ko HC, Brower JV, Witek ME, Kimple RJ, Lloyd RV, et al. Small cell carcinoma of the head and neck: An analysis of the national cancer database. Oral Oncol 2017;69:92-8.  Back to cited text no. 9
Hatoum GF, Patton B, Takita C, Abdel-Wahab M, LaFave K, Weed D, et al. Small cell carcinoma of the head and neck: The university of Miami experience. Int J Radiat Oncol Biol Phys 2009;74:477-81.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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