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 Table of Contents  
Year : 2020  |  Volume : 8  |  Issue : 2  |  Page : 284-286

Primary small cell carcinoma of tonsil: A rare case with review of literature

1 Department of Pathology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India
2 Department of Medicine, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India

Date of Submission10-Jun-2020
Date of Decision06-Jul-2020
Date of Acceptance07-Jul-2020
Date of Web Publication23-Dec-2020

Correspondence Address:
Dr. Kavita Mardi
Set No. 14, Type VI Quarters, IAS Colony, Meheli, Shimla, Himachal Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/amhs.amhs_131_20

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Small cell neuroendocrine carcinoma (NEC) that originates in the tonsil is extremely rare and carries a poor prognosis. Only a few cases of this tumor have been reported so far. Here, we describe a 60-year-old woman who presented with throat pain and dysphagia for about 3 months. Computed tomographic scan revealed a 4 cm × 2 cm tumor with moderate enhancement in the right tonsil and a neck mass in the right level II. A biopsy of the tonsillar mass was performed, and histologic examination revealed oval-to-elongated tumor cells arranged in sheets, having hyperchromatic nuclei, inconspicuous nucleoli, and scant cytoplasm. Prominent nuclear molding and mitotic figures were readily identified. Immunohistochemical staining showed that tumor cells were strongly positive for synaptophysin, chromogranin, and CK7. A diagnosis of primary small cell NEC of the right tonsil was obtained.

Keywords: Immunohistochemistry, neuroendocrine carcinoma, small cell carcinoma, tonsil

How to cite this article:
Negi L, Mardi K, Chaudhury A, Negi A, Negi R. Primary small cell carcinoma of tonsil: A rare case with review of literature. Arch Med Health Sci 2020;8:284-6

How to cite this URL:
Negi L, Mardi K, Chaudhury A, Negi A, Negi R. Primary small cell carcinoma of tonsil: A rare case with review of literature. Arch Med Health Sci [serial online] 2020 [cited 2022 May 19];8:284-6. Available from: https://www.amhsjournal.org/text.asp?2020/8/2/284/304707

  Introduction Top

Tonsil is one of the most common sites from where primary oropharyngeal tumors originate. Squamous cell carcinoma is the most common tumor of tonsil, while minor salivary tumors, lymphomas, melanoma, and sarcomas also arise from tonsil.[1] Small cell carcinoma is poorly differentiated neuroendocrine tumor, most commonly present in lungs, gastrointestinal tract, and genitourinary system, although it has been reported that they can also occur in many other organs throughout the body.[2] In head-and-neck region, the incidence of primary small cell neuroendocrine carcinoma (NEC) is low. Larynx is the relatively most commonly involved site, followed by salivary glands, nasal cavity, and paranasal sinuses.[3] Small cell carcinomas of the tonsil are extremely rare, and only a few cases were reported since it was first reported by Koss et al. in 1972.[4] We herein report an additional case of small cell carcinoma of the tonsil in a 60-year-old female presenting with enlarged cervical lymph nodes.

  Case Report Top

A 60-year-old female presented with throat pain and dysphagia for 3 months. On examination, there was an ulceroproliferative growth involving whole of the right tonsil with enlarged right cervical lymph nodes. Fine-needle aspiration cytology done from enlarged right cervical lymph node revealed dispersed population of small-to-medium-sized cells having round-to-oval nuclei, fine nuclear chromatin, inconspicuous nucleoli, and scant cytoplasm; nuclear molding, numerous mitotic figures, paranuclear blue inclusions, and streaking artifacts were seen. Possibility of metastatic deposits of small cell carcinoma in the cervical lymph node was suggested [Figure 1]. Biopsy of growth tonsil was done. Microscopic examination revealed tumor cells predominantly arranged in sheets, having oval-to-elongated irregular hyperchromatic nuclei, inconspicuous nucleoli, and scant amount of cytoplasm. Prominent nuclear molding, brisk mitotic figures, and marked crush artifacts were also seen [Figure 1]. These histological features were suggestive of small cell carcinoma.
Figure 1: Photomicrograph of tonsillar biopsy revealing sheets of small hyperchromatic cells with scant cytoplasm in the lamina propria (H and E, ×10)

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Immunohistochemically, tumor cells showed positivity for synaptophysin [Figure 2], chromagranin [Figure 3], and CK7 [Figure 4]. Keeping in view these histopathological and immunohistochemical findings, diagnosis of small cell carcinoma of the tonsil was rendered.
Figure 2: Tumor cells showing positivity for synaptophysin (IHC, ×40)

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Figure 3: Tumor cells showing positivity for chromogranin (IHC, ×40)

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Figure 4: Tumor cells showing positivity for CK7 (IHC, ×40)

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  Discussion Top

NEC is an epithelial malignancy with neuroendocrine differentiation which has been reported in many organs throughout the body. Although the terminology that refers to NECs varies in different anatomic sites and the classification is controversial, NECs are basically divided into two categories: well or poor differentiated. According to the latest WHO Classification of Head and Neck Tumors (2005), typical (Grade I) and atypical carcinoid (Grade II) are well-differentiated NECs, while small cell NEC (Grade III) is classified as poorly differentiated tumor.[5]

Small cell carcinomas are most commonly seen in the lungs and have been reported to occur in some extrapulmonary sites, mainly in the alimentary and genitourinary system.[2] A limited number of reports have described small cell carcinoma arising from the head-and-neck region and show the relative propensity for larynx, followed by salivary glands and sinonasal region.[3] Small cell carcinomas that primarily occur in the tonsil are extremely rare.

Most small cell NECs of the tonsil occurred in patients between 50 and 66 years of age (range, 49–78 years), with a male/female ratio 1.75:1. Cardinal symptoms of the patient of this tumor include throat pain, dysphagia, odynophagia, and progressively enlarging neck mass. The rapid clinical course (from 2 weeks to 3 months) and progressive enlargement of the neck mass may suggest the malignant behavior of this tumor. On examination, growth in the tonsil is usually recognized as asymmetric swelling and ulceration. Computed tomography (CT) and/or magnetic resonance imaging can evaluate the tumor size and its infiltration.

On microscopic examination, hallmarks of small cell NEC includes small round-to-oval cells packed in sheets, cords, or ribbons with hyperchromatic nuclei, sparse cytoplasm, high nuclear/cytoplasmic ratio, and frequent necrosis and mitosis. In immunohistochemistry, cytokeratin and epithelial membrane antigen are immunoreactive, and positive staining of general neuroendocrine markers including synaptophysin, CD56, and chromogranin can provide evidence of neuroendocrine differentiation of tumor cells. The diagnosis of small cell NEC is based on its pathological and immunohistochemical features.

When a diagnosis of extrapulmonary small cell carcinoma arising in the head-and-neck region is made, the patient should be evaluated first to determine whether the disease is a primary or metastatic cancer. Primary small cell carcinoma is rarely occult because it is aggressive and fast growing.[6] In our case, chest and abdominal CT revealed no lesions, and the tonsillar mass was determined to be the primary lesion.

Although the treatment of this tumor has not been defined clearly, it can require surgical excision of the localized tumor, radiation therapy to the primary site, multiple-drug chemotherapy, or a combination of these modalities. The chemotherapeutic regimens for extrapulmonary small cell carcinoma (EPSCC) are similar to those employed in small cell lung carcinoma (SCLC). Platinum-based chemotherapy, such as the use of etoposide (VP-16) and Cisdiamminedichloroplatinum (CDDP), has been recognized as the standard regimen for EPSCC.[7] Recently, CPT-11 combined with CDDP was found to be more effective than VP-16 and CDDP.[8]

  Conclusion Top

Small cell carcinoma of the tonsil is extremely rare and highly aggressive with poor prognosis. With a paucity of studies, standard treatment protocol remains uncertain while radiotherapy combined with chemotherapy seems to be the relative appropriate option.

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

Stambuk HE, Karimi S, Lee N, Patel SG. Oral cavity and oropharynx tumors. Radiol Clin North Am 2007;45:1-20.  Back to cited text no. 1
Jang H, Yuk SM, Kim JO, Han DS. A rare case of primary malignant small cell carcinoma combined with urothelial cell carcinoma in the ureter. World J Surg Oncol 2013;11:181.  Back to cited text no. 2
Renner G. Small cell carcinoma of the head and neck: A review. Semin Oncol 2007;34:3-14.  Back to cited text no. 3
Koss LG, Spiro RH, Hajdu S. Small cell (oat cell) carcinoma of minor salivary gland origin. Cancer 1972;30:737-41.  Back to cited text no. 4
Barnes L. Neuroendocrine tumors. In: Barnes L, Eveson JW, Reichart P, Sidransky D, editors. Pathology and Genetics of Head and Neck Tumors (World Health Organization Classification of Tumors). Lyon: IARC; 2005. p. 135-9.  Back to cited text no. 5
Segawa Y, Nakashima T, Shiratsuchi H, Tanaka R, Mitsugi K, Komune S. Small cell carcinoma of the tonsil treated with irinotecan and cisplatin: A case report and literature review. Case Rep Oncol 2011;4:587-91.  Back to cited text no. 6
Renner G. Small cell carcinoma of the head and neck: A review. Semin Oncol 2007;34:3-14.  Back to cited text no. 7
Noda K, Nishiwaki Y, Kawahara M, Negoro S, Sugiura T, Yokoyama A, et al. Irinotecan plus cisplatin compared with etoposide plus cisplatin for extensive small-cell lung cancer. N Engl J Med 2002;346:85-91.  Back to cited text no. 8


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


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