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CASE REPORT
Year : 2022  |  Volume : 18  |  Issue : 3  |  Page : 804-806

Spindle cell carcinoma masquerading as a benign polyp of the soft palate


1 Department of Pathology, Kasturba Medical College, Mangalore, Karnataka, India
2 Department of Otorhinolaryngology and Head and Neck Surgery, Kasturba Medical College, Mangalore, Karnataka, India
3 Department of Radiation Oncology, Kasturba Medical College, Mangalore, Karnataka, India

Date of Submission12-May-2020
Date of Decision29-Aug-2020
Date of Acceptance20-Oct-2020
Date of Web Publication15-Jun-2022

Correspondence Address:
K Kshithi
Department of Otorhinolaryngology and Head and Neck Surgery, Kasturba Medical College Hospital, Attavar, Mangalore - 575 001, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcrt.JCRT_617_20

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 > Abstract 


Spindle cell carcinoma of the head and neck is a rare biphasic neoplasm. The presentation mimics other head-and-neck malignancies and hence the diagnosis hinges upon histopathological confirmation along with positive immunohistochemistry (IHC) markers denoting the presence of both epithelial and mesenchymal components. At present, there are no standard management criteria for these tumors with the options varying from surgery alone to surgery combined with adjuvant radiotherapy. We discuss here the case of a patient presenting with an oropharyngeal mass that had benign clinical features and the final diagnosis of spindle cell carcinoma could only be established after histopathology with IHC typing.

Keywords: Biphasic tumor, head-and-neck malignancy, oropharynx, Spindle cell carcinoma


How to cite this article:
Kini JR, Dosemane D, Kshithi K, Banerjee S. Spindle cell carcinoma masquerading as a benign polyp of the soft palate. J Can Res Ther 2022;18:804-6

How to cite this URL:
Kini JR, Dosemane D, Kshithi K, Banerjee S. Spindle cell carcinoma masquerading as a benign polyp of the soft palate. J Can Res Ther [serial online] 2022 [cited 2022 Aug 10];18:804-6. Available from: https://www.cancerjournal.net/text.asp?2022/18/3/804/347679




 > Introduction Top


Spindle cell carcinoma of the head and neck is a rare biphasic neoplasm[1] with reported incidences ranging from 1% to 3% in the head-and-neck region. It consists of squamous cell carcinoma and malignant spindle cell component with a mesenchymal appearance but epithelial origin.[2] The presentation is commonly as a polypoidal exophytic growth. The diagnosis is difficult and often requires immunohistochemistry (IHC) to demonstrate the epithelial and mesenchymal components. Management of these aggressive tumors is not standardized and often includes surgery or a combination of complete surgical excision followed by adjuvant radiotherapy.[3]


 > Case Report Top


A 65-year-old male patient with no comorbidities presented with difficulty and pain on swallowing of 2 months. There was also a history of cough with whitish expectoration and a sticking sensation in the throat. The patient also complained of a change in voice and cough while drinking water in addition to the regurgitation of food. The patient had been consuming alcohol, smoking beedis, and chewing tobacco for the past 25 years. As the patient described his symptoms, it was noted that he had the characteristic hot potato voice. On examination, there was a 4 cm × 3.5 cm globular mass seen behind the posterior aspect of the tongue, hanging from the soft palate [Figure 1]. The overlying mucosa was intact and there was no ulceration and the mass did not bleed on touch. Indirect laryngoscopy was done which revealed the mass as a pedunculated growth arising from the soft palate. Laryngeal structures were normal. A contrast-enhanced computed tomography scan was obtained which showed heterogeneously enhancing mass in the oropharynx with internal vascularity [Figure 2]. There were level IB and II lymph nodes measuring 6–10 mm on the left and right level IB node measuring 11 mm.
Figure 1: Preoperative photograph showing the mass in the oropharynx arising from the soft palate

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Figure 2: Contrast-enhanced computed tomography scan coronal (a) and axial (b) sections demonstrating the heterogeneously enhancing mass in the oropharynx and its extent

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The patient underwent wide excision of the mass with 1 cm margin in all directions under general anesthesia. Intraoperatively, there was a 4 cm × 3.5 cm globular proliferative mass arising from the lateral aspect of the uvula on the left side with pedicular attachment to the soft palate and left tonsil posterior pillar. The mass was excised based on the pedicle using electrocautery. Mucosal layer covering left tonsil pillar, soft palate, and right tonsil pillar were stripped using electrocautery leaving behind muscle layer [Figure 3]. The mass was sent for histopathological examination. The neck was not addressed surgically since the procedure was an excision biopsy to ascertain the diagnosis.
Figure 3: Postoperative photograph after complete excision of the tumor

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On histopathology, there was a polypoidal mass lined by ulcerated stratified squamous epithelium with underlying tumor tissue composed of pleomorphic spindle cells arranged in intersecting fascicles and whorls. These tumor cells had a high N: C ratio, pleomorphic vesicular nuclei, prominent eosinophilic nucleoli, and scanty cytoplasm. Numerous bizarre cells and multinucleated tumor giant cells were also present. Numerous mitotic figures including atypical forms were seen. The stroma around the tumor showed hyalinization, desmoplasia, and chronic inflammatory infiltrate. Multiple repeat sections revealed a focus of invasive well-differentiated squamous cell carcinoma [Figure 4]. IHC revealed strong positivity with vimentin and focal positivity with cytokeratin and p63 [Figure 5]. The final diagnosis of spindle cell (squamous) carcinoma was rendered. All margins were negative for tumor.
Figure 4: (a) Histopathological slide showing malignant squamous epithelium with keratin pearls. (b) Tumor showing spindle cell morphology. High power (×40) view in the inset

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Figure 5: (a) Immunohistochemistry showing vimentin positivity in the spindle cell area. (b) Immunohistochemistry showing cytokeratin positivity in the spindle cell area

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Following recovery, the patient was referred for radiotherapy and he received a complete course of adjuvant radiotherapy (total dose of 70 Gray in 35 fractions over seven weeks). The patient is on regular follow-up and during each visit, he is clinically and endoscopically evaluated for any evidence of recurrence.


 > Discussion Top


Spindle cell carcinomas are rare biphasic neoplasms. They are poorly differentiated variants of squamous cell carcinomas.[4] These neoplasms are seen more often in males commonly in the fifth and sixth decades of life.[2] Cigarette smoking, alcohol abuse, and previous irradiation to the head-and-neck region have been implicated as risk factors.[4] In the current case, the patient had a history of smoking, alcohol use, and tobacco chewing for the past 25 years.

Spindle cell carcinomas commonly present as polypoidal exophytic growth[4],[5] with surface ulceration and bleeding on touch. However, our patient presented with a benign-appearing mass without any ulceration or bleeding. In addition, since the tumor was situated in the oropharynx, the patient also had a characteristic hot potato voice. The top differentials based on clinical presentation were benign lesions such as myoepithelioma, fibrovascular polyp, and rhinosporidiosis.

The diagnosis of spindle cell carcinoma relies on a proper histopathological evaluation of the lesion to demonstrate the presence of epithelial component along with the spindle cells which represent the mesenchymal component. Various theories have been proposed to the presence of the mesenchymal component within the tumor out of which the monoclonal origin theory is the most accepted. This theory proposes that the epithelial and mesenchymal components arise monoclonally from a single stem cell and the spindle cell component arises due to a de-differentiation of the epithelial cell. Histopathology alone may not always be sufficient to confirm the diagnosis of spindle cell carcinoma and IHC is often required to make a final diagnosis. This is especially of note when multiple sections from the tumor fail to reveal coexistent areas of invasive squamous cell carcinoma even a focus with a squamous pearl or any residual overlying dysplastic epithelium. In such instances, a panel of immunohistochemical markers may be essential for a definitive diagnosis. Vimentin and cytokeratin coexpression in the neoplastic cells play a key role in the definite diagnosis.[5],[6] Multiple IHC epithelial markers such as cytokeratin, EMA, AE1/AE3, HMWCK, p63, and p40 may be essential to prove the epithelial/squamous differentiation in these tumors. Markers for mesenchymal differentiation include vimentin and variable expression of mesenchymal filaments such as SMA, desmin, and S-100.[7],[8] In our case, apart from the focus of well-differentiated squamous cell carcinoma, the IHC was positive for cytokeratin and vimentin, hence proving the presence of both epithelial and mesenchymal components.

Spindle cell carcinomas are aggressive tumours[9] and the main treatment options are either surgery alone or surgery followed by adjuvant radiotherapy.[3],[10] In our patient, we performed complete resection of the tumor with an adequate margin of clearance. Postoperatively, the patient received a complete course of adjuvant radiotherapy. The disease progression is characterized by recurrences and metastasis[10], and hence regular follow-up is essential to detect recurrences early.


 > Conclusion Top


Spindle cell carcinomas are rare but aggressive neoplasms and hence accurate diagnosis is crucial to provide optimal treatment plan for the patient. IHC markers may need to be performed to confirm the diagnosis whenever spindle cells are visualized on histopathology. The management plan should include surgery and radiotherapy due to the aggressive nature of the tumor and the patient needs to be informed about the prognosis and importance of regular follow-ups to detect recurrence and metastasis early.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 > References Top

1.
Virchow R. Die Krankhaften Geschwulste. Berlin, Germany: Hirschwald; 1863. p. 181-2.  Back to cited text no. 1
    
2.
Viswanathan S, Rahman K, Pallavi S, Sachin J, Patil A, Chaturvedi P, et al. Sarcomatoid (spindle cell) carcinoma of the head and neck mucosal region: A clinicopathologic review of 103 cases from a tertiary referral cancer centre. Head Neck Pathol 2010;4:265-75.  Back to cited text no. 2
    
3.
Iqbal MS, Paleri V, Brown J, Greystoke A, Dobrowsky W, Kelly C, et al. Spindle cell carcinoma of the head and neck region: Treatment and outcomes of 15 patients. Ecancermedicalscience 2015;9:594.  Back to cited text no. 3
    
4.
Romañach MJ, Azevedo RS, Carlos R, de Almeida OP, Pires FR. Clinicopathological and immunohistochemical features of oral spindle cell carcinoma. J Oral Pathol Med 2010;39:335-41.  Back to cited text no. 4
    
5.
Thompson LD, Wieneke JA, Miettinen M, Heffner DK. Spindle cell (sarcomatoid) carcinomas of the larynx: A clinicopathologic study of 187 cases. Am J Surg Pathol 2002;26:153-70.  Back to cited text no. 5
    
6.
Gupta R, Singh S, Hedau S, Nigam S, Das BC, Singh I, et al. Spindle cell carcinoma of head and neck: An immunohistochemical and molecular approach to its pathogenesis. J Clin Pathol 2007;60:472-5.  Back to cited text no. 6
    
7.
Silva DFB, Santos HBP, León JE, Gomes DQC, Alves PM, Nonaka CFW. Clinicopathological and immunohistochemical analysis of spindle cell squamous cell carcinoma of the tongue: A rare case. Einstein (Sao Paulo) 2019;17:eRC4610.  Back to cited text no. 7
    
8.
Shah BJ, Tupkari JV, Joy T. Sarcomatoid squamous cell carcinoma of mandible: A report of two cases. J Oral Maxillofac Pathol 2019;23:163.  Back to cited text no. 8
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9.
Dai L, Fang Q, Li P, Liu F, Zhang X. Oncologic outcomes of patients with sarcomatoid carcinoma of the hypopharynx. Front Oncol 2019;9:950.  Back to cited text no. 9
    
10.
Spector ME, Wilson KF, Light E, McHugh JB, Bradford CR. Clinical and pathologic predictors of recurrence and survival in spindle cell squamous cell carcinoma. Otolaryngol Head Neck Surg 2011;145:242-7.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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