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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 28  |  Issue : 3  |  Page : 324-328

Clear cell carcinoma of hard palate: A rare case report


1 Department of Oral Medicine and Radiology, Dr. Syamala Reddy Dental College and Hospital, Bengaluru, Karnataka, India
2 Department of Oral Medicine and Radiology, Vydehi Institute of Dental sciences and Research Centre, Bengaluru, Karnataka, India

Date of Submission14-Dec-2015
Date of Acceptance29-Nov-2016
Date of Web Publication13-Dec-2016

Correspondence Address:
Dr. Lekshmy Jayasree
Department of Oral Medicine and Radiology, Dr. Syamala Reddy Dental College and Hospital, Marathahalli, Bengaluru
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-1363.195665

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   Abstract 

Clear cell carcinoma is a rare low-grade carcinoma. It occurs almost exclusively in the intraoral minor salivary glands and represents less than 1% of all salivary gland tumors. It is a new addition in the recent World Health Organization (WHO) classification of salivary gland tumors. The condition follows an indolent course and treatment includes wide surgical excision with or without adjuvant radiotherapy. This case report highlights a very rare case of a 40-year-old female patient with characteristic clinical and radiographic features, diagnosis and management including significant histopathologic features. Combining our case with those reported in literature, a total of 70 cases confirm that clear cell carcinoma is a low-grade malignant neoplasm with distinctive clinical and pathologic features, which highlight the rarity of the condition.

Keywords: Clear cell carcinoma, hard palate, minor salivary gland


How to cite this article:
Jayasree L, Srinivasamurthy P, Jayalekshmi R, Bhat S. Clear cell carcinoma of hard palate: A rare case report. J Indian Acad Oral Med Radiol 2016;28:324-8

How to cite this URL:
Jayasree L, Srinivasamurthy P, Jayalekshmi R, Bhat S. Clear cell carcinoma of hard palate: A rare case report. J Indian Acad Oral Med Radiol [serial online] 2016 [cited 2022 Dec 3];28:324-8. Available from: http://www.jiaomr.in/text.asp?2016/28/3/324/195665


   Introduction Top


Clear cell tumors of the oral mucosa, jaws and salivary glands may be either odontogenic, salivary gland, or metastatic in origin.[1] Minor salivary gland neoplasms are rare, accounting for 9–23% of all salivary gland neoplasms, out of which clear cell carcinoma (CCC) accounts for only 0.2%.[2] In 2005, the term Clear cell Carcinoma Not Otherwise Specified (CCC-NOS) was officially introduced in the third edition of the World Health Organization classification of salivary tumors.[3] The lesion predominantly occurs between the fourth and eighth decades with a slight female predominance. The palate and tongue are the most commonly involved areas intraorally.[4] Moreover, the biologic behavior of this entity is not well characterized because of the limited reported cases and relatively short follow-up.


   Case Report Top


A 40-year-old female patient presented with the chief complaint of swelling on the upper right back tooth region and right middle half of the face since 7 years. Swelling was initially small in size (peanut size) intraorally, which gradually increased and reached the present size. Patient gave a history of extraction of 16 four years ago due to mobility of tooth was uneventful. Both her parents had succumbed to carcinoma. Patient revealed a habit history of cigarette smoking since 10 years, 10–11 times per day.

Extraorally, on inspection [Figure 1], asymmetry was detected as a diffuse swelling on the right side of the face mainly in relation to the middle one-third. Swelling was extending anteriorly from a vertical line dropped from the corner of the mouth to a vertical line from the outer canthus of eye posteriorly, and superiorly extending from the infraorbital margin to a horizontal line connecting the corner of the mouth and right ear lobe. On palpation, swelling was hard in consistency and nontender. Intraorally [Figure 2], a well-defined growth measuring approximately 6 × 7cm in greatest dimension was noted in relation to the right side alveolar ridge and hard palate. Surface of the growth appeared lobulated. Anteriorly, extension was from the distal aspect of 13 till the distal aspect of 18 posteriorly. Medially, the growth was extending from the midline of the palate till the buccal vestibule laterally, with obliteration of the buccal vestibule. Mild erythematous border was noted in relation to the buccal vestibule. Missing 16 was noted. On palpation, growth was firm in consistency and nontender. Grade II mobility was noted in relation to 14 and 18, and grade III mobility in relation to 15 and 17. Based on history and clinical presentation, a provisional diagnosis of benign minor salivary gland tumor in relation to the right posterior maxilla was considered. Differential diagnosis considered was pleomorphic adenoma, monomorphic adenoma, mucoepidermoid carcinoma, and adenocarcinoma.
Figure 1: Extraoral photograph of the patient

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Figure 2: Intraoral photograph of the patient with a solitary growth measuring 6 × 7 cm with lobulated surface and missing 16

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To confirm the diagnosis, radiological investigations were performed [Figure 3], [Figure 4], [Figure 5]. Intraoral periapical radiograph was taken in relation to 14, 15, 17 and 18, which showed missing 16. Severe irregular alveolar bone loss was noted extending till the apical one-third of the root portion giving the tooth a “floating in air” appearance. Displacement of the tooth was noted in relation to 15 and 17 with absence of lamina dura. Maxillary lateral occlusal radiograph revealed areas of severe irregular bone loss. Panoramic radiograph also revealed the same features. To get a more detailed view of the lesion, computed tomography images were obtained [Figure 5]a and [Figure 5]b. Images revealed the presence of a homogenous mass in relation to the maxilla measuring 6 × 7 cm in greatest dimension and extending into the maxillary sinus with severe erosion of the maxillary alveolar ridge. Excisional biopsy was done under general anesthesia with placement of palatal obturator and the gross specimen [Figure 6] was sent for histopathological examination. Hematoxylin and eosin (H and E) stain at 10 × magnification revealed cell clusters with prominent nucleus (hyperchromatic) and eccentrically placed clear cytoplasm [Figure 7]. To further investigate the nature of clear cells, immunohistochemical examination was performed [Figure 8], [Figure 9], [Figure 10]. Immunohistochemistry played an important role in distinguishing the nature of the cells involved. In the present case, the tumor cells were positive for staining with markers such as ck5/6, S-100, and p63; whereas tumour cells were negative for smooth muscle actin (SMA), CD-10 and Vimentin [Table 1].
Figures 3: (a and b) Severe irregular alveolar bone loss appreciated extending till the apical one-third of the root portion giving the tooth a “floating in air” appearance. Displacement of tooth noted in relation to 15 and 17 with absence of lamina dura

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Figure 4: Panoramic radiograph revealing severe irregular alveolar bone loss appreciated extending till the apical one-third of the root portion giving the tooth a “floating in air” appearance

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Figure 5: (a and b) Computed tomography images revealing the presence of homogenous mass in relation to the maxilla measuring 6 × 7 cm in greatest dimension extending into the maxillary sinus with severe erosion of the maxillary ridge

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Figure 6: Tissue specimen after surgical resection

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Figure 7: Hematoxylin and eosin under 10× magnification revealing cell clusters, prominent nucleus (hyperchromatic), eccentrically placed, and clear cytoplasm

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Figure 8: Immunohistochemistry done for CK5/6 shows that cells have taken up the stain confirming epithelial origin

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Figure 9: Immunohistochemistry done for S-.100 shows that cells have taken up the stain confirming neural crest origin

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Figure 10: Immunohistochemistry done for P63 shows that cells have taken up the stain to reveal the proliferative rate

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Table 1: Results of immunohistochemistry

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The above mentioned findings of the histochemical study indicates that the tumor cells were of epithelial and neural crest origin with proliferative rate giving a final diagnosis of low grade CCC of salivary gland origin in relation to the right posterior maxilla. After surgical resection of the lesion [Figure 11]a,[Figure 11]b,[Figure 11]c radiotherapy was planned. Patient was advised follow-up once in 3 months. Unfortunately, after surgical resection, the patient did not turn up for radiotherapy.
Figure 11: Postoperative photographs of (a) extraoral view, (b) intraoral view, and (c) panoramic radiograph

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   Discussion Top


The recognition and consequent reporting of CCC increased significantly after it was first reported by Milchgrub et al. in 1994.[5] Literature review suggests that this lesion occurs almost exclusively in the intraoral minor salivary glands with a prevalence of 0.2–1%, most commonly found in the palate. Females have been reported to be affected twice as often as males.[6] The natural course is an indolent, painless, submucosal mass that occurs predominantly in the minor salivary glands of elderly women.

Microscopically, individual tumor cells are principally characterized by optically clear cytoplasm with well-defined borders and a centrally placed nucleus with minimal nuclear pleomorphism with infiltrative borders.[5],[7],[8] Immunohistochemical evaluation is essential in distinguishing these tumors, however, their sensitivity and specificity is occasionally not sufficient to confirm the diagnosis, which causes a dilemma to specialists.[9] CCC of the salivary glands usually express epithelial markers (cytokeratins, epithelial membrane antigen, and carcinoembroyonic antigen) but lacks expression of myoepithelial markers (S-100 protein, actin, vimentin and glial fibrillary acid protein). CD10 immunoreactivity was observed in 90–94% of the cases of renal CCC, which permits more specificity in the histopathologic diagnosis.[10]

Wide excision is the treatment of choice for most CCC, even though neck dissection and radiotherapy have also been performed in a few cases.[5] The rate of recurrence is 17% and the metastatic rate is 21%. Close follow-up is, therefore, important. [Table 2] lists the previously reported cases of clear cell carcinoma in the past 7 years. Yang et al.,[3] in 2008, reported approximately 65 confirmed cases of CCC.
Table 2: Reported cases of clear cell carcinoma in the past 7 years

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   Conclusion Top


It has become really difficult to determine the biological behavior of this tumor due to a limited number of reported cases, relatively less frequency of the tumor and due to short follow-up periods. Many tumors of the minor salivary glands present with almost similar clinical and histopathological features. Immunohistopathological evaluation has clearly become very important to distinguish between the various forms.

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.[11]

 
   References Top

1.
Saleh KA, Nurishma MI, Firouzeh GN, Goh BS. Primary clear cell carcinoma of minor salivary gland of soft palate: A case report. Med J Malaysia 2012;67:335-6.  Back to cited text no. 1
    
2.
Agrawal J, Kumar YP, Damera DA, Kumar S. Clear cell carcinoma of minor salivary gland: A case of clinical dilemma. Contemp Clin Dent 2014;5:389-92.  Back to cited text no. 2
[PUBMED]  Medknow Journal  
3.
Yang S, Zhang J, Chen X, Wang L, Xie F. Clear Cell Carcinoma, not otherwise specified, of salivary glands: A clinicopathologic study of 4 cases and review of literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008;106:712-20.  Back to cited text no. 3
    
4.
Yamashita K, Kawakami F, Nakashima Y, Murakami K. Clear cell carcinoma of minor salivary gland: An autopsy case with multiple metastases 29 years after the initial surgery and a review of literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009;107:819-25.  Back to cited text no. 4
    
5.
Milchgrub S, Gnepp DR, Vuitch F, Delgado R, Albores-Saavedra J. Hyalinizing clear cell carcinoma of salivary gland. Am J Surg Pathol 1994;18:74-82.  Back to cited text no. 5
    
6.
Ponniah I, Suresh Kumar P, Karunakaran K. Clear cell carcinoma of minor salivary gland- Case report. Ann Acad Med Singapore 2007;36:857-60.  Back to cited text no. 6
    
7.
Simpson RH, Sarsfield PT, Clarke T, Babajews AV. Clear cell carcinoma of minor salivary glands. Histopathology 1990;17:433-8.  Back to cited text no. 7
    
8.
Rezende RB, Drachenberg CB, Kumar D, Blanchaert R, Ord RA, Ioffe OB, et al. Differential diagnosis between monomorphic clear cell adenocarcinoma of salivary glands and renal (clear) cell carcinoma. Am J Surg Pathol 1999;23:1532-8.  Back to cited text no. 8
    
9.
Seijas BP, Franco FL, Sastre RM, García AA, López-Cedrún Cembranos JL. Metastatic renal cell carcinoma presenting as a parotid tumor. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;99:554-7.  Back to cited text no. 9
    
10.
Yang B, Ali SZ, Rosenthal DL. CD10 facilitates the diagnosis of metastatic renal cell carcinoma from primary adrenal cortical neoplasm in adrenal fine-needle aspiration. Diagn Cytopathol 2002;27:149-52.  Back to cited text no. 10
    
11.
Rodríguez MS, Reija MFG, Rodilla IG. Primary clear cell carcinoma of parotid gland: Case report and review of literature. J Oral Maxillofac Pathol 2013;17:101-5.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11]
 
 
    Tables

  [Table 1], [Table 2]


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