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CASE REPORT
Year : 2012  |  Volume : 3  |  Issue : 2  |  Page : 113-116

Adenoid cystic carcinoma of the hard palate: A case report with a review of the literature on therapeutic and prognostic aspects


Department of Oral Pathology and Microbiology, Government Dental College, Trivandrum, Kerala, India

Date of Submission02-Aug-2012
Date of Acceptance31-Aug-2012
Date of Web Publication24-Jan-2013

Correspondence Address:
Kanaram Choudhary
B-8, 201, Sidhivinayaka Co-HS, Shantividyanagari, Hatkesh, Mira Road (E), District Thane, Maharastra - 401 107
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0976-6944.106466

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  Abstract 

Salivary gland neoplasms of the head and neck consist of 1-5% of the total malignancies. Adenoid cystic carcinoma (AdCC) is the second most common malignancy of the salivary gland. It is considered as an intermediate grade of salivary gland neoplasms. Here we report a case of AdCC involving the left hard palate, which was initially diagnosed as periapical granuloma. We have also reviewed different treatment options available for AdCC, and prognostic factors as well.

Keywords: Adenoid cystic carcinoma, cylindroma, salivary gland malignancy


How to cite this article:
Choudhary K, Beena V T, Rajeev R, Manju S, Sivakumar R, Padmakaumar S K. Adenoid cystic carcinoma of the hard palate: A case report with a review of the literature on therapeutic and prognostic aspects. Indian J Oral Sci 2012;3:113-6

How to cite this URL:
Choudhary K, Beena V T, Rajeev R, Manju S, Sivakumar R, Padmakaumar S K. Adenoid cystic carcinoma of the hard palate: A case report with a review of the literature on therapeutic and prognostic aspects. Indian J Oral Sci [serial online] 2012 [cited 2017 May 25];3:113-6. Available from: http://www.indjos.com/text.asp?2012/3/2/113/106466


  Introduction Top


Adenoid cystic carcinomas (AdCCs) are rare tumors of the head and neck accounting for approximately 10-15% of malignant tumors of the salivary glands. [1] They are characterized by a rather slow growth pattern and have a perineural spread. The parotid gland is the single most common site of origin (25%) in the head and neck. Most of the AdCCs arise in the minor salivary glands (60%). AdCC of minor salivary gland origin occurs most frequently in the oral cavity (palate). AdCCs arising from the minor salivary glands are often advanced at the time of diagnosis, and complete excision is limited by their large size (with perineural extension involving the cranial base) and the proximity of the tumor to important neural and vascular structures. Standard treatment so far consists of complete surgical resection preferably, followed by adjuvant irradiation in case of close margins, perineural invasion, extensive primary tumor (T3, T4) or high-grade histology. [2],[3],[4],[5]


  Case Report Top


A 56-year-old Indian male reported to the Department of Oral and Maxillofacial Surgery, Government Dental College, Trivandrum, Kerala, India, with a painless swelling of the left maxillary posterior region of six-month duration in November 2010. The size of the swelling was 4 × 3 cm at its greatest dimension [Figure 1]; there was no ulceration. There was a history of the swelling one year back in the same area, as the patient had undergone extraction of 16, 17. The biopsy report then was suggestive of periapical granuloma. The patient was relatively asymptomatic for one month and again gradually developed the swelling that reached the present size. There was a complaint of left nasal blockage spontaneously. There was no other systemic illness including lymphadenopathy. Oral hygiene was poor and the patient was a chronic smoker. Computed tomography (CT) scan showed an inhomogeneously enhancing mass obliterating the left maxillary sinus and infiltrating to the surrounding bone [Figure 2]. From the above investigation, a differential diagnosis of ameloblastoma or minor salivary gland neoplasm was made. Incision biopsy was taken under local anesthesia. A hematoxylin and eosin (H and E) section showed moderately collagenous connective stroma infiltrated by numerous cystic spaces lined by basaloid cells in cribriform pattern. Duct-like areas were filled by eosinophilic coagulum. Basaloid cells demonstrated pleomorphism and occasional mitotic figures [Figure 3]. A diagnosis of AdCC was made. The patient was referred back to the oral and maxillofacial surgery department. The subtotal maxillectomy through modified Weber-Fergusson incision was done under general anesthesia (GA). In the final histopathological examination, the margins were clear and patient has been on continuous follow-up for one year without any evidence of recurrence. For functional and aesthetic rehabilitation of the patient, an obturator was given [Figure 4].
Figure 1: Nonulcerated swelling of left maxillary posterior region (4×3 cm)

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Figure 2: Computed tomography scan shows inhomogenously enhancing mass

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Figure 3: Hematoxylin and eosin section shows basaloid cells arranged in ductal form and with coagulum (×10)

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Figure 4: Postoperative picture with obturator

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  Discussion Top


AdCC is a part of the malignant epithelial tumors of the salivary glands and has been recognized as a specific variant of adenocarcinoma of the salivary and mucous glands since 1853. [6] The term cylindroma, a pathological entity later called 'adenoid cystic carcinoma', was coined by Billroth [7] to describe a salivary gland tumor composed of entwined cylinders of hyaline stroma and epithelial cells, (indeed the illustrations from Billroth's [7] study depict the typical architectural pattern of cylindromas/AdCC, and the cribriform structures with pseudoglands found in AdCC. The term adenoid cystic carcinoma (carcinoma adenoides cysticum) of the salivary glands was first used by Ewing. [8] It usually presents as a slow-growing swelling. Because of the propensity for perineural invasion, pain may be present. [9] In advanced cases, fixation to skin or deeper tissues can occur. [9] Owing to its growth pattern, it should also be noted that at the time of presentation, these tumors have often invaded beyond the clinically apparent borders. [10] AdCC of the salivary gland is a slow-growing but aggressive cancer which is reflected by the good short-term but very poor long-term outcome of patients with this disease. [9] Histologically, AdCC of the salivary glands presents with variable combinations of the three main growth patterns (i.e., cribriform, tubular, and solid) in each individual case. The cribriform is the most characteristic and is almost invariably found, at least focally. [9] Cytologically, the basaloid cells constitute the major cell population, showing mild nuclear pleomorphism and few or no mitoses; in the solid variants, these cells usually show a more pleomorphic appearance and mitoses are more commonly found. [9] Salivary gland AdCCs are graded using a specific three-tier grading system, originally proposed by Szanto et al. in 1984, which is solely based on the main type of growth pattern presenting the tumor. Grade 1 AdCCs are well differentiated and composed of tubular and cribriform patterns without solid components, grade 2 AdCCs are characterized by a pure cribriform pattern or mixed with less than 30% of solid areas, and grade 3 AdCCs are tumors with a marked predominance of the solid pattern. [11] The prognostic factors of AdCCs depend on the site of the tumor, stage of the tumor, the presence of perineural invasion, and grade of the tumor. Tubular and cribriform subtypes have better prognosis than solid subtypes. [12] Many authors described worse prognosis for tumors of the minor salivary glands, due to early local infiltration and invasion of surrounding tissue and bone. [13],[14]

By the use of immunohistocytochemistry, the staining pattern of p53, bcl-2, P-glycoprotein, glutathione S transferase, and topoisomerase, as well as sequencing of p53 were analyzed, due to their proved association with poor prognosis and therapy resistance. [15] These results have demonstrated that p53 alteration is an independent prognostic marker, and its overexpression is known for the association with resistance to radiotherapy and chemotherapy. It has been suggested that those molecules could influence the outcome of new therapeutic approaches. AdCC is known to have aggressive tumor behavior by its ability to invade and metastasize. One such factor regulating these functions is the urokinase-type plasminogen activator and its receptor. The urokinase-type plasminogen activator receptor participates in several normal cellular processes but also influences invasion and metastasis of the tumor by facilitating the destruction of extracellular matrices. Clinically, the cellular expression of urokinase-type plasminogen activator receptor denotes a worse prognosis for many malignancies. So far, in studies of skull base AdCC, urokinase-type plasminogen activator receptor expression seem to be a negative prognostic factor. [16] Other factors showing some preliminary usefulness include the proliferating cell nuclear antigen and c-erbB-2 oncoprotein expression, [17] the transmembrane tyrosine kinase receptor c-Kit (CD117), [18] and the intercellular adhesion molecule-1. [19] However, there is still a need to identify those patients who have a more aggressive AdCC disease to identify them for more novel and possibly experimental therapeutic regimens. The five-year survival is about 60-75%, whereas the 10-year survival drops to 30-54%; most patients eventually die of disease after multiple local recurrences and development of distant metastases (distant metastases are more common than involvement of regional lymph nodes). [20],[21]

The treatment of choice consists of total resection of tumor. [22] However, there is still controversy regarding the adjuvant treatment of this tumor. Several authors recommend postoperative radiation, as radiation often produces regression of the tumor and relieves symptoms. [23],[24],[25] Prokopakis and Kokemueller, doubted that postoperative radiation may influence the course of the disease. [22],[26] The role of chemotherapy in AdCC is controversial. Some authors report it as ineffective, [27] whereas others are more positive and recommend chemotherapy as the palliative treatment in advanced cases of AdCC. [28] Chemotherapy is currently seeking a role in the management of advanced and metastatic tumors of the salivary glands. Recently, a high incidence of mitochondrial mutation in AdCC, a novel finding, has been investigated. [29] Further investigation is warranted to detect the functional implications of these mutations in carcinogenesis. [27] The relevance of c-Kit expression lies in its possible role to direct treatment of nonresectable or metastatic disease, although the use of imatinib mesylate as a tyrosine kinase inhibitor is still controversial. In this respect, there are some contradictory results in the literature, as for example, favorable results have been presented by Alcedo et al.[30] in two cases of unresectable AdCC treated with imatinib mesylate, one for recurrent disease and the other for a locally advanced tumor; however, there are other authors who have observed progression of the disease during treatment with imatinib mesylate. [31] In a study, expression of c-Kit was assessed in seven patients and all were positive; however, activation of c-Kit was not assessed, which would be an important step in deciding whether to treat with tyrosine kinase inhibitors or not, because mutations in 11 c-Kit and 9 c-Kit exons condition an 83.5 and 47.8% response, respectively, in contrast to patients with nondetectable mutation of c-Kit, who do not present an objective response. [32]


  Conclusion Top


AdCC is a common malignancy of major as well as minor salivary glands. Numerous advancements have been made in the treatment of AdCC such as chemotherapeutic agents and radiotherapy; yet, total excision with clear tumor margins is the mainstay of the treatment. Adequate rehabilitation of the patient with proper follow-up is mandatory. As in this case, the initial biopsy was of periapical granuloma, sufficient biopsy samples from the representative site are necessary to rule out any malignant process, lesion or entity.


  Acknowledgments Top


Oral and Maxillofacial Surgery Department, Government Dental College, Trivandrum.

 
  References Top

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



 

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