|Year : 2014 | Volume
| Issue : 3 | Page : 195-198
Adenoid cystic carcinoma of the palate and tongue: Report of two cases
Sumanta Kumar Kolay1, Raman Kant Sinha1, Narendra Nath Singh2, Rajkumar Parwani3
1 Department of Oral and Maxillofacial Pathology, Sarjug Dental College and Hospital, Laheriasari, Darbhanga, Bihar, India
2 Department of Oral Pathology and Microbiology, Kothiwal Dental College and Research Centre, Moradabad, Uttar Pradesh, India
3 Department of Oral and Maxillofacial Pathology, Modern Dental College and Research Centre, Indore, Madhya Pradesh, India
|Date of Web Publication||14-Aug-2014|
Raman Kant Sinha
Gokul Dental Clinic, Moti Mension Appartment, Near Bharat Petrol Pump, Lohiya Nagar, Kankarbagh, Patna - 800 020, Bihar
Source of Support: None, Conflict of Interest: None
Adenoid cystic carcinoma is a rare cancer that arises in major and minor salivary glands with an affinity for nerve invasion. The most common malignancies associated with head and neck and perineural spread are tumors of the minor salivary glands, mucosal and cutaneous origin. However, it is the commonest malignant tumor salivary glands, the palate being the commonest site. A 45-years old female patient reported with an asymptomatic growth over left lateral border of the tongue; since last three months. Computed tomography scan revealed the nature of the growth was malignant. An incisional biopsy was performed. Another 45-years old female patient reported with complaints of pain and swelling over palate since last one month. Clinical examination revealed a large multi-lobulated growth over left side of palate. Provisional diagnosis as a salivary gland neoplasm was given. An incisional biopsy was performed. Histological examination confirmed the diagnosis as adenoid cystic carcinoma of tongue as well as of palate for case one and two respectively and both the case were referred to surgical excision of tumors and advised for periodic follow up. Adenoid cystic carcinoma is a slowly growing but highly invasive cancer with high recurrence rate. Lymphatic spread to local lymph nodes is rare. Pleomorphic adenoma with cribriform pattern and polymorphous low grade adenocarcinoma should be considered in the differential diagnosis since they present similarities in the histological pattern. Surgery is the cornerstone of treatment, and its extension depends primarily on the size of the tumor.
Keywords: Adenoid cystic carcinoma, perineural spread, salivary glands
|How to cite this article:|
Kolay SK, Sinha RK, Singh NN, Parwani R. Adenoid cystic carcinoma of the palate and tongue: Report of two cases. SRM J Res Dent Sci 2014;5:195-8
|How to cite this URL:|
Kolay SK, Sinha RK, Singh NN, Parwani R. Adenoid cystic carcinoma of the palate and tongue: Report of two cases. SRM J Res Dent Sci [serial online] 2014 [cited 2022 May 16];5:195-8. Available from: https://www.srmjrds.in/text.asp?2014/5/3/195/138756
| Introduction|| |
The most common intraoral site for minor salivary gland tumors is the hard palate, followed by the base of the tongue,  whereas the lower lip, retro molar - tonsilar pillar region and sublingual gland are affected less frequently.  Other primary sites are breast, lacrimal glands, lung, and prostate. 
Adenoid cystic carcinoma (ACC) of salivary glands is a rare slow growing malignant tumor, characterized by wide local infiltration and accounts about 1% of all malignant tumors of the oral and maxillofacial region.  It is the second most common malignancy to occur in salivary glands, and the most common malignancy found in minor salivary glands.  Upto 96% of all salivary gland tumors are malignant, and ACC represents about 30% of them. 
We hereby present two cases of a malignant salivary gland tumor (one each in the tongue and palate).
| Case reports|| |
A 45-year-old systemically healthy female[Figure 1]a patient reported to our department, with a complaint of an asymptomatic growth over the tongue, since last 3 months.
|Figure 1: (a) Clinical photograph of the patient. (b) Nodular growth over base of the tongue. (c) Computed tomographic scan shows infiltrating heterogeneous mass near the base of tongue. (d) Stroma reveals the presence of myoepithelial cells and ductal cells having a cribriform pattern|
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On intraoral examination, a firm sessile nodule was present over left posterolateral border of tongue [Figure 1]b; with color appearing as normal surrounding mucosa with mild tender on palpation. Size approximately 2 cm in longest diameter with an indurated border and clinically provisional diagnosis was given as irritational fibroma of tongue and suggested hematological as well as computed tomography to ruled out any malignant nature of the lesion. Hematologic investigations were within normal limit, but computed tomography scan revealed a malignant growth over the base of the tongue [Figure 1]c. For, confirmation an incisional biopsy was performed and microscopically the connective tissue stroma revealed the presence of myoepithelial cells and ductal cells having a cribriform pattern. These cribriform patterns showed epithelial cell nests that form multiple cylindrical psuedocyst like pattern resembling a swiss-cheese or honeycomb appearance suggestive of ACC [Figure 1]d.
A 45-year-old female[Figure 2]a reported to our department with complaints of pain and swelling over the palate since last 1 month. Intraoral examination showed that there was a large growth over the left side of palate [Figure 2]b; size was approximately 4 cm × 3 cm. Surface appeared erythematous, tense with multiple lobulation and tortuous prominent delicate blood vessels. Left submandibular lymph node was tender on palpation and fixed with underlying tissue; size approximately 0.5 cm. Occlusal radiograph shows club shaped radiolucent lesion along the midline with well demarcated borders [Figure 2]c. Provisional diagnosis as a salivary gland neoplasm was given. Routine hematologic investigations were within normal range; an incisional biopsy was performed and histopahtologically connective tissue stroma showed round to cuboidal cells with hyperchromatic nucleus, resembling ductal cells. Some cells were round to oval, with hyperchromatic nucleus, showed plasmacytoid appearance and resembling myoepithelial cells. The tumor cells are arranged in multiple small duct like structures or in solid sheets. Some areas of the tumor mass shows a typical swiss-cheese pattern, with multiple cyst like spaces within solid sheets of tumor cells and diagnosed as ACC [Figure 2]d.
|Figure 2: (a) Clinical photograph of the patient. (b) Large growth over left side of palate. (c) Occlusal radiograph shows club shaped radiolucent lesion along the midline. (d) Tumor cells are arranged in solid sheets with multiple cyst like spaces|
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| Discussion|| |
Adenoid cystic carcinoma, a malignant epithelial neoplasm of salivary gland origin, was originally described by Robin and Laboulbene in 1853 (Tauxe et al. 1962).  The term "cylindroma" was coined by Billroth (1859) due to the tumor's characteristic Swiss-cheese or cribriform appearance.  Spies in 1930 was the first to use the term ACC. 
Adenoid cystic carcinoma is a malignant epithelial tumor characterized by slow growth, late onset of metastasis (mainly to the lungs and bones)  and poor prognosis.  It is noteworthy that ACC can arise in salivary glands and also other mucous glands from different anatomic like the upper aerodigestive tract, eye and skin. In some reports, ACC from other head and neck sites such as the nose, sinuses, nasopharynx and larynx are included.  When located in the tongue, the clinical course of this neoplasm is usually asymptomatic, with gradual submucosal tumoral growth, which hinders an early diagnosis.  Similarly our case, shows asymptomatic lesion over tongue and for the same she had not received treatment for next 3 months.
It most commonly affects adults, with a peak incidence in the 4 th -6 th decades.  Evesson and Cawson found a discreet predominance of ACC cases in women (F:M 1.2:1) with ages varying from 24 to 78 years.  Our cases also shows the similar age and sex distribution.
Presenting signs and symptoms are related to the anatomical site of the lesion.  ACC is frequently associated with pain, which is considered as an early finding in the course of the disease , as ACC presents a strong neurotropism, with a tendency to invade nerves adjacent to the lesion; ,, similar pain complained was also reported by our case number two. Facial pain, parasthesia in trigeminal nerve distribution is commonly reported reflecting the frequency of involvement of Gasserian ganglia, and possibility of perineural spread along the trigeminal nerve. Literature is consistent that the time between the onset of neurological signs and symptoms, and the time of diagnosis range between few months to 3 years. 
It is a slowly growing but highly invasive cancer with high recurrence rate. Lymphatic spread to local lymph nodes is rare but in our case number two shows left submandibular lymph node was tender and fixed to underlying structures, which might be indicating that there was a local spread of malignant cells. Hematogenous spread, however, occurs often in the course of the disease. ,
Radiographically, most reports pertaining to intra-osseous salivary gland tumors appear to describe their presentation as unilocular or multilocular osteolytic radiolucencies as described by Brookstone and Huvos in 1992.  But case two shows a radiolucent bony defect of palate, which may be a localized bony destruction due to pressure or an invasion of the tumor mass or might be the intraoral lesion was a counterpart of intra-osseous lesion.
Various growth patterns and cytological details in ACCs establish the diagnostic criteria for this neoplasm.
Extensive sheets, uniform bands, or cribriform nests usually composed of relatively small, darkly stained, slightly separated basal/myoepithelial cells and small, at times inconspicuous duct like structures, which may contain secretory products. Round to oval, often fairly uniformly sized intercellular spaces, termed pseudocysts, containing pale grayish blue to pinkish granulofibrillar material at times with a reticular pattern, which develop in relation to the basal/myoepithelial cells
Presence of bilayered duct like structures, generally composed of an inner layer of cuboidal to columnar ductal cells with moderate amounts of eosinophilic cytoplasm and outer, smaller darker staining cells.
Arranged as variable, at times fairly uniformly sized groups or as sheets of small, darkly stained tumor cells, those are excess proliferations of the basal/myoepithelial cell component. Small duct like structures must be identifiable among the basaloid cells. Nests or sheets of basaloid cells with the above features form 30% or more of the neoplasm. ,,,,
Most ACCs are composed of a combination of these patterns and the final classification of this neoplasm depends on the predominant type. 
Grading of the tumor
Grade I: The tumor consisting only of cribriform and tubular histomorphology.
Grade II: A mixture of cribriform, tubular and solid growth patterns, with solid growth pattern <30% of the tumor.
Grade III: Tumors with predominantly solid features (>30% or more of the tumor). 
We consider our case one as Grade I tumor because histologically cribriform pattern was predominant whereas case two as Grade III tumor because histologically it shows predominantly solid patterns.
Surgery is the cornerstone of treatment, and its extension depends primarily on the size of the tumor.  Because these tumors frequently exhibit neurologic invasion, postoperative radiation is often recommended, even for Grade I tumors. Radiation therapy is also commonly used when the tumor involves, or is very near, the surgical margins.  On the other hand, chemotherapy use for ACC is controversial. 
The Author would like to thank the patients for providing consent to use their photographs in this article.
| References|| |
|1.||Srivastava S, Jaiswal R, Agarwal A, Singh PK, Singh SN. Cytological diagnosis of adenoid cystic carcinoma of the parotid metastatic to kidney and lung. J Cytol 2007;24:201-2. |
|2.||Jain VS, Singh KK, Sachdeva Y. A case of adenoid cystic carcinoma parotid with perineural spread. Med J Armed Forces India 2007;63:67-8. |
|3.||Goodwin L. Adenoid cystic adenocarcinoma of a minor salivary gland - An under-estimated risk? J Insur Med 2004;36:339-40. |
|4.||Luna-Ortiz K, Carmona-Luna T, Cano-Valdez AM, Mosqueda-Taylor A, Herrera-Gómez A, Villavicencio-Valencia V. Adenoid cystic carcinoma of the tongue - Clinicopathological study and survival analysis. Head Neck Oncol 2009;1:15. |
|5.||Shamim T, Varghese VI, Shameena PM, Sudha S. Primary intraosseous adenoid cystic carcinoma of the mandible with lung metastasis: A case report. J Oral Sci 2008;50:95-8. |
|6.||Khalili M, Salamat F. A 20-year retrospective study of salivary gland adenoid cystic carcinoma in a sample of Iranian patients. J Dent Tehran Univ Med Sci 2009;6:1-5. |
|7.||Abdul-Hussein A, Morris PA, Markova T. An unusual presentation of adenoid cystic carcinoma of the minor salivary glands with cranial nerve palsy: A case study. BMC Cancer 2007;7:157. |
|8.||Chen YK, Chen CH, Lin CC, Hsue SS, Lin YR, Lin LM. Central adenoid cystic carcinoma of the mandible manifesting as an endodontic lesion. Int Endod J 2004;37:711-6. |
|9.||Soares EC, Carreiro Filho FP, Costa FW, Vieira AC, Alves AP. Adenoid cystic carcinoma of the tongue: Case report and literature review. Med Oral Patol Oral Cir Bucal 2008;13:E475-8. |
|10.||Mahajan A, Kulkarni M, Parekh M, Khan M, Shah A, Gabhane M. Adenoid cystic carcinoma of hard palate: A case report. Oral Maxillofac Pathol J 2011;2:127-31. Available from:www.doaj.org/doaj?func=openurl&genre=article&issn=09761225&date=2011&volume=2&issue=1&spage=127. |
[Figure 1], [Figure 2]