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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 33  |  Issue : 3  |  Page : 340-343

Adenoid cystic carcinoma of submandibular salivary gland: A case report with extensive management


1 Department of Oral Medicine and Radiology, Ragas Dental College and Hospital, Chennai, Tamil Nadu, India
2 Department of Oral Medicine and Radiology, Rajah Muthiah Dental College and Hospital, Annamalai University, Chidambaram, Tamil Nadu, India
3 Department of Oral Pathology and Microbiology, Sree Balaji Dental College and Hospital, Chennai, Tamil Nadu, India
4 Department of Oral Medicine and Maxillofacial Radiology, Tagore Dental College and Hospital, Chennai, Tamil Nadu, India

Date of Submission12-Jan-2021
Date of Decision28-Jun-2021
Date of Acceptance13-Jul-2021
Date of Web Publication28-Sep-2021

Correspondence Address:
Dr. N Narmatha
Department of Oral Medicine and Radiology, Tagore Dental College and Hospital, Chennai, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaomr.jiaomr_11_21

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   Abstract 


Adenoid cystic carcinoma (ACC) is a relatively rare epithelial malignant tumor of the major and minor salivary glands with deceptive benign histologic appearance characterized by indolent, locally invasive growth with a high propensity for local recurrence and distant metastasis. Typical ACC has a cribriform pattern, presenting a very aggressive course among the histologic variants. According to the Armed Forces Institute of Pathology (AFIP), ACC was considered the fifth most common malignant epithelial tumor of the salivary gland. ACC is more common in the minor salivary gland, the palate being the most common site, submandibular salivary gland and least followed by sublingual and parotid glands. ACC accounts for about 1% of all malignant tumors of the oral and maxillofacial regions. Thus, considering its rarity, we would like to discuss a case report of ACC of the submandibular salivary gland in a 52-year-old female patient with a typical histological pattern, which would epitomize all cases of ACC along with extensive management.

Keywords: Adenoid cystic carcinoma, cribriform pattern, submandibular gland


How to cite this article:
Aparna P V, Venkatesh J, Sankari S L, Narmatha N. Adenoid cystic carcinoma of submandibular salivary gland: A case report with extensive management. J Indian Acad Oral Med Radiol 2021;33:340-3

How to cite this URL:
Aparna P V, Venkatesh J, Sankari S L, Narmatha N. Adenoid cystic carcinoma of submandibular salivary gland: A case report with extensive management. J Indian Acad Oral Med Radiol [serial online] 2021 [cited 2021 Nov 29];33:340-3. Available from: https://www.jiaomr.in/text.asp?2021/33/3/340/326879




   Introduction Top


According to the WHO histological classification of tumors of salivary glands, adenoid cystic carcinoma (ACC) is under malignant epithelial tumors.[1] ACC was first described by Theodor Billroth in 1856. Initially, it was termed cylindromas based on the histological features, and it has been changed as adenoid cystic carcinoma by Kaiser.[2] It has been entrenched that cribriform pattern is the most common among histologic variants of ACC.[3] The exact etiology of ACC has not been got yet in the literature. Recurrent chromosomal rearrangements characterized adenoid cystic carcinoma.

MYB overexpression was clear in the ACC pathogenesis. Though not clear in all cases, overexpression of transcription factors from the family of MYB genes plays a vital role in the pathogenesis of ACC.[4],[5],[6] Thus, we are presenting a case report of adenoid cystic carcinoma along with management.

Patient information

A 52-year-old female patient visited the department of oral medicine and radiology with the chief complaint of painless swelling in the lower front tooth region for the past 2 months. History of presenting illness revealed swelling increased slowly to reach the present size and shape Timeline of history presented in [Figure 1]. Medical history was noncontributory. Personal history revealed no adverse oral habits.
Figure 1: Timeline of history

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Clinical description

The patient's general examination revealed no abnormality. Vital signs were normal, moderately built, and nourished. Extra-oral examination revealed a double chin appearance with no other signs of systemic involvement. Intraoral examination showed a moderate amount of calculus and stains. Hard tissue examination showed missing 31, 41. Upon soft tissue examination, growth was evident at the mandibular alveolar mucosa concerning the lingual aspect of edentulous 31, 32, and from dentate 34 to 43 region [Figure 2]. The growth was well-circumscribed in nature with no secondary changes, measuring 5 × 3 cm, extending mesio distally from the distal aspect of 34 to the distal aspect of 43. On palpation, the growth was non-tender, hard in consistency, fixed to the underlying structures. Bucco lingual obliteration was evident in the anterior mandibular region. On lymph nodes examination, a single right submandibular lymph node measuring approximately 1 × 1 cm was present. The lymph node was non-tender, hard in consistency, and fixed to the underlying structure.
Figure 2: Intraoral examination revealing swelling in relation to the lingual aspect of edentulous 31, 32 and from dentate 34 to 43 region

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Diagnostic assessment

Occlusal radiograph revealed buccolingual resorption in relation to edentulous space of 32, 42 with floating of 42, 43, 32 with only apical bone remaining [Figure 3]. Ortho pantomography revealed diffuse radiolucency involving the symphysial region extending from 34 to 44 with an intact inferior border of the mandible. Loss of lamina dura with resorption in the apical one-third roots of 42 and 43 [Figure 4]. Axial CT revealed a homogeneous hypodense cystic lesion with buccal cortical expansion and perforation extending from 33 to 43 [Figure 5]. The reconstructive image showed the perforation of the buccal cortex in the anterior mandibular region [Figure 6]. Based on the history, clinical presentation, and radiographic features, the differential considerations included major or minor salivary gland malignancy.
Figure 3: Occlusal radiograph reveals buccolingual resorption in relation to edentulous space of 32, 42

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Figure 4: OPG reveals diffuse radiolucency from 34 to 44 region, loss of lamina dura with resorption in the apical one-third roots of 42 and 43

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Figure 5: Axial CT reveals homogeneous hypodense cystic lesion with buccal cortical expansion and perforation extending from 33 to 43

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Figure 6: Reconstructive image shows perforation of buccal cortex in the anterior mandibular region

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Therapeutic intervention

The patient was managed with anterior mandibular segmental resection along with left submandibular and sublingual gland excision and reconstructed with stainless steel reconstruction plates and intermaxillary fixation for occlusal stability [Figure 7]. The excised specimen was sent for histopathological examination. Histopathology revealed a cribriform architectural growth pattern with the presence of basaloid and hyperchromatic neoplastic cells, suggestive of adenoid cystic carcinoma [Figure 8].
Figure 7: Anterior mandibular segmental resection with stainless steel reconstruction plates

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Figure 8: Histopathology reveals a cribriform architectural growth pattern

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Follow-up and outcome

The patient is under follow-up for 6 months, and there is no recurrence reported to date, and the prognosis has been good.


   Discussion Top


ACC accounts for about 1% of all malignant tumors of the oral and maxillofacial regions and 10%–15% of malignant tumors of salivary glands.[7] More common in the fifth and sixth decades of life and among females.[8] The most common site is minor salivary glands, submandibular, and sublingual salivary glands.[3] Our case was reported in the fifth-decade female, among the most common site, submandibular salivary glands. Apart from genetic background, ACC is thought to arise from the structures derived from the foregut (i.e.) parotid, submandibular and sublingual glands, and mucus glands throughout the upper respiratory tract.[9] These tumors are difficult to diagnose at the early stage because of their indolent growth, rendering it to be asymptomatic in most cases.[10] Our case was diagnosed at the early stage because of the interference in speech and difficulty in mastication co-existing with edentulous 31, 41. Considering the three histologic variants of ACC – tubular, cribriform, solid type – “cribriform pattern” is the most common type[7] and gives the entire structure a typical “swiss-cheese” appearance. It is moderately differentiated and has a good prognosis compared to the solid variant, which is poorly differentiated. ACC is characterized by a propensity of perineural invasion, a high frequency of local and distant recurrence, and a poor long-term prognosis.[11] Our case presented with the most common type of cribriform histologic pattern and rendered a good prognosis. Prognosis is also attributed to the site; the more favorable outcome is with major salivary gland ACC because of its early recognition. There was no history of perineural invasion on clinical examination. Management includes surgical excision, chemotherapy, and radiotherapy. Our case underwent wide surgical excision along with minor salivary glands removal to prevent a recurrence. The underlying principles in all ACC therapy are that tumor cells extend well beyond the clinical or radiographic margins. Therefore, it requires excision with the widest margins possible.

Strength and limitations

Our case was diagnosed at the early stage as these tumors are difficult to diagnose at the early stage, presented with the most common type of cribriform histologic pattern and rendered a good prognosis. Extensive diagnostic assessment such as MRI was not performed and considered as one limitations in our study.


   Conclusion Top


Adenoid cystic carcinoma has an indolent nature and a good survival rate in case of early recognition with no evidence of distant metastasis. Complete follow-up is needed to avoid recurrences in the future. Thus, considering its rarity, dentists should be cognizant of the prevalence of adenoid cystic carcinoma and its role in distant metastasis.

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.
Hellquist H, Skalova A. Histopathology of Salivary Glands. Berlin, Heidelberg: Springer; 2014. p .221-60.  Back to cited text no. 1
    
2.
Dutta NN, Baruah R, Das L. Adenoid cystic carcinoma-clinical presentation and cytological diagnosis. Indian J Otolaryngol Head Neck Surg 2002;54:62-4.  Back to cited text no. 2
    
3.
Pushpanjali M, Sujata DN, Subramanyam SB, Jyothsna M. Adenoid cystic carcinoma: An unusual presentation. J Oral Maxillofac Pathol 2014;18:286-90.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Uraizee I, Cipriani NA, Ginat DT. Adenoid cystic carcinoma of the oral cavity: Radiology– pathology correlation. Head Neck Pathol 2018;12:562-6.  Back to cited text no. 4
    
5.
Wysocki PT, Izumchenko E, Meir J, Ha PK, Sidransky D, Brait M. Adenoid cystic carcinoma: Emerging role of translocations and gene fusions. Oncotarget 2016;7:66239-54.  Back to cited text no. 5
    
6.
Persson M, Andren Y, Mark J, Horlings HM, Persson F, Stenman GV. Recurrent fusion of MYB and NFIB transcription factor genes in carcinomas of the breast and head and neck. Proc Natl Acad Sci USA 2009;106:18740-4.  Back to cited text no. 6
    
7.
Spiro RH. Salivary neoplasms: Overview of a 35-year experience with 807 patients. Head Neck Surg 1986;8:177-84.  Back to cited text no. 7
    
8.
Chummun S, McLean NR, Kelly CG, Dawes PJ, Meikle D, Fellows S, et al. Adenoid cystic carcinoma of the head and neck. Br J Plast Surg 2001;54:476-80.  Back to cited text no. 8
    
9.
Jones AS, Hamilton JW, Rowley H, Husband D, Helliwell TR. Adenoid cystic carcinoma of the head and neck. Clin Otolaryngol Allied Sci 1997;22:434-43.  Back to cited text no. 9
    
10.
Monteiro D, Lino J, Bernardo T, Fernandes J, Monteiro E. Adenoid cystic carcinoma of the head and neck: Epidemiology andpredictors of prognosis. Int J Head Neck Surg 2013;2:165-73.  Back to cited text no. 10
    
11.
Ganly I, Amit M, Kou L, Palmer FL, Migliacci J, Katabi N, et al. Nomograms for predicting survival and recurrence in patients with adenoid cystic carcinoma. An international collaborative study. Eur J Cancer 2015;51:2768-76.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

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