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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 34  |  Issue : 1  |  Page : 116-119

Adenoid cystic carcinoma of palate: A unique case report


1 Department of Oral Medicine and Radiology, Haldia Institute of Dental Sciences and Research, Haldia, West Bengal, India
2 Department of Oral Pathology and Microbiology, Haldia Institute of Dental Sciences and Research, Haldia, West Bengal, India

Date of Submission29-Jun-2021
Date of Decision01-Sep-2021
Date of Acceptance27-Nov-2021
Date of Web Publication25-Mar-2022

Correspondence Address:
Dr. Mainak Datta
Department of Oral Medicine and Radiology, Haldia Institute of Dental Sciences and Research, Haldia, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaomr.jiaomr_178_21

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   Abstract 


Adenoid cystic carcinoma (ADCC) is one of the malignant salivary gland neoplasm and constitutes for 1% of head and neck malignancies and 22% of all salivary gland malignancies. Palate is the most common site to be involved in the oral cavity followed by parotid region, tongue, and floor of mouth. It is commonly seen in middle age population with slight female predilection. It usually appears as asymptomatic, slow growing infiltrative growth with high recurrence, often presence of perineural invasion which appears as pain. In this present case of ADCC, a 65-year-old female patient reported with a swelling on the left side of hard palatal area since 3 months with occasional pain and history of epistaxis, which was diagnosed clinically, radiographically, and histopathologically and confirmed by immunohistochemistry as Grade III solid variety of ADCC with a poor prognosis.

Keywords: Cylindroma, minor salivary gland, palatal swelling, perineural invasion, solid variety


How to cite this article:
Datta M, Sinha R, Sarkar S, Shome S. Adenoid cystic carcinoma of palate: A unique case report. J Indian Acad Oral Med Radiol 2022;34:116-9

How to cite this URL:
Datta M, Sinha R, Sarkar S, Shome S. Adenoid cystic carcinoma of palate: A unique case report. J Indian Acad Oral Med Radiol [serial online] 2022 [cited 2022 May 27];34:116-9. Available from: https://www.jiaomr.in/text.asp?2022/34/1/116/340734




   Introduction Top


Adenoid cystic carcinoma (ADCC) is one of the malignant salivary gland neoplasm, first described by Theodar Bilroth in 1856 as “cylindroma.”[1] This clinical entity of carcinoma was termed as ADCC by Ewing in 1954. ADCC accounts for 1% of total head and neck malignancies and 22% of all salivary gland malignancies.[2],[3] It is predominantly encountered in middle age population with slight female predilection. This is a slow-growing tumor, but considered under aggressive one due to its remarkable recurrence capacity. ADCC is seen with three distinct histological pattern namely cribriform, tubular, solid variety and graded accordingly as Grade I, tubular and cribriform areas without solid components; Grade II, cribriform tumors with less than 30% of solid areas; and Grade III, predominantly solid pattern.[4]


   Case Report Top


A 65-year-old female patient reported to department of Oral Medicine and Radiology, HIDSAR with the chief complaint of swelling of left sided middle 1/3rd of face and left half of hard palatal area with occasional pain since last 3 month. The swelling appears as gradually enlarging mass associated with mild occasional pain, which was sudden in onset, dull and radiating in nature, along with a history of epistaxis from left nose over this period. Medical history was non-contributory.

Extraoral examination revealed facial asymmetry present due to presence of swelling of left middle 1/3rd of face [Figure 1]a and [Figure 1]b. Left Submandibular (IB) and Left Upper jugular (IIA) lymph nodes were founded as rounded, nodular, fluctuant, mobile, firm in consistency and non-tender on palpation. Clinical intra-oral examination revealed an ill-defined soft tissue swelling present at left sided posterior 1/3rd of glandular region of hard palate involving left sided gingivo-buccal sulcus (GBS), which is ovoid in shape, measuring around 4 × 6 × 4 cm3 and extended antero-posteriorly from left sided posterior glandular 1/3rd of hard palate, involving left GBS in relation to 23--27 region, crossing medially mid-palatal septum [Figure 1]c. Superficial mucosa appeared slightly erythematous and surrounding mucosa appeared normal. Hard tissue finding reveals multiple missing tooth and multiple retained root stump with a compromised oral hygiene.
Figure 1: (a-c) Front Profile, Lateral Profile of Patient, Intra-oral view of the lesion

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Clinically correlating all features, provisional diagnosis of space occupying aggressive lesion involving left maxillary sinus was given with differential diagnosis as nasopharyngeal carcinoma, sino-nasal carcinoma, spindle cell tumor.

Maxillary cross-sectional occlusal radiograph reveals an ill-defined non-homogenous radiolucency measuring about 4 cm × 6 cm × 6 cm in diameter [Figure 2]a present involving left side of hard palatal floor, extending postero-laterally upto lateral pterygoid plate with few radio-opaque septa. Orthopantomogram (OPG) view reveals an ill-defined non- homogenous radiolucency [Figure 2]b present involving left side of hard palate, involving hard palatal floor and occupying maximum area of left sided maxillary sinus, extending superiorly towards just below the left sided orbital cavity.
Figure 2: (a-e) Maxillary Cross sectional occlusal radiograph; Orthopantomogram (OPG), Para-nasal sinus View (PNS). CECT Showing presence of mass in Coronal and axial section

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PNS view confirms all previous radiological findings [Figure 2]c and showed haziness of involved sinus. CECT of head and neck reveals heterogeneously ill-defined soft tissue hyperdense mass present involving left side of hard palate measuring about 4 × 6 × 6 cm3 in diameter [Figure 2]d and [Figure 2]e, which extends supero-inferiorly compressing left orbital floor, involving lateral pterygoid plate extending upto cranial base- infra-temporal fossa and extended inferiorly upto left half of hard palatal floor. Nasal septum deviated to affected side.

Pre-operative hemogram was within normal limit. Serology tests were non-reactive for HIV-I & II, HBV. Incisional biopsy was done under local anesthesia (LA). Histopathological findings reveals presence of parakeratotic stratified squamous surface epithelium supported by fibro-vascular connective tissue stroma [Figure 4]a and [Figure 4]b. The most confluent feature is the presence of actively invading and proliferating irregular islands, nests and sheets of basaloid cells having isomorphic and deeply basophilic round to angular nuclei. Ductal and epithelial cells are arranged in multiple tubular pattern with few areas of pseudocyst formation. Stromal component is predominantly fibro-vascular in nature intermingled with focal areas of hyalinization. Few areas of hemorrhages and chronic inflammatory cell infiltrates within the connective tissue.
Figure 4: (a and b) Histopathological Slide in 10X and in 40X H/E stained; showing presence of actively invading and proliferating irregular islands, nests and sheets of basaloid cells having isomorphic deeply basophilic round to angular nuclei. (c and d) Immunohistochemistry (IHC) immune positive stained slide for SOX- gene, Calponin

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Multiplanar multi-echo MRI of head and neck region was advised which showed large T1 isointense with few hyperintense area and T2 heterogenous hyperintensity with multiple separation occupying left postero-medial wall of maxillary sinus, and invading towards left infra-temporal fossa. Para-pharyngeal and carotid spaces appeared normal [Figure 3]a.
Figure 3: (a) Showing MRI of head and neck region, showing extension of mass and invading to left infra-temporal fossa. (b and c) 18-F- FDG PET-CT showing no evidence of metabolically active hypersence mass suggestive of no distant metastasis

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18F- Fluoro-deoxyglucose whole body PET-CT was done to rule out any distant metastasis. But there was no evidence of hypermetabolic soft tissue density [Figure 3]b and [Figure 3]c.

Considering clinical, radiographical, histopathological findings, a diagnosis made as Grade III ADCC in left maxillary sinus (Predominantly solid with mixed tubular variety) with three distinct histopathological differential diagnosis as basal cell adenocarcinoma, basaloid Squamous cell carcinoma, adenocystic carcinoma was given.

Full panel immunohistochemistry (IHC) was then done to rule out histopathological differential diagnosis, which confirms it as poorly differentiated ADCC, which showed solid pattern with focal tubular differentiation of 18MF/10HPF [Figure 4]c and [Figure 4]d. The tumor was also immune-positive for SOX-10/Pancytokeratin (AE1/AE 3)/CK14/HMWCK/p40 (focal)/p63 (focal)/EMA/Smooth muscle actin/Calponn (Focal)/Smooth muscle heavy chain (Myosin) and was found immune-negative for CK5 & 6/Synaptophysin, which confirmed it as Grade 3 ADCC on palate with a poor prognosis.

Patient was then referred to Dept. of maxillofacial surgery for evaluation, but no surgery was advised due to age and systemic factors along with maxillary sinus and initiation of middle cranial base involvement and was further sent to department of radio-oncology where radiation therapy started. Intensity modified radiotherapy (IMRT) was started with dosage protocol of 2 gy/day weekly for 5 days for 1 month, and follow-up at 30 days, 60 days, 90 days interval.


   Discussion Top


The ADCC is slow growing, low-graded malignant neoplasm of salivary gland origin, constitutes of approximately 29.6% of all minor salivary gland tumors. Most commonly seen in palate but also can be seen at tongue, floor of mouth, lip also. Rarely, it may also present as primary intra-osseous tumors involving jaws.[5] ADCC is mainly seen in middle aged population in between 40 and 60 years of age with a female predominance. Protein produced by the fusion of two transcription factor genes, MYB-NFIB, plays an important role in the development of ADCC tumors. Point mutations in the NOTCH-1 gene are also thought to be associated with ADCC.[6]

Most frequent clinical feature of ADCC affected major salivary gland is presence of slow growing tumor, usually 2--4 cm at its greatest diameter. The lesion is generally non-capsulated and invasive to surrounding tissue structure. Pain is common, striking clinical findings due to its perineural invasion property. Signs of facial nerve paralysis can be seen, where ADCC involves the parotid region. Distant metastasis generally occurs mainly in lung and bones. Direct extension of lesion of the base of skull shows poorest prognosis.[7]

Diagnostic section includes gold standard diagnostic test---biopsy for histopathological evaluation. Besides the radiographic examination including orthopantomogram, CT scan of head and neck region, MRI are important diagnostic tools to assess the exact extension of lesion. Early invasive low-grade tumors tend to be well-defined, in contradistinction to high-grade tumors, which appear infiltrative to surrounding tissue.[8] It frequently associated with perineural spread (via cranial nerve VII), which is well appreciated on MRI. Specialized imaging tests are performed to determine possible infiltration of regional lymph nodes and the presence of distant metastases. Genetic sequence analysis of MYB-NFIB are emerging diagnostic tool till date.[9]

Histopathologically, there are three common patterns seen as cribriform, tubular, and solid; among which cribriform is the most common one, in which epithelial cells are arranged in multiple cylindrical spaces, shows a pseudo cystic appearance, which often contain a hyalinized material. The tubular type also shows ductal pattern, with presence of one or two layers of cells similar to the myoepithelial cells. The solid variant generally present solid epithelial islands with central areas of necrosis; ductal cells are basophilic and hyperchromatic with a densely granulated nucleus and few mitotic figures. The solid type shows poor prognosis contrary to the cribriform type.[9]

SOX genes (SRY-related HMG-box genes) that is immune positive for ADCC in IHC, family of transcription factors that bind at the minor groove in DNA, and belong to a super-family genes characterized by a homologous sequence are called the HMG box (for high mobility group). This HMG box is a DNA binding domain that is highly conserved throughout eukaryotic species.[10]

After confirming the diagnosis, by correlating the clinical features with radiographical and histopathological findings , in most of the cases wide margin surgical resection is the treatment of choice along with post-surgical radiotherapy with or without chemotherapy.[10] Important prognostic factors include primary lesion size (T), anatomical localization, presence or absence of nodal metastasis (M) at diagnosis time, perineural invasion and the histopathology grading (G).[9] Long-term follow-up of patients with salivary gland neoplasms is mandatory because of the frequently inert but often surrounding invasive behavior associated with late loco-regional recurrence and distant metastasis of adenoid cystic carcinoma with nodal involvement.[10]


   Conclusion Top


Salivary gland tumors should be considered in the differential diagnosis of aggressive lesions in maxilla and mandible and especially when the aggressive lesion is involving palate, ADCC involving minor salivary gland tumors should be considered in differential diagnosis. The primary treatment objective in ADCC patients is early control with maintenance of normal functionality and prevention of distant nodal metastasis. For this purpose, early detection of the lesion is very important, in order to enable a more favorable prognosis and better quality of life.

Declaration of patient consent

The authors certify that they have obtained all appropriate consent forms from patient. In the form, the patient(s) has given his consent for images and other clinical information to be reported in the journal. The patient had understood that his personal information will not be published.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Matsuba HM, Spector GJ, Thawley SE, Simpson JR, Mauney M, Pikul FJ. Adenoid cystic salivary gland carcinoma: A histomorphologic review of treatment failure patterns. Cancer 2015;67:519-24.  Back to cited text no. 1
    
2.
Spiro RH, Huvos AG, Strong EW. Adenoid cystic carcinoma: Factors influencing survival. Am J Clin Pathol 2014;138:579-83.  Back to cited text no. 2
    
3.
Kim HK, Sung MW, Chung PS, Rhee CS, Park CI, Kim WH. Adenoid cystic carcinoma of the head and neck. Arch Otolaryngol Head Neck Surg 2012;120:721-6.  Back to cited text no. 3
    
4.
Gnepp DR, Henley JD, Roderick HW, Simpson, Eveson J. Salivary and lacrimal glands. In: Gnepp Douglas R, editor. Diagnostic Surgical Pathology of the Head and Neck. 2nd ed. Philadelphia: Saunders; 2019. p. 482-6.  Back to cited text no. 4
    
5.
Szanto PA, Luna MA, Tortoledo ME, White RA. Histologic grading of adenoid cystic carcinoma of the salivary glands. Cancer 2014;54:1062-9.  Back to cited text no. 5
    
6.
Tarpley TM Jr, Giansanti JS. Adenoidcystic carcinoma-analysis of fifty oral cases. Oral Surg Oral Med Oral Pathol 2002;41:484-9.  Back to cited text no. 6
    
7.
Triantafillidou K, Dimitrakopoulos J, Iordanidis F, Koufogiannis D. Management of adenoid cystic carcinoma of minor salivary glands. J Oral Maxillofac Surg 2016;64:1114-20.  Back to cited text no. 7
    
8.
Waldron CA, El-Mofty SK, Gnepp DR. Tumors of the intraoral minor salivary glands: A demographic and histologic study of 426 cases. Oral Surg Oral Med Oral Pathol 2018;66:323-33.  Back to cited text no. 8
    
9.
Huang M, Ma D, Sun K, Yu G, Guo C, Gao F. Factors influencing survival rate in adenoid cystic carcinoma of the salivary glands. Int J Oral Maxillofac Surg 2016;26:435-9.  Back to cited text no. 9
    
10.
Ellis GL, Auclair PL, Gnepp DR. Surgical Pathology of Salivary Glands. Otolaryngology–Head and Neck Surgery, 1995;112:352-3.  Back to cited text no. 10
    


    Figures

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



 

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