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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 26  |  Issue : 3  |  Page : 359-361

An unusual swelling in the submandibular region: A case report


1 Departments of Oral Medicine and Radiology, HKDET's Dental College and Hospital, Humnabad, Karnataka, India
2 Mamata Dental College and Hospital, Khammam, Telangana, India
3 Sri Sai College of Dental Surgery, Vikarabad, Telangana, India
4 Panineeya Mahavidhyalaya Institute of Dental Sciences and Research Centre, Hyderabad, Telangana, India

Date of Submission12-Aug-2014
Date of Acceptance12-Nov-2014
Date of Web Publication19-Nov-2014

Correspondence Address:
Kotya Naik Maloth
Senior Lecturer, Department of Oral Medicine and Radiology, Mamata Dental College and Hospital, Khammam - 507 002, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-1363.145034

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   Abstract 

Adenoid cystic carcinoma (ACC) is a slow-growing tumor of the salivary gland with high malignant potential, most commonly involving the minor salivary glands of the head and neck region. It is known for its long clinical course, indolent growth, local recurrence, and late distant metastasis. We report an unusual case of ACC in an adult male patient which remained undiagnosed for more than 15 years.

Keywords: Adenoid cystic carcinoma, carcinoma of palate, pleural metastasis


How to cite this article:
Sangmesh M, Maloth KN, Velpula N, Waghray S. An unusual swelling in the submandibular region: A case report . J Indian Acad Oral Med Radiol 2014;26:359-61

How to cite this URL:
Sangmesh M, Maloth KN, Velpula N, Waghray S. An unusual swelling in the submandibular region: A case report . J Indian Acad Oral Med Radiol [serial online] 2014 [cited 2022 Jan 26];26:359-61. Available from: https://www.jiaomr.in/text.asp?2014/26/3/359/145034


   Introduction Top


Adenoid cystic carcinoma (ACC), also known as cylindroma, was first described by Theodar Billroth in 1859. [1] The term "cylindroma" was widely used till 1953, when Foote and Frazell renamed the lesion as "adenoid cystic carcinoma." [2] It is also known as adenocystic carcinoma, adenocystic basal cell carcinoma, pseudo-adenomatous basal cell carcinoma, and basaloid mixed tumor. [3] Adenoid cystic carcinoma is a malignant tumor that arises from the secretory epithelial cells of the salivary glands of the head and neck, and accounts for less than 10% of all salivary neoplasms. [4] Although ACC has been classified with salivary gland tumors, in some cases it may arise from other primary sites such as skin, breast, cervix, prostate gland, external auditory canal, nasopharynx, lacrimal gland, vulva, esophagus, and trachea. [5] Approximately 31% of the lesions affect minor salivary glands, particularly in the palate. [6] Among the major salivary glands, the submandibular gland is most commonly involved. [5] These tumors have slight female predilection (female:male ratio is 3:2). [7] Peak incidence is predominantly between fifth and sixth decades of life. [8] The characteristic feature of ACC is slow growth, multiple local recurrences, and a prolonged clinical course with often a delayed development of distant metastasis, [9] and there is a propensity of perineural invasion. [3] A usual feature of ACC is the low incidence of metastasis to the regional lymph nodes. [10] Lung is the most common site of metastasis, followed by the liver. Bone metastasis usually indicates fulminant clinical course. [5] Studies have shown chromosomal abnormalities and genetic deletions occurring in ACC, and there are evidences of inactivation of p53 tumor suppressor genes in advanced and aggressive form of this neoplasm. No specific abnormality underlying this disease process is known. [11]


   Case Report Top


A 32-year-old male patient reported to the Department of Oral Medicine and Radiology with the chief complaint of swelling in the right neck region since 15 years and pain since 1 month [Figure 1]. The patient first noticed the swelling 15 years back when it was peanut sized, which had progressively increased to the present size. Mild, continuous pain was associated with the swelling since 1 month that aggravated with application of pressure. The patient did not report any discharge from the swelling or the use of medication for tuberculosis. On extraoral examination, a diffuse, roughly oval swelling was seen in the right submandibular region, measuring approximately 3 × 4 cm in size. The overlying and surrounding skin was normal. The swelling extended anteroposteriorly 1 cm posterior to the chin up to 1 cm anterior to the angle of mandible. Superoinferiorly, it extended 1 cm below the inferior border of mandible toward the submandibular area up to a distance of 2 cm (inferiorly). No visible pulsations were seen. On palpation, the swelling was firm, mobile, tender, non-compressible, non-reducible, and non-fluctuant, and no pulsations were felt. Lymph nodes were not palpable. No abnormalities were found in relation to the teeth and soft tissues on intraoral examination. However, on bimanual palpation of the right submandibular area, a tender and firm swelling was noted. Based on the clinical features, a provisional diagnosis of chronic lymphadenitis of the right submandibular region was made, and tubercular lymphadenitis, benign lymphoid hyperplasia, lymphoma, sialadenitis, pleomorphic adenoma, and ACC were included under the differential diagnoses.

The orthopantomograph and mandibular occlusal radiograph revealed no relevant findings. Fine needle aspiration cytology (FNAC) was suggestive of pleomorphic adenoma. Ultrasonography revealed a hypoechoic lesion which was noted in the right submandibular region with two adjacent lymph nodes. The right submandibular gland was excised [Figure 2] and the specimen was sent for histopathological examination. Histopathological examination revealed cribriform growth pattern displaying several prominent pseudocysts surrounded by basaloid cells with hyperchromatic angulated nuclei, suggestive of ACC of the right submandibular gland [Figure 3]. Postoperatively, the patient was subjected to radiotherapy. Initially, 45 Gy was given over a period of 25 days. Then 18 Gy was given over a period of 10 days, sparing the spinal cord. The patient is under follow-up since 2 years, and there is no recurrence reported till date and the prognosis has been good.
Figure 1: Photograph showing swelling in the right submandibular region

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Figure 2: Intraoperative photograph showing surgical excision of the right submandibular gland

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Figure 3: Photomicrograph showing numerous islands of highly basaloid epithelial cells containing multiple cylindric cyst-like spaces resembling characteristic Swiss cheese appearance

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   Discussion Top


Salivary gland ACC is a slow-growing malignant tumor which has a prolonged clinical course and delayed development of distant metastasis. [1] The age of the patient reported here is 32 years, which is in contrast to the usual peak incidence of ACC, that is between 50 and 60 years. The growth of the tumor was slow and asymptomatic in the present case as seen in the other reported cases in literature. Lymph node involvement was not noted in this case, which is similar to the cases reported by Allen and Marsh. [10] In a study done by Sung et al., the disease-specific survival rates were noted to be 80%, 58%, and 48% at 5, 10, and 15 years, respectively. [12] Spiro reported that one-third of the patients with ACC were found to have distant metastasis during the follow-up period without any evidence of loco-regional failure. [13] Tumor size >3 cm, loco-regional recurrence, and cervical node involvement were reported to be highly predictive of distant metastasis. [13] The median survival period after the diagnosis of distant metastasis was 38 months, and was reported to be in the range of 15-70 months. [13] Three growth patterns for ACC have been described: Cribriform, tubular, and solid. The cribriform subtype is the most frequent. It is composed of islands of basaloid cells surrounding variably sized cyst-like spaces forming a "Swiss cheese" or sieve-like pattern. [3] The case reported here was of the cribriform subtype. Treatment modalities for ACC include surgical therapy, radiotherapy, chemotherapy, and combined therapy. [2] Surgery has been the mainstay of treatment for ACC. In the case reported here, excision of the entire right submandibular gland was done. Patients with high-grade carcinomas as well as those with positive margins usually receive adjuvant postoperative radiotherapy. [7] In the case reported here, the surgery was followed up with radiotherapy with a good prognosis.


   Conclusion Top


Although ACC has an indolent behavior, is slow growing, rarely shows any symptoms, and does not cause much discomfort to the patient, its potential for local recurrence and distant metastasis must be considered, and prompt treatment with regular long-term follow-up of the patient is necessary.

 
   References Top

1.Spiro RH, Huvos AG, Strong EW. Adenoid cystic carcinoma of salivary origin. A clinicopathologic study of 242 cases. Am J Surg 1974;128:512-20.  Back to cited text no. 1
    
2.Al-Sukhun J, Lindqvist C, Hietanen J, Leivo I, Penttilä H. Central adenoid cystic carcinoma of the mandible: Case report and literature review of 16 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;101:304-8.  Back to cited text no. 2
    
3.Shah JP, Patel SG, Singh B. Salivary gland tumors. In: Jatin Shah's Head and Neck Surgery and Oncology. 4 th ed. Philadelphia: Mosby publishers; 2012. p. 526-69.  Back to cited text no. 3
    
4.Matsuba HM, Spector GJ, Thawley SE, Simpson JR, Mauney M, Pikul FJ. Adenoid cystic salivary gland carcinoma: A histopathologic review of treatment failure patterns. Cancer 1986;57:519-24.  Back to cited text no. 4
    
5.Sharma K, Rathi AK, Khurana N, Mukherji A, Kumar V, Singh K, et al. A retrospective study of 18 cases of adenoid cystic cancer at a tertiary care centre in Delhi. Indian J Cancer 2010;47:424-9.  Back to cited text no. 5
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6.Chundru NS, Amudala R, Thankappan P, Nagaraju CD. Adenoid cystic carcinoma of palate: A case report and review of literature. Dent Res J 2013;10:274-8.  Back to cited text no. 6
[PUBMED]  Medknow Journal  
7.Mendenhall WM, Morris CG, Amdur RJ, Werning JW, Hinerman RW, Villaret DB. Radiotherapy alone or combined with surgery for adenoid cystic carcinoma of the head and neck. Head Neck 2004;26:154-62.  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 1988;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 1997;26:435-9.  Back to cited text no. 9
    
10.Allen MS Jr, Marsh WL Jr. Lymph node involvement by direct extension of adenoid cystic carcinoma: Absence of classic embolic lymph node metastasis. Cancer 1976;38:2017-21.  Back to cited text no. 10
    
11.Kiyoshima T, Shima K, Kobayashi I, Matsuo K, Okamura K, Komatsu S, et al. Expression of p53 tumor suppressor gene in adenoid cystic and mucoepidermoid carcinomas of the salivary glands. Oral Oncol 2001;37:315-22.  Back to cited text no. 11
    
12.Sung MW, Kim KH, Kim JW, Min YG, Seong WJ, Roh JL, et al. Clinicopathologic predictors and impact of distant metastasis from adenoid cystic carcinoma of the head and neck. Arch Otolaryngol Head Neck Surg 2003;129:1193-7.  Back to cited text no. 12
    
13.Spiro RH. Distant metastasis in adenoid cystic carcinoma of salivary origin. Am J Surg 1997;174:495-8.  Back to cited text no. 13
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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