Journal of Indian Academy of Oral Medicine and Radiology

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

An unusual swelling in the submandibular region: A case report


Mithare Sangmesh1, Kotya Naik Maloth2, Nagalaxmi Velpula3, Shefali Waghray4,  
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

Correspondence Address:
Kotya Naik Maloth
Senior Lecturer, Department of Oral Medicine and Radiology, Mamata Dental College and Hospital, Khammam - 507 002, Telangana
India

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.



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;26:359-361


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 May 18 ];26:359-361
Available from: https://www.jiaomr.in/text.asp?2014/26/3/359/145034


Full Text

 Introduction



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



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}{Figure 2}{Figure 3}

 Discussion



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



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.

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