Aggressive adenoid cystic carcinoma involving palate extending to maxillary sinus in a young female

Santosh R Patil; Abdalwahab M Alzwiri Zwiri; Krishna A Rao; Ravi Kumar Gudipaneni; Kuraym Khalid Alenazi

doi:10.4103/2249-4987.182484

CASE REPORT

Year : 2016 | Volume : 8 | Issue : 1 | Page : 20-22

Aggressive adenoid cystic carcinoma involving palate extending to maxillary sinus in a young female

Santosh R Patil¹, Abdalwahab M Alzwiri Zwiri¹, Krishna A Rao², Ravi Kumar Gudipaneni³, Kuraym Khalid Alenazi⁴
¹ Department of Oral Medicine and Radiology, College of Dentistry, AlJouf University, Sakakah, Aljouf, Saudi Arabia
² Department of Oral Pathology, College of Dentistry, AlJouf University, Sakakah, Aljouf, Saudi Arabia
³ Department of Pedodontics, College of Dentistry, AlJouf University, Sakakah, Aljouf, Saudi Arabia
⁴ Department of Intern, College of Dentistry, AlJouf University, Sakakah, Aljouf, Saudi Arabia

Date of Web Publication

16-May-2016

Correspondence Address:
Santosh R Patil
Department of Oral Medicine and Radiology, College of Dentistry, AlJouf University, Sakakah, Aljouf
Saudi Arabia

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/2249-4987.182484

Abstract

Adenoid cystic carcinoma (ACC) is a rare malignant tumor that affects the head and neck region. It is a malignant tumor arising from minor salivary glands, palate being the most common intraoral location, usually affecting individuals of the fifth decade. This tumor generally has a slow growth rate, and it is often present for several years before the patient seeks treatment. We report a case of extensive ACC involving palate, extending to a maxillary sinus in a young lady.

Keywords: Adenoid cystic carcinoma, cylindroma, minor salivary glands, prognosis

How to cite this article:
Patil SR, Alzwiri Zwiri AM, Rao KA, Gudipaneni RK, Alenazi KK. Aggressive adenoid cystic carcinoma involving palate extending to maxillary sinus in a young female. J Oral Res Rev 2016;8:20-2

How to cite this URL:
Patil SR, Alzwiri Zwiri AM, Rao KA, Gudipaneni RK, Alenazi KK. Aggressive adenoid cystic carcinoma involving palate extending to maxillary sinus in a young female. J Oral Res Rev [serial online] 2016 [cited 2022 Jun 27];8:20-2. Available from: https://www.jorr.org/text.asp?2016/8/1/20/182484

Introduction

Adenoid cystic carcinoma (ACC) is considered as a rare malignancy of the head and neck, which constitutes about 2–4% of all head and neck tumors and nearly 10% of all salivary gland malignancies.^[1] ACC was first described by Theodor Billroth in 1856, he termed it as “cylindroma” because of its characteristic histological features, later the term ACC was designated by Foote and Frazell.^[2] Even though it is commonly seen involving the minor salivary glands, cases involving the major salivary glands have also been reported in the literature. Involvement of nose and paranasal sinuses (PNSs) has also been noted apart from salivary glands. Nearly, 50% of all intraoral ACCs are reported to be involving the palate. A 1:1 sex ratio is commonly noted with slight more inclination towards female population.^[3] The purpose of this paper is to report a case of ACC, which was progressing rapidly with the involvement of maxillary sinus in a young female.

Case Report

A 22-year-old lady reported to Department of Oral medicine and Radiology of Jodhpur Dental College and Hospital in June 2013 with a chief complaint of ulceroproliferative growth over the palatal region. The patient noticed a small swelling 1 month back which rapidly progressed into a large mass. There were no associated symptoms and patient did not give any significant medical history. Extraoral examination revealed a nontender diffuse swelling over the left maxillary region. Intraorally, a well-defi ned swelling was noted on the left side of the hard palate, measuring about 2 cm × 2 cm with a center area of ulceration [Figure 1]. The swelling was firm and nontender. There was no evidence of paresthesia or motor nerve deficiency. Regional lymph nodes were not clinically palpable. With these observations, a provisional diagnosis of ACC and a differential diagnosis of necrotizing sialometaplasia and mucoepidermoid carcinoma was arrived at. Orthopantomograph revealed discontinuity of the lower border of the left maxillary sinus, mesial displacement of 35, over retained 63, and impacted 33 [Figure 2]. PNS X-ray view revealed diffuse radiopacity in the left maxillary sinus and destruction of the lateral wall [Figure 3]. Chest posteroanterior X-ray, ultrasound abdomen did not recognize any distant organ involvement. An incisional biopsy was performed under local anesthesia. The tissue was submitted for histopathological examination which revealed features of solid type of ACC [Figure 4]. The patient was treated with left hemimaxillectomy and adjuvant radiotherapy. The patient was advised for follow-up, every 6 months.

Figure 1: Ulcerated swelling

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Figure 2: Cropped orthopantomograph showing destruction of the floor of maxillary sinus

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Figure 3: Paranasal sinus X-ray showing radiopacity of left maxillary sinus

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Figure 4: Photomicrograph of the H and E stained tissue (×10), showing cribriform pattern

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Discussion

ACCs are the most common malignant neoplasm of the lacrimal gland, and the second most common type of carcinoma arising in the salivary glands, following mucoepidermoid carcinoma.^[4] Even though ACC is well known for its prolonged clinical course, but in the present case, a very short-term course has been observed. The peak incidence of ACC is noted in the fifth and sixth decade but in the present case, a young female has been affected.^[5] There are no universally accepted agreed upon predisposing factors for the development of ACC, and there seems to be no family tendency. Patients characteristically present with a gradually enlarging mass that, because of its indolent growth pattern, can be quite large.^[6] The tumor usually lack symptoms, nonpainful but bone invasion or perineural spread leading to pain or hypoesthesia may be seen in some cases. Majority of these tumors are locate at submucosal level, appearing as smooth, domed swellings without ulceration of the overlying mucosa.^[7] However, in our case, the mucosal tissue overlying the swelling was ulcerated. The tumors are usually encapsulated and are well circumscribed; this feature may be misleading as these tumors present an insidiously infiltrative growth pattern. Three different types of histological growth patterns were noted in relation with this tumor which includes cribriform, tubular, and solid type. Distant metastases and infiltration into the regional lymph nodes is noted in <10% of cases. Even though lungs are recognized as the organs of propensity for distant metastases, spread to bone, brain, and hepatic tissues have been also been noted in few cases.^[8] The prognosis of ACC is dependent on multiple factors, and the overall 5-year survival rate is 47%. Advanced imaging modalities such as computed tomography and magnetic resonance imaging are considered beneficial in the diagnosis of this tumor, also to assess the actual location and extension and to plan the surgical treatment.^[9] Mucoepidermoid carcinomas, acinic cell carcinoma, malignant mixed salivary gland tumors, adenocarcinoma, and necrotizing sialometaplasia should be considered in the differential diagnosis of ACC. The management of ACC includes surgical resection with wide margins, paying specific attention to obtain clear margins in relation to regional nerves.^[8] Neck dissection should be carried out in patients having clinical and radiological evidence of metastasis to cervical lymph nodes. Administering postoperative radiation therapy is known to augment local and regional control in patients with ACC. Chemotherapy was proven to be ineffective in the management of ACC. Long-term follow-up is necessary in these cases because of the high rate of local recurrence and regional metastasis.^[10]

Conclusion

ACCs, previously termed as cylindromas, are rare tumors arising from the mucous glands, more specifically in the minor salivary glands of oral cavity. The characteristic presenting features of the ACC such as local recurrences, perineural spread, and distant metastases renders the local control of this tumor difficult. Timely diagnosis and adequate surgical therapy with adjuvant radiotherapy play an important role in long-term survival of the patient.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Coca-Pelaz A, Rodrigo JP, Bradley PJ, Vander Poorten V, Triantafyllou A, Hunt JL, et al. Adenoid cystic carcinoma of the head and neck – An update. Oral Oncol 2015;51:652-61.
2.	Foote FW, Frazell EL: Tumours of the major salivary glands. Cancer 1953;6:1065-133.
3.	van der Wal JE, Becking AG, Snow GB, van der Waal I. Distant metastases of adenoid cystic carcinoma of the salivary glands and the value of diagnostic examinations during follow-up. Head Neck 2002;24:779-83.
4.	Gnepp DR, Henley JD, Simpson RH, Eveson J. Salivary and lacrimal glands. In: Gnepp DR, editor. Text Book of Diagnostic Surgical Pathology of the Head and Neck. 2^nd ed. Philadelphia: Saunders Elsevier Publisher; 2009. p. 482-4.
5.	Fordice J, Kershaw C, El-Naggar A, Goepfert H. Adenoid cystic carcinoma of the head and neck: Predictors of morbidity and mortality. Arch Otolaryngol Head Neck Surg 1999;125:149-52.
6.	Mehta DN, Parikh SJ. Adenoid cystic carcinoma of palate. J Nat Sci Biol Med 2013;4:249-52.
7.	Zhao C, Liu JZ, Wang SB, Wang SC. Adenoid cystic carcinoma in the maxillary gingiva: A case report and immunohistochemical study. Cancer Biol Med 2013;10:52-4.
8.	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.
9.	Deepak C. Adenoid. Cystic carcinoma of the maxilla – A case report and 5 year follow-up. J Clin Case Rep 2012;2:221.
10.	Conley J, Baker DG. Cancer of the salivary glands. In: Suen JY, Meyer EN, editors. Cancer of the Head and Neck. New York: Churchill Livingstone; 1981. p. 524-56.