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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 3  |  Issue : 2  |  Page : 72-75

Adenoid cystic carcinoma mimicking benign mucosal cyst of maxillary sinus


Department of Oral and Maxillofacial Surgery, Sri Sai College of Dental Surgery, Vikarabad, Telangana, India

Date of Web Publication16-Jun-2016

Correspondence Address:
Uday Uppada Uppada
Department of Oral and Maxillofacial Surgery, Sri Sai College of Dental Surgery, Vikarabad, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2348-2915.184219

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  Abstract 

Adenoid cystic carcinoma (ACC) is an infrequent neoplasm arising in minor salivary glands and contiguous tissues in the head and neck region. It is further communal in the minor salivary glands (>25%) fairly than in the major salivary glands (about 5%). ACC has distinctive histologic features, with cribriform and tubular growth patterns of basaloid cells revealing principally a myoepithelial cellular phenotype. The clinical and pathological findings emblematic of this tumor include slow growth, perineural invasion, and impending local recurrence. This report presents a solitary lesion of the left maxilla which mimicked a benign mucosal cyst of the maxillary sinus. Initial clinical presentation and investigations were misleading while the microscopic examination of the excised lesion revealed an ACC.

Keywords: Adenoid cystic carcinoma, maxillary sinus, minor salivary gland tumor


How to cite this article:
Jadhav P, Paul D, Uppada UU. Adenoid cystic carcinoma mimicking benign mucosal cyst of maxillary sinus. J Dent Res Rev 2016;3:72-5

How to cite this URL:
Jadhav P, Paul D, Uppada UU. Adenoid cystic carcinoma mimicking benign mucosal cyst of maxillary sinus. J Dent Res Rev [serial online] 2016 [cited 2020 Dec 3];3:72-5. Available from: https://www.jdrr.org/text.asp?2016/3/2/72/184219


  Introduction Top


Clinical diagnostic skills and good judgment form the key to successful management of pathologies of the maxillofacial region. A careful observant eye can add to the diagnostic skills of the clinician and is considered important for proper patient care. Adenoid cystic carcinoma (ACC) is one such entity which requires careful evaluation and management. ACC is considered to be one of the rare tumors of the head and neck. They account for <1% of all head and neck malignancies. They include about 4–10% of all salivary gland tumors. Nevertheless, it is the most common malignant tumor of the minor salivary glands.[1],[2],[3]

The parotid gland is predominantly involved. Intraorally, palate accounts for 50% of ACC's.[4] It is most often clinically deceptive by its small size and slow growth, which actually overlies its extensive subclinical invasion and marked ability for early metastasis making the prognosis questionable.[5] Complete surgical resection with wide margins is the gold standard. However, adjuvant irradiation is indicated in clinical situations of close margins, perineural invasion, extensive primary tumor, or high-grade histology.[6],[7]

This article is intended to add to the existing literature pertaining to ACC, particularly in the maxillofacial region since it is commonly misdiagnosed or ignored in the list of differential diagnosis.


  Case Report Top


A 29-year-male reported to our unit with a complaint of pain, swelling, and mobility of teeth in the left upper back teeth region since a month. He gives a history of the same problem 1 year back, for which offending tooth was removed in a private clinic. Since there was no symptomatic relief, he reported to our unit after 6 months. Regional lymphadenopathy was not present.

Radiographic examination revealed a radiolucent lesion in the left maxillary sinus. Fine needle aspiration was done, and the cytology revealed inflammatory exudate. Based on the history and clinical presentation, also considering the slow growth of the observed lesion, we arrived at a provisional diagnosis of benign mucosal cyst of the maxillary antrum. Surgical enucleation of the cystic lesion was carried out through a caldwell-luc approach under general anesthesia. The complete specimen was sent to histopathological examination and was diagnosed as adenoid cystic carcinoma.

Mean time, the patient reported back with an intraoral asymptomatic volume growth on the palate. Swelling was a solitary oval of approximately 2 cm × 4 cm extending anteroposteriorly from the left 1st premolar to maxillary tuberosity, mediolaterally from midpalatal raphae to labial vestibule. It is well defined, soft, fluctuant, cystic in consistency, nontender, nonpulsatile and was not fixed to the underlying tissues [Figure 1].
Figure 1: Preoperative view revealing a well defined, nontender, soft, and fluctuant lesion affecting the maxilla

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Contrast computed tomography in coronal view shows radiolucency of the maxillary sinus, lateral wall of the nose and inferior orbital rim suggestive of a lesion extending into the maxillary sinus and lateral wall of the nose [Figure 2]. Excision of the lesion with subtotal maxillectomy was planned under general anesthesia. The procedure was performed through a Weber–Fergusons approach, extending intraorally from the midline toward the posterior aspect and clear margins were obtained intraoperatively [Figure 3].
Figure 2: Coronal section of the computed tomography showing the lesion revealing radiolucency of the maxillary sinus, lateral wall of the nose and inferior orbital rim suggestive of lesion extending into maxillary sinus and lateral wall of nose

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Figure 3: Intraoperative view. (a) Midpalatal split, (b) osteotomy along the anterolateral aspect of maxilla through a Weber–Fergusson approach, (c) excised lesion

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Histopathological findings of the post excision specimen revealed pseudostratified columnar ciliated epithelial lining with basaloid cells forming cribriform structure. Deposits of intercellular hyaline are seen in some areas of connective tissue stroma suggestive of adenoid cystic carcinoma [Figure 4]a and b]. The patient is advised postsurgical radiotherapy as an adjunctive therapy and found to have no evidence of recurrence in the past 6 years.
Figure 4: Histopathologic picture of the lesion. (a) Histopathological examination revealed pseudostratified columnar ciliated epithelial lining with basaloid cells forming cribriform structure. Deposits of intercellular hyaline are seen in some areas of connective tissue stroma, (b) H and E under ×40 shows lesional tissue containing nests of tumor cells as islands arranged in a tubular pattern. Each island consists of peripheral palisading basaloid cells with hyperchromatic nuclei. Few islands exhibit lumina. The intervening connective tissue shows dense bundles of hyalinized connective tissue fibers. Perineural invasion is also evident

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  Discussion Top


The protracted natural history even in the presence of local recurrence or distant metastases and its propensity to spread along perineural lymphatics makes adenoid cystic carcinoma (ACC) unique.[8],[9] Intraorally, they seldom grow larger than 3 cm at its greatest diameter.[10] Clinically, they are associated with pain early in the course of the disease before there is a noticeable swelling.[11] Most commonly seen during fourth to sixth decades of life with a 3:2 prevalence for females.[10] Most ACCs present submucosally.[12] Very rarely, intraosseous lesions occur in the posterior mandible. Bone invasion or a perineural spread can at times cause pain or hypoesthesia.[13]

ACCs arising from the minor salivary glands have poor prognosis than those arising from the major salivary glands.[14],[15] Tumors involving the paranasal sinuses generally have a poor prognosis. This is attributed to the delay in diagnosis.[16] Tumors arising from the minor salivary glands usually tend to infiltrate the extraglandular soft tissues and bone thereby allowing intensified dissemination of the lesion. Involvement of the lymph nodes is uncommon and is attributed to contiguous spread rather than lymphatic permeation or embolization.[17]

Three histological variants of ACC have been stated - cribriform, tubular, and solid types. Cribriform type is the most common variant with the solid type being the least common. Tubular pattern (well differentiated) is believed to have the best prognosis compared to the cribriform pattern (moderately differentiated) and solid pattern (poorly differentiated).[18] According to Szanto et al., ACC is graded as cribriform or tubular (Grade I), <30% solid (Grade II) or >30% solid (Grade III).[19]

The list of differential diagnosis for ACC generally comprises pleomorphic adenoma, polymorphous low-grade adenocarcinoma, basal cell adenoma, mixed tumor, and basaloid squamous cell carcinoma.[4]

Surgical intervention, radiotherapy, chemotherapy, and combined therapy are the available treatment modalities. Surgical intervention proves to be effective only when the lesion is excision with the widest surgical margins possible as tumor cells extend well beyond the clinical and radiographic margins. An effective treatment option could be surgical resection with postsurgical radiotherapy (not <60 Gy), which enhances the local and regional control.[4],[20],[21] Neutron therapy involving larger particles of greater energy can be employed to achieve reasonable local control as a primary therapeutic modality.[4],[22]

Adoptive immunotherapy along with chemoradiotherapy has recently shown promising results. E-cadherin expression can be used as a prognostic marker.[4] In the time of need, high argyrophilic nuclear organizing regions counts may be used to predict metastasis.[18] Anatomic location of the lesion, the size of the primary lesion, presence or absence of metastasis at the time of diagnosis, perineural invasion, and the histological variant are the factors that determine the survival rate of the patient.[23]

Long-term survival is mostly short in Grade III tumors. Distant metastasis occurs in 25–50% of patients with the lung being most commonly involved.[5],[24] A 5-year survival rate after effective treatment is 75%, but long-term survival rates are low (10 years - 20% and 15 years - 10%).[4],[5] With respect to our case, the age of occurrence of the lesion was unusual without much evidence of palatal swelling. Radiographs revealed radiolucency involving the maxillary sinus. Clinical and radiographic presentation has lead us to the provisional diagnosis of benign mucosal cyst of the maxillary sinus.


  Conclusion Top


This paper emphasizes the necessity of evaluating any excised lesion that affects the oral cavity by a trained pathologist for a correct diagnosis, thus enabling implementation of the appropriate treatment. When an aggressive lesion in the maxilla is encountered, especially in the palate, adenoid cystic carcinoma involving minor salivary gland tumors should always be considered in the list of differential diagnosis. Despite of the presence of adjuvant treatment options for ACC in the form of chemotherapeutic agents and radiotherapy, total excision with clear tumor margins is the main-stay of the treatment. Adequate rehabilitation of the patient with proper long-term follow-up is mandatory.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
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2.
Clark JM, Triana RJ, Meredith SD. Uncontrolled central adenoid cystic carcinoma: Case report. Ear Nose Throat J 2000;79:784-6.  Back to cited text no. 2
    
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Eneroth CM, Hjertman L, Moberger G. Adenoid cystic carcinoma of the palate. Acta Otolaryngol 1968;66:248-60.  Back to cited text no. 3
    
4.
Chundru NS, Amudala R, Thankappan P, Nagaraju CD. Adenoid cystic carcinoma of palate: A case report and review of literature. Dent Res J (Isfahan) 2013;10:274-8.  Back to cited text no. 4
    
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Chen AM, Granchi PJ, Garcia J, Bucci MK, Fu KK, Eisele DW. Local-regional recurrence after surgery without postoperative irradiation for carcinomas of the major salivary glands: Implications for adjuvant therapy. Int J Radiat Oncol Biol Phys 2007;67:982-7.  Back to cited text no. 6
    
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Terhaard CH, Lubsen H, Rasch CR, Levendag PC, Kaanders HH, Tjho-Heslinga RE, et al. The role of radiotherapy in the treatment of malignant salivary gland tumors. Int J Radiat Oncol Biol Phys 2005;61:103-11.  Back to cited text no. 7
    
8.
Spiro RH. Salivary neoplasms: Overview of a 35-year experience with 2,807 patients. Head Neck Surg 1986;8:177-84.  Back to cited text no. 8
    
9.
van der Wal JE, Snow GB, van der Waal I. Intraoral adenoid cystic carcinoma. The presence of perineural spread in relation to site, size, local extension, and metastatic spread in 22 cases. Cancer 1990;66:2031-3.  Back to cited text no. 9
    
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Gondivkar SM, Gadbail AR, Chole R, Parikh RV. Adenoid cystic carcinoma: A rare clinical entity and literature review. Oral Oncol 2011;47:231-6.  Back to cited text no. 10
    
11.
Neville BW, Damm D, Allen CM, Bouquot JE. Salivary gland pathology. In: Neville BW, editor. Oral and Maxillofacial Pathology. 3rd ed. Missouri: Saunders; 2009. p. 495-7.  Back to cited text no. 11
    
12.
Ellis GL, Auclair PL, Gnepp DR. Adenoid cystic carcinoma. In: Surgical Pathology of Salivary Glands. Philadelphia: WB, Saunders; 1991. p. 333-46.  Back to cited text no. 12
    
13.
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.  Back to cited text no. 13
    
14.
Nascimento AG, Amaral AL, Prado LA, Kligerman J, Silveira TR. Adenoid cystic carcinoma of salivary glands. A study of 61 cases with clinicopathologic correlation. Cancer 1986;57:312-9.  Back to cited text no. 14
    
15.
Dal Maso M, Lippi L. Adenoid cystic carcinoma of the head and neck: A clinical study of 37 cases. Laryngoscope 1985;95:177-81.  Back to cited text no. 15
    
16.
Kokemueller H, Eckardt A, Brachvogel P, Hausamen JE. Adenoid cystic carcinoma of the head and neck – A 20 years experience. Int J Oral Maxillofac Surg 2004;33:25-31.  Back to cited text no. 16
    
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Allen MS Jr., Marsh WL Jr. Lymph node involvement by direct extension in adenoid cystic carcinoma. Absence of classic embolic lymph node metastasis. Cancer 1976;38:2017-21.  Back to cited text no. 17
    
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Gnepp DR, Henley JD, Roderick HW, Simpson, Eveson J. Salivary and lacrimal glands. In: Gnepp DR, editor. Diagnostic surgical pathology of the Head and Neck. 2nd ed. Philadelphia: Saunders; 2009. p. 482-6.  Back to cited text no. 18
    
19.
Szanto PA, Luna MA, Tortoledo ME, White RA. Histologic grading of adenoid cystic carcinoma of the salivary glands. Cancer 1984;54:1062-9.  Back to cited text no. 19
    
20.
Mehta DN, Parikh SJ. Adenoid cystic carcinoma of palate. J Nat Sci Biol Med 2013;4:249-52.  Back to cited text no. 20
    
21.
Mitra S, Kundu S, Pattari SK, Ghosal AG. Metastatic pleural effusion: A rare presentation of salivary gland adenoid cystic carcinoma. Indian J Chest Dis Allied Sci 2011;53:107-10.  Back to cited text no. 21
    
22.
Buchholz TA, Shimotakahara SG, Weymuller EA Jr., Laramore GE, Griffin TW. Neutron radiotherapy for adenoid cystic carcinoma of the head and neck. Arch Otolaryngol Head Neck Surg 1993;119:747-52.  Back to cited text no. 22
    
23.
Barrett A, Speight P. The controversial adenoid cystic carcinoma. The implications of histological grade and perineural invasion. In: McGurk M, Renehan A, editors. Controversies in the Management of Salivary Gland Disease. Oxford: Oxford University Press; 2002. p. 211-7.  Back to cited text no. 23
    
24.
Locati LD, Guzzo M, Bossi P, Massone PP, Conti B, Fumagalli E, et al. Lung metastasectomy in adenoid cystic carcinoma (ACC) of salivary gland. Oral Oncol 2005;41:890-4.  Back to cited text no. 24
    


    Figures

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


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