Adenoid cystic carcinoma of the buccal vestibule: A case report and review of the literature

Adenoid cystic carcinoma of the buccal vestibule: A case report and review of the literature

Oral Oncology (2006) 42, 1029– 1032 available at journal homepage: CASE REPORT ...

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Oral Oncology (2006) 42, 1029– 1032

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Adenoid cystic carcinoma of the buccal vestibule: A case report and review of the literature Peter J. Giannini a,*, Kishore V. Shetty b, Sara L. Horan c, Wayne D. Reid d, Loven L. Litchmore e a

University of Nebraska Medical Center College of Dentistry, 40th and Holdrege Streets, Box 830740, Lincoln, NE 68583-0740, United States b The University of Texas Dental Branch at Houston, Houston, TX, United States c Massachusetts General Hospital, Boston, MA, United States d University of Colorado School of Dentistry, Aurora, CO, United States e Temple University School of Dentistry, Philadelphia, PA, United States Received 24 February 2006; received in revised form 30 April 2006; accepted 9 June 2006


Summary Minor salivary gland tumors of the buccal vestibule are relatively rare. Adenoid cystic carcinoma is the fifth most common salivary gland malignancy following mucoepidermoid carcinoma, adenocarcinoma not otherwise specified (NOS), acinic cell adenocarcinoma and polymorphous low-grade adenocarcinoma (PLGA). Greater than half of adenoid cystic carcinomas occur in the parotid and submandibular glands. The most common intraoral site is the palate. Adenoid cystic carcinoma tends to have a protracted clinical course with wide infiltration and late distant metastases. We present a case of an adenoid cystic carcinoma of the buccal vestibule in a 59-year-old Caucasian female patient that she had been aware of for 15 years. c 2006 Elsevier Ltd. All rights reserved.

Carcinoma; Adenoid cystic; Buccal; Vestibule


Adenoid cystic carcinoma of the salivary glands was first described in 1856 and at the time was referred to as cylindroma due to its unique histologic appearance.1 In 1928 it was renamed adenoid cystic carcinoma (ACC) and since then has been the generally accepted term for this neoplasm.2 * Corresponding author. Tel.: +1 402 472 4160; fax: +1 402 472 2551. E-mail address: [email protected] (P.J. Giannini).

Adenoid cystic carcinoma comprises less than 1% of all malignancies of the head and neck and is the fifth most common malignancy of salivary gland origin, representing 10% of salivary gland malignancies.3,4 The parotid and submandibular glands are the two most common sites for ACC accounting for 55% of the cases. Among the major glands the parotid is the most common site of occurrence. Intraorally 50% of ACCs occur on the palate with other less common sites of involvement including the lower lip, retromolar-tonsillar pillar region, sublingual gland, buccal mucosa, and floor of mouth.5 ACC has

1368-8375/$ - see front matter c 2006 Elsevier Ltd. All rights reserved. doi:10.1016/j.oraloncology.2006.06.005

1030 previously been shown to occur at a variety of anatomic sites in addition to the major and minor salivary glands including lacrimal glands, mucous glands of the aerodigestive tract, skin, and breast. The nose and paranasal sinuses represent the most common sites for minor gland ACCs.6 The age of patients affected with major salivary gland tumors has been shown to be younger (mean 44 years) compared with the age of those who developed tumors of the minor glands (mean 54 years) with a female/male ratio of 1.6:1.7 Adenoid cystic carcinoma characteristically displays an indolent, but persistent and recurrent growth pattern, long clinical course, and late onset of metastases. In contrast to other types of carcinomas, distant hematogenous metastases are far more frequent than regional lymph node metastases. In 40–60% of cases distant metastases develop and are most common in the lung, bone, and soft tissues.7 Distant metastases often develop despite local control of disease. Pain is typically noted with increased growth and duration as would be expected given that ACC frequently demonstrates peripheral nerve invasion. When occurring on the palate ulceration is common. ACCs of the parotid may exhibit symptoms of facial nerve paralysis. The three recognized histopathologic patterns of adenoid cystic carcinoma are cribiform, tubular, and solid with cribiform being the most common and easily recognized pattern and solid the least common histopathologic subtype. The cribiform subtype of ACC is thought to have the best prognosis and the solid subtype the worst with the tubular form possessing an intermediate prognosis.8–14 Often, more than one histopathologic pattern is observed in a single neoplasm. All three patterns of ACC consist of both ductal and myoepithelial cells. The cribiform pattern has been described as ‘‘swiss cheese-like’’ and consists of pseudocystic spaces that either contain basophilic glycosaminoglycan or eosinophilic basal lamina material. The tubular pattern demonstrates more conspicuous ductal spaces and represents the most differentiated microscopic pattern of ACC. The solid pattern contains few or no cyst-like spaces and exhibits a greater degree of nuclear and cellular pleomorphism as well as mitotic activity when compared to the cribiform and tubular patterns. In addition, occasional comedonecrosis may also be observed which is uncommonly observed in the other subtypes.

Case report A 59-year-old Caucasian female presented to the dental clinic for evaluation of a mass of the left buccal vestibule. The patient reported that it initially presented as an asymptomatic nodule of the left cheek region approximately 15 years ago. She had noticed that the lesion had been increasing in size over the past several months, accompanied by occasional symptoms of pain. The extraoral examination was within normal limits with no evidence of lymphadenopathy. An intraoral examination revealed a red,f firm lesion of the left buccal mucosa measuring 5 cm f For interpretation of color in Figs. 1 and 3, the reader is referred to the web version of this article.

P.J. Giannini et al.

Figure 1 Clinical image of a mass of the buccal vestibule with prominent telangiectatic vessels.

Figure 2 Clinical image demonstrating the relationship of the focally ulcerated mass to the maxillary complete denture.

in greatest dimension (Fig. 1). The patient was completely edentate and was wearing a maxillary complete denture (Fig. 2). The lesion appeared to be attached to the alveolar ridge in the #12/13 region with an area of focal ulceration. The remainder of the intraoral examination was within normal limits. The patient subsequently underwent an incisional biopsy. At gross examination, the specimen consisted of an irregular pink-tan fragment of mucosal tissue measuring 0.8 · 0.5 · 0.2 cm in greatest dimension. Histologically the lesion consisted of mucosa overlying an infiltrating tumor composed of discrete sheets, islands, and nests of neoplastic epithelial cells exhibiting both cribiform and solid patterns and forming duct-like spaces (Fig. 3). Most of the tumor cells contained scant cytoplasm with ovoid and angular darkly staining nuclei with prominent nucleoli. The cells lining the duct-like spaces contained abundant eosinophilic cytoplasm and paler staining nuclei (Fig. 4). The lumina contained an amorphous mucicarmine positive and PAS positive material. Numerous typical and atypical mitotic figures and occasional small calcifications were observed (Fig. 5). The preliminary diagnosis was adenocarcinoma of salivary gland origin with a comment that some features were suggestive of adenoid cystic carcinoma. A second biopsy yielded a similar diagnosis to the previous one, and it was subsequently sent to the United States Armed Forces Institute of Pathology (AFIP) for consultation.

Adenoid cystic carcinoma of the buccal vestibule: A case report and review of the literature


yngology for definitive management. Work-up of the patient did not reveal radiographic evidence of tumor invasion involving the maxilla. In addition, there was neither clinical evidence of regional nodal involvement nor metastatic disease. Treatment consisted of wide surgical excision with a cheek flap and lower lip split followed by free flap reconstruction and radiation therapy. The final diagnosis of the surgical specimen was adenoid cystic carcinoma. To our knowledge the patient is currently free of disease two years post operatively.

Discussion Figure 3 A medium power photomicrograph demonstrating a neoplasm of glandular epithelial origin within the lamina propria with a focal cribiform pattern (H&E · 100).

Figure 4 Evidence of perineural invasion can been seen with formation of glandular and pseudoglandular elements and a characteristic cribiform pattern (H&E · 100).

Figure 5 A high power photomicrograph illustrating cuboidal cells with hyperchromatic nuclei and visible accumulation of an eosinophilic hyalinized material within pseudocystic spaces (H&E · 400).

The AFIP diagnosis confirmed the original diagnosis of adenocarcinoma, not otherwise specified, intermediate grade, with features of adenoid cystic carcinoma. The patient was ultimately referred to the Department of Otorhinolar-

Although the buccal mucosa is an uncommon site for adenoid cystic carcinoma, the relatively indolent growth pattern of the currently reported case is rather typical for this type of salivary gland malignancy. ACC has a peak incidence in the fourth to sixth decades of life with a 3:2 prevalence for females,7 which are the parameters that fit with our case. Adenoid cystic carcinomas of the minor glands have been reported to have a worse prognosis that those of the major salivary glands.15,16 This may be explained by the fact that tumors of the minor salivary glands can more readily infiltrate extraglandular soft tissues and bone thereby allowing for increased dissemination of the tumor rendering complete excision more problematic. The histopathology of the present case demonstrated a combination of cribiform and solid growth patterns suggestive of an intermediate grade lesion. The histopathologic differential diagnosis of adenoid cystic carcinoma can include either polymorphous low-grade adenocarcinoma (PLGA) or epithelial–myoepithelial carcinoma (EMC). Adenoid cystic carcinoma, PLGA, and EMC each are composed of a biphasic population of luminal epithelial and abluminal myoepithelial cells. In addition, both PLGA and ACC have a propensity to infiltrate around peripheral nerves.7 The distinction between adenoid cystic carcinoma and polymorphous low grade adenocarcinoma is important due to the significantly different biologic behavior of these neoplasms. PLGA is typically comprised of a relatively uniform population of epithelial cells arranged in variable patterns with mitotic activity being distinctly uncommon. ACC conversely exhibits myoepithelial cells with more angular and hyperchromatic nuclei that surround luminal ductal epithelial structures. Although tubular and cribiform growth patterns may also be observed in PLGA, they are more limited in comparison to ACC.7 Although epithelial–myoepithelial carcinoma demonstrates a similar biphasic pattern to that noted in ACC, it is more conspicuous in EMC. Furthermore, the abluminal myoepithelial cells in EMC are typically larger possessing round nuclei and clear cytoplasm which is in contrast to the angular hyperchromatic nuclei of the smaller abluminal cells noted in ACC. The large clear cells noted in EMC may comprise a dominant portion of the neoplasm and contain glycogen, unlike in ACC. Furthermore, EMC does not demonstrate a cribiform growth pattern although, tubular growth and the formation of prominent basal lamina material may be noted in EMC as is also noted in ACC. The basal lamina material imparts a hyalinized appearance to the stroma.7

1032 The treatment of choice for adenoid cystic carcinoma is wide surgical excision and postoperative radiation therapy for the best chance of long-term survival.16–18 Previous studies have also demonstrated favorable results with cisplatin as well as high-dose melphalan chemotherapy, however, statistically there was no difference noted in survival rates when compared to placebo.19–21

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