Clinical Review & Education

Review

Minor Salivary Gland Basal Cell Adenocarcinoma A Systematic Review and Report of a New Case David W. Cuthbertson, MD; Nikhila Raol, MD; John Hicks, MD, DDS, PhD; Linda Green, MD; Robert Parke, MD

IMPORTANCE Basal cell adenocarcinoma (BCAC) of the minor salivary gland is an extremely rare disease: the most recent substantive literature review reports only 25 cases. Owing to the rarity of this disease, it has not yet been well characterized in the literature. OBJECTIVE We sought to expand the knowledge of minor salivary gland BCAC by performing an exhaustive literature review and adding to it a new case that is rare owing to the tumor’s size, aggressive nature, and mixed histologic pattern. The review emphasizes epidemiologic patterns, diagnostic characteristics, treatment patterns, and expected prognosis for minor salivary gland BCAC. EVIDENCE ACQUISITION In June 2012, PubMed was queried using the term “salivary gland basal cell adenocarcinoma,” and the resultant articles were reviewed. Those specifically mentioning a minor salivary gland BCAC were included in this study. Those that did not differentiate minor salivary gland BCAC from major salivary gland BCAC were excluded. The search was not limited by language and included articles from North America, Europe, Africa, and Asia from 1978 to June 2012. RESULTS The PubMed search resulted in 195 articles, of which 33 articles reported at least 1 case of minor salivary gland BCAC. We report herein 72 cases of minor salivary gland BCAC (71 cases from the literature review and 1 new case reported herein). The mean patient age at the time of presentation was 56 years (range, 24-90 years), and the disease showed no sex predilection. The most common location was the palate, and the average lesions size was 2.4 cm (range, 0.7-4.2 cm). The treatment modality of choice was wide local excision (n = 57; 79%). There was a high local recurrence rate (n = 30; 41%) but a low rate of distant metastasis (n = 8; 11%). CONCLUSIONS AND RELEVANCE We present a comprehensive review of minor salivary gland BCAC, describing nearly 3 times as many cases as has been previously reported. This review characterizes a rare disease and increases awareness of the disease among otolaryngologists. Minor salivary gland BCAC is similar to major salivary gland BCAC and minor salivary gland tumors in general with regard to patient age, sex, tumor site, treatment modality, recurrence, metastasis, and mortality.

Author Affiliations: Bobby R. Alford Department of Otolaryngology–Head and Neck Surgery, Baylor College of Medicine, Houston, Texas (Cuthbertson, Raol, Parke); Department of Pathology and Immunology, Baylor College of Medicine, Houston, Texas (Hicks, Green); Michael E. Debakey Department of Veteran’s Affairs Medical Center, Houston, Texas (Green, Parke).

JAMA Otolaryngol Head Neck Surg. 2015;141(3):276-283. doi:10.1001/jamaoto.2014.3344 Published online January 2, 2015. 276

Corresponding Author: David W. Cuthbertson, MD, Bobby R. Alford Department of Otolaryngology–Head and Neck Surgery, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030 ([email protected]). (Reprinted) jamaotolaryngology.com

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Minor Salivary Gland Basal Cell Adenocarcinoma

Review Clinical Review & Education

B

asal cell adenocarcinoma (BCAC) is a rare malignant lesion of the salivary gland, accounting for less than 2% of all salivary neoplasms.1 Approximately 90% are found in the parotid gland,2-4 and the remainder occur in the submandibular gland. Minor salivary gland BCAC is exceedingly rare: the most recent substantial review of the literature reports only 25 total cases, 10 of which arose from the palate.5,6 We present a new case of an extensively destructive palatal BCAC involving the maxillary sinus, nasal cavity, masticator space, and pterygopalatine fossa, to our knowledge only the second such case reported to date.6 We also performed an exhaustive literature search, including international and non-English literature, and found reports of 72 total cases of minor salivary gland BCAC.

Report of a Case A man in his 50s with a 30 pack-year smoking history presented after he noticed air passing between his nasal and oral cavities when his mouth was shut. The patient was otherwise asymptomatic. Findings of the physical examination were unremarkable except for a small right anterior hard palate fistula extending into the right nasal cavity and a tender fullness of the right nasal floor surrounding the opening of the fistula. A preliminary biopsy showed the mass to be a BCAC, possibly arising from a basal cell adenoma (BCA). A maxillofacial computerized tomographic (CT) study revealed a destructive mass measuring 2.7 × 2.3 × 3.0 cm centered in the posterior right maxillary sinus (Figure 1). Circumferential bony- and soft-tissue destruction were present, and perineural spread was evident along the maxillary branch of the trigeminal nerve. Results of contrast-enhanced magnetic resonance imaging (MRI) confirmed the CT findings. Positron emission tomography (PET) showed no evidence of distant metastases. Interestingly, the patient had imaging on file from 4 years earlier that showed no evidence of disease. A right infrastructure maxillectomy and radical ethmoidectomy were performed, with surgical repair by split-thickness skin graft and obturator placement. Owing to the extent of disease in-

vasion, a subtotal resection was performed. Histopathologic examination of the resected specimen confirmed the diagnosis of BCAC arising from salivary gland tissues with perilymphatic and perineural invasion. The patient was unable to tolerate chemoradiation and was treated palliatively. The resected specimen measured 4.8 cm in maximum dimension and consisted of tan-pink to red soft tissue interspersed with bony spicules. Focal areas were lined by smooth and glistening mucosa without tumor excrescences on the mucosal surface. Microscopic examination of the mass revealed tumor cells organized into several patterns, including tubular, trabecular, and solid patterns (Figures 2A and B). The tumor was composed of 2 cell layers within the tubular and trabecular pattern regions (Figures 2A and B) with centrally placed large polygonal cells and readily identified eosinophilic to somewhat amphophilic cytoplasm. At the periphery of these large polygonal cells were smaller cells with more dense nuclei and less conspicuous cytoplasm. Occasional typical mitotic figures were seen. The tumor nests were separated by a modest amount of stroma. Readily identified vascular (Figure 2C) and perineural (Figures 2D and E) invasion were present. There was diffuse tumor invasion of bone (Figure 2F), skeletal muscle (Figure 2G), and adjacent salivary gland lobules (Figure 2H). In all tissue sections, there was no tumor capsule separating the tumor from adjacent normal tissue. The tumor diffusely infiltrated the soft tissue and bone. Frozen and permanent sections of the tumor showed multifocal extension of tumor to the resection margins. This case is being reported for the first time herein. It was not found as part of the literature search described in the Methods section.

Methods In June 2012, PubMed was queried using the term “salivary gland basal cell adenocarcinoma,” and the resultant articles were reviewed. Those specifically mentioning a minor salivary gland BCAC, whether as a case report or as a data point in a database review, were included in this study. When cases of minor salivary gland BCAC were

Figure 1. Preoperative Maxillofacial Computed Tomographic Images With Contrast From the Present Case Report A

B

A, Sagittal view shows the mass eroding through the hard palate (red arrowhead) and extending into pterygopalatine fossa (asterisk). B, Axial view shows the mass extending into the nasal cavity (plus sign) and pterygopalatine fossa (asterisk). In both panels, the tumor edges are marked with yellow arrowheads. jamaotolaryngology.com

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Minor Salivary Gland Basal Cell Adenocarcinoma

Figure 2. Hematoxylin-Eosin–Stained Specimens of Basal Cell Adenocarcinoma Involving the Palate From the Present Case Report A

B

C

D N N

E

F

G

H S S

N

A and B, Tumor with tubular pattern composed of centrally placed large polygonal cells with a moderate amount of cytoplasm and peripherally placed smaller cells with modest amount of cytoplasm and dense nuclei (original magnification ×200 [A] and ×400 [B]). C, Prominent vascular invasion by tumor cells (arrowheads) (original magnification ×100). D, Perineural invasion by tumor cells (arrowheads) (original magnification ×200). E, Perineural

invasion by tumor cells at higher magnification (original magnification ×400). F, Tumor invading between reactive bone trabeculae (original magnification ×200). G, Tumor invading skeletal muscle (original magnification ×200). H, Tumor infiltrating adjacent nonneoplastic minor salivary gland lobule; note the absence of a tumor capsule (original magnification ×100). In all panels where they appear, N indicates nerve fibers; S, skeletal muscle.

not differentiated from major salivary gland BCAC, the authors were contacted for clarification. If ambiguity persisted, the study was excluded. Pertinent references from the included articles were reviewed for cases of minor salivary gland BCAC as well. The search was not limited by language and included articles from North America, Europe, Africa, and Asia. Translators were used to glean data from articles written in languages other than English.

Note that we have included a total of 72 cases of minor salivary gland BCAC in the present report: 71 cases of minor salivary gland BCAC found in our literature search, and 1 case of minor salivary gland BCAC we report herein for the first time. However, in Table 2, you will find 73 BCAC cases listed. This is because we have included a case of major salivary gland BCAC (No. 14) reported by Fonseca and Soares,12 of interest for its rarity; it was the only reported sublingual gland BCAC found in the literature. We found that minor salivary gland BCAC was typically treated by wide local excision alone (n = 57; 79%), but several patients required irradiation and/or chemotherapy for additional treatment. There was a high local recurrence rate of 41% (n = 30), and 11% of patients were found to have locoregional metastases (n = 8), only 1 of whom died of disease. Fortunately the prognosis for BCAC remains quite favorable owing to its indolent nature: 89% of patients were alive at the time their cases were reported (n = 64). These findings are in keeping with previously reported statistics for both major and minor salivary gland BCAC, which include an average patient age of 60 years, a 37% to 44% local recurrence rate, 11% to 12% rate of metastasis, and a 95% 5-year survival rate.8,27 Distant metastases were found to be quite rare, but there was 1 case of cutaneous metastasis8 and 3 cases of lung metastasis. One of the cases of lung metastases was from the parotid gland,2 and 2 were from unspecified salivary glands.7

Results A total of 195 articles were found through the PubMed search. These articles and their references were reviewed, and a total of 33 articles were confirmed to report at least 1 minor salivary gland BCAC. The articles were all published between 1978 and June 2012. In an effort to update the literature and provide the most accurate portrayal of the disease, we performed an exhaustive literature review that, combined with the present new case report, revealed 72 total cases of minor salivary gland BCAC. The mean patient age at the time of presentation was 56 years (range, 24-90 years); the disease showed was no sex predilection (Table 1). In general, over three-quarters of the tumors occurred de novo, and the rest arose from BCAs.8,9 Patients usually presented with a slowly enlarging mass. Symptoms were caused by local expansion and depended on the location of the mass. The most common location for minor salivary gland BCAC was the palate (32%), followed by the buccal mucosa (18%), nasal cavity and/or sinuses (10%), floor of the mouth (7%), and upper lip (7%). The average size was 2.4 cm (range, 0.7-4.2 cm) as measured in the largest dimension (Table 2).2,5-7,9-36 Because the disease remains a relatively rare entity, specific risk factors have not yet been identified but may be similar to risk factors for salivary gland tumors in general, including radiation exposure and occupational exposures such as silica dust and nitrosamines.37 278

Discussion Basal cell adenocarcinoma, previously known as basaloid salivary carcinoma, carcinoma ex monomorphic adenoma, and malignant BCA, accounts for 1.6% of all salivary neoplasms and 2.9% of all malignant salivary neoplasms.21,24,38 The overwhelming majority arise in the parotid (90%) and submandibular glands,8 with very few reported in the minor salivary glands. It appears to have been first de-

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scribed between 1974 and 1978,1,39 but it was not referred to as a basal cell adenocarcinoma until 1988.40 The lesions were found only in the parotid and submandibular salivary glands until Luna et al9 reported the first minor salivary gland BCAC, located in the palate. First recognized as a salivary gland neoplasm by the World Health Organization in 1991, BCAC was more specifically defined in 2005 as an epithelial neoplasm that has cytological characteristics of basal cell adenoma (BCA), but a morphologic growth pattern indicative of malignancy.41 Minor salivary gland BCAC continues to be an extremely rare entity: the most recent substantive literature review lists only 25 cases.5 In an effort to update the literature and provide the most accurate portrayal of the disease, we performed an exhaustive literature review that, combined with the present new case report, revealed 72 total cases of minor salivary gland BCAC. Grossly, BCAC appears as a solid gray-tan mass and has been reported as large as 7 cm in diameter in the major salivary glands.29 The tumor is composed of 2 cell types—small cells with darkly staining nuclei and a high nuclear-to-cytoplasm ratio, and larger polygonal cells with pale basophilic nuclei and eosinophilic to amphophilic cytoplasm. The cells are usually arranged in nests of varying size embedded in collagenous stroma with a palisading pattern of smaller cells at the periphery of the nests.42 There are 4 major BCAC morphologic patterns: the most common is solid, followed in descending frequency by membranous, tubular, and trabecular.6,42 Some researchers have proposed combining the tubular and trabecular forms and recognize an additional cribriform pattern.29 Specimens from BCAC tumors are almost uniformly reactive for cytokeratin, and most also test positive for epithelial membrane antigen (EMA) and carcinoembryonic antigen (CEA). Not surprisingly, smooth muscle actin, S-100, and vimentin—all markers of myoepithelial differentiation—are expressed by BCAC with a predilection for tumor cells at the periphery of cell nests. Immunohistochemical stains are helpful but not always diagnostic because cells lose their ability to express typical antigens as the tumor progresses and dedifferentiates. Indeed, staining patterns vary among different BCAC tumors and even within an individual tumor, making these markers at most only a helpful adjunct in the diagnostic process.6,43 Differentiating BCAC from other minor salivary gland tumors such as BCA, adenoid cystic carcinoma (ACC), basaloid squamous cell carcinoma (BSCC), basal cell carcinoma, or peripheral ameloblastoma is clinically important because of the differences in prognosis for these diseases. For example, while BCAC has a 10-year survival rate well above 75% owing to its low-grade behavior and low metastatic rate, the 10-year survival rate for ACC is as low as 29%.10,21 Distinguishing BCAC from BCA may be the most difficult of these distinctions owing to their histopathologic similarities. Often BCAC shows considerable cytologic atypia and an increased mitotic rate compared with BCA (a proliferative index >10%, as measured by Ki-67 [mib-1] immunostaining, raises suspicion for malignancy). However, these differences alone are not sufficiently reliable to render a definitive diagnosis.26 The hallmark characteristics of malignancy are gross or microscopic invasion into the surrounding soft tissue and perineural or vascular invasion. Additionally, BCA tends to be encapsulated, whereas BCAC typically is not. Some BCAs have features that imitate BCAC such as multinodularity, a pushing growth pattern, or multifocal origin. One must take extra precaution in cases such as these to carefully distinguish between the entities.21 jamaotolaryngology.com

Table 1. Clinicopathologic Features of Reported Cases of Minor Salivary Gland BCAC Characteristica Patient age, mean, median (range),c y (n = 50)

Measureb 56.1, 60.5 (24-90)

Men, mean (n = 25), y

56.6

Women, mean (n = 25), y

55.6

Sex (n = 50), No. Men

25

Women

25

Tumor site (n = 73) Palate

23 (32)

Buccal mucosa

13 (18)

Nasal cavity, sinus

7 (10)

Upper lip

5 (7)

Floor of mouth

5 (7)

Oral cavity, unspecified

4 (5)

Tongue

4 (5)

Reported as “other”

3 (4)

Unreported location

3 (4)

Alveolar mucosa

2 (3)

Sublingual glandd

1 (1)

Supraglottic larynx

1 (1)

Upper gingiva

1 (1)

Lower gingiva Tumor size, mean, median (range), cm (n = 20)

1 (1) 2.4, 2.5 (0.7-4.2)

Treatment (n = 28) Surgery alone

22 (79)

Surgery + irradiation

3 (11)

Irradiation alone

1 (3)

Surgery + chemotherapy

1 (3)

Surgery + irradiation + chemotherapy

1 (3)

Local recurrence (n = 37) Yes

15 (41)

No

22 (59)

Cervical metastasis (n = 35) Yes

4 (11)

No

31 (89)

Follow-up (n = 35) Alive Dead of diseasee

31 (89) 4 (11)

Abbreviation: BCAC, basal cell adenocarcinoma. a

Not all reports included complete information for each case. For each category in the table, only the cases for which information was specifically reported were used. The number of cases qualifying for each category is noted.

b

Unless otherwise indicated, data are reported as number (percentage) of cases.

c

Yu et al7 reported a 4-year-old with BCAC, but it is unclear whether this was in a major or minor salivary gland.

d

This case was included because it was the only sublingual BCAC found in the literature.

e

A total of 35 cases were reported with mortality. The 4 patients who died of their disease are not the same 4 who had cervical metastases.

Adenoid cystic carcinoma is similar to BCAC in that perineural and intravascular invasion are common to both, as are hyaline nodules within their basement membranes.21 However, ACC is composed of pale cells with irregularly angulated nuclei, unlike the dual

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Table 2. List of All Minor Salivary Gland Basal Cell Adenocarcinoma Cases Reported Source Luna et al,9 1989

Patient No./Sex/ Age, y

Tumor Site

Size, cm

Treatment

Local recurrence

Cervical Metastasis

S

+



Alive Alive

Outcome

1/F/66

Palate



2/M/27

Buccal

0.7

S

+

+

3/M/67

Buccal



S





Lo et al,10 1992

4/F/28

Palate

4

S





Yoshida et al,11 1996

5/M/43

Lower gingiva

2.5

S





Alive

6/F/51

Palate





+



Dead

7/M/63

Tongue





+



Dead

8/M/60

Buccal





+

+

Alive

9/F/43

Sinus





+



Dead

10/M/66

Sinus









Alive

11/F/70

Buccal





+



Alive

12/M/68

Buccal









13/F/62

Nasal cavity





+

+

14a/F/71

Sublingual









Alive

15/F/65

Tongue









Alive

Ellis and Wiscovitch,2 1990

Fonseca and Soares,12 1996 (12 BCAC cases, including 1 in major salivary gland [No. 14], included for its rarity)

Antonescu and Terzakis,13 1997 Lopes et al,14 1998b Plath and Dallenbach,15 1998

Yu et al,7 1999b,c (12 cases)

Wada et al,16 2000

… Alive

… Dead

16/F/56

Nasal cavity





+



Alive

17/M/52

Sinus





+



Alive

18/M/37

Upper lip



S





Alive











19



20/F/42

Palate

1.3

S





Alive

21

Palate











22

Palate











23

Palate











24

Palate











25

Palate











26

Palate











27

FOM











28

FOM











29

Cheek











30

Cheek











31

Sinus









32/M/65

Buccal

3

S+R





Alive Alive



de Sousa et al,17 2000

33/F/24

Palate

3

S





Ramchandani et al,18 2000

34/F/73

Upper lip



S







35/F/90

Buccal

3









36/F/28

Sinus

Ruíz-Godoy et al,19 2001 Jansisyanont et al,20 2002b

37























38/M/51

Upper lip

3.5

S





Alive

Jayakrishnan et al,21 2003

39/F/67

Buccal

1

S

+



Alive

40/F/47

Palate

1.3

Gross et al,22 2004

41/M/55

Buccal

3

Toida et al,23 2005

42/M/59

Palate

Poulopoulos et al,24 2005

43/M/61

Upper lip

S





Alive

S+R





Alive



S

+



1.5

S





… Alive

Ito et al,25 2005

44/F/51

Hard palate

2

S+R





Yamagata et al,26 2006

45/F/86

Palate

2

R

+



Alive

46/F/66

Buccal



S





Alive

47/F/42

Palate



S





Alive

FOM









49/M/65

Upper gingiva

4.2

S





Alive

Ward et al,29 2009

50/F/69

Palate

2

S





Alive

Gupta et al,30 2010

51/M/65

Tongue

1

S





Alive

Parashar et al,27 2007 Subhashraj,28 2008b Hirai et al,5 2009

48





(continued)

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Table 2. List of All Minor Salivary Gland Basal Cell Adenocarcinoma Cases Reported (continued) Source Maddox et al,31 2010

Patient No./Sex/ Age, y 52/M/35

Tumor Site

Size, cm

Treatment

Local recurrence

Cervical Metastasis



S





BOT

Erovic et al,32 2010b

53





+



Bailey et al,33 2011

54/F/83

Supraglottis



S+C



+

55

Oral cavity











56

Oral cavity











57

Oral cavity











58

Oral cavity











59

Other











60

Other











61

Other











62

Palate











63

Palate











64

Palate











65

Palate











66

FOM











67

FOM











68

Buccal











69

Alv mucosa











70

Alv mucosa









S





Alive

Bjørndal et al,34 2011b,d (7 cases)

Venkata and Irulandy,35 2011e (9 cases)



… Alive



Sakallioğlu et al,36 2012

71/M/41

Upper lip

2.5

Akiyama et al,6 2012

72/M/69

Palate

4

S





Alive

Present case, 2012

73/M/52

Palate

3

S+R+C

+



Alive

patients were male; 9 were female. All were treated surgically; 2 had cervical metastases and received radiation therapy. Of the 13 patients who followed up, 9 experienced recurrence; 2 had metastases to the lungs; 5 died of their disease, 4 were alive with disease; and 4 were alive with no evidence of disease.

Abbreviations: Alv, alveolar; BCAC, basal cell adenocarcinoma; BOT, base of tongue; C, chemotherapy; FOM, floor of mouth; R, radiation therapy; S, surgery; ellipses, data not reported; plus sign, event occurred; minus sign, event did not occur. a

Outcome Alive

This case is not of minor salivary gland BCAC; although the tumor occurred in a major salivary gland, this case was included because it is the only reported sublingual gland BCAC found in the literature.

b

Study did not explicitly detail patient sex and age.

c

Yu et al7 reported a total of 14 cases of BCAC, 1 in the parotid gland, 2 in the submandibular gland, and the other 11 listed here. Because the data for the minor salivary glands were not provided separately from the major salivary glands, the exact epidemiologic data for each case could not be ascertained. For the 14 cases, the median patient age was 45 years (range, 4-79 years). Five

population of large pale cells and small dark cells of BCAC.42 A high mitotic rate and increased frequency of necrosis also favor the diagnosis of ACC over BCAC.21,42 In addition, ACC is commonly associated with a cribriform pattern and pseudocysts filled with basophilic mucin and eosinophilic PAS-positive hyaline, which are not found in BCAC.26,42 Differentiating between BCAC and ACC is of particular importance due to the differences in prognosis. The 10-year survival rate for BCAC is above 75%, whereas the 10-year survival rate for ACC is around 30% owing to its tendency for hematogenous spread, especially to the lungs.10,21 Basaloid squamous cell carcinoma may also be mistaken for BCAC because of the similar characteristics in basaloid morphology, hyaline deposits, and peripheral palisading.21,26 However, BSCC can be differentiated by histopathologic findings of central necrosis, high tumor grade, and squamous differentiation.5,21,42 Furthermore, BSCC is commonly found in the hypopharynx, base of the tongue, and supraglottic larynx, locations where BCAC is rarely encountered.5,42 The distinction between BSCC and BCAC is further aided by immunohistochemical findings: BSCC stains negajamaotolaryngology.com

d

Bjørndal et al34 reported a total of 14 cases of BCAC, but only 7 were in minor salivary glands. For all 14 cases, half the patients were female; half were male; and the mean age was 65 years.

e

Venkata and Irulandy35 reported 9 cases but did not report exact data for each patient. The cases of 5 men and 4 women were reported. The average age for men was 63 years, and the average age for women was 45 years (overall range, 30-75 years). The specific tumor locations were listed but not linked to particular patients.

tively for Ber-EP4, vimentin, and S-100 protein, whereas BCAC may stain positively for these 3 proteins.5,26 Other neoplasms in the differential diagnosis of BCAC include basal cell carcinoma, polymorphous low-grade adenocarcinoma, small cell (neuroendocrine) carcinoma, and canalicular adenoma. Salivary gland basal cell carcinomas are exceptionally rare. Citing the lack of intraoral structures containing basal cells, some have contended that these tumors may actually be peripheral ameloblastomas misdiagnosed as basal cell carcinomas.26 Polymorphous low-grade adenocarcinomas have varied growth patterns with single-file cellular infiltration and cystic structures. Small cell (neuroendocrine) carcinoma is usually composed of short, spindled cells with nuclear molding that stain positively for synaptophysin and neuronspecific enolase.21,22 Canalicular adenoma frequently occurs in minor salivary glands of the upper lip, and its multifocal origin may be mistaken for malignant invasion. It can be differentiated from similar malignant neoplasms by its uniform beaded double strands of columnar epithelium, cyst formation, loose vascular stroma, and lack of destruction or invasion.21

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The widely accepted treatment for BCAC is complete surgical excision. With major salivary glands, either superficial or whole-gland excision is recommended, depending on the gland and the depth of tumor invasion. Minor salivary gland tumors have a tendency for infiltration of adjacent soft tissues and therefore require wide local excision to ensure complete resection. Frozen section microscopic examination of the surgical margins should be performed to ensure complete excision of both major and minor salivary gland tumors.2,16,24,26,44 Regional lymph node dissection is recommended only if there is evidence of metastatic disease to the cervical chain, and adjuvant radiation therapy has been used for invasive tumors and local recurrences.6,22

Conclusions Salivary gland BCAC is still considered a very rare disease, as evidenced by a recent review of nearly 7000 salivary gland tumors that ARTICLE INFORMATION Submitted for Publication: August 16, 2014; final revision received October 5, 2014; accepted October 17, 2014. Published Online: January 2, 2015. doi:10.1001/jamaoto.2014.3344. Author Contributions: Dr Parke had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. Study concept and design: Cuthbertson, Raol, Hicks, Parke. Acquisition, analysis, or interpretation of data: Cuthbertson, Hicks, Green. Drafting of the manuscript: Cuthbertson, Hicks, Parke. Critical revision of the manuscript for important intellectual content: Cuthbertson, Raol, Hicks, Green. Statistical analysis: Cuthbertson. Administrative, technical, or material support: Hicks, Parke. Study supervision: Raol, Hicks, Green, Parke. Conflict of Interest Disclosures: None reported. Previous Presentation: This research was presented as a poster at the combined meeting of the American Head and Neck Society and the International Federation of Head and Neck Oncologic Societies; July 28, 2014; New York, New York.

parotid gland: case report and review of the literature. Med Oral Patol Oral Cir Bucal. 2006;11(2): 206-209. 4. Franzen A, Koegel K, Knieriem HJ, Pfaltz M. Basal cell adenocarcinoma of the parotid gland: a rare tumor entity: case report and review of the literature [in German]. HNO. 1998;46(9):821-825. 5. Hirai H, Harada H, Okada N, Omura K. A case of basal cell adenocarcinoma of the upper gingiva. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2009;107(4):542-546. 6. Akiyama K, Karaki M, Hosikawa H, Mori N. A massive basal cell adenocarcinoma of the palatal minor salivary gland that progressed into the pterygopalatine fossa. Int J Oral Maxillofac Surg. 2012;41(4):444-447.

17. de Sousa SO, Schwarzschild M, de Araújo NS, de Araújo VC. Basal cell adenocarcinoma of the palate with squamous metaplasia. J Clin Pathol. 2000;53 (2):153-156. 18. Ramchandani M, Brown AM, Rippin JW, Murray PI. Labial adenocarcinoma after treatment with cyclosporin a in a patient with panuveitis. Am J Ophthalmol. 2000;130(1):127-128.

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Additional Contributions: The authors acknowledge the administrative support supplied by staff members of the Bobby R. Alford Department of Otolaryngology–Head and Neck Surgery and Department of Pathology and Immunology, Baylor College of Medicine.

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found only 15 major salivary gland BCACs and no minor salivary gland BCACs.45 The new case of minor salivary gland BCAC we report herein is of particular interest for its large tumor size, aggressive nature, and mixed histologic pattern. Furthermore, it brings the total number of minor salivary gland BCACs recorded in the literature to 72. Our aim in presenting this exhaustive literature review is to increase awareness of minor salivary gland BCAC, better characterize the disease, and provide a reference point for BCAC research in the future. Basal cell adenocarcinoma is a low-grade, infiltrative, locally destructive malignant neoplasm with a predilection for local recurrence that rarely metastasizes. It is treated with complete surgical excision and possible adjuvant radiation therapy. The patient age at the time of presentation, rates of recurrence and metastasis, and prognosis of minor salivary gland BCAC appear to be comparable to those of major salivary gland BCAC.2,8,12,27 Additional investigation of risk factors as well as cytogenetic analyses could provide valuable information regarding the specifics of BCAC development at a histopathologic level.

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14. Lopes MA, Santos GC, Kowalski LP. Multivariate survival analysis of 128 cases of oral cavity minor salivary gland carcinomas. Head Neck. 1998;20(8): 699-706.

21. Jayakrishnan A, Elmalah I, Hussain K, Odell EW. Basal cell adenocarcinoma in minor salivary glands. Histopathology. 2003;42(6):610-614. 22. Gross M, Maly B, Goldfarb A, Eliashar R. Basal cell adenocarcinoma in a buccal minor salivary gland. Acta Otolaryngol. 2004;124(2):213-216. 23. Toida M, Shimokawa K, Makita H, et al. Intraoral minor salivary gland tumors: a clinicopathological study of 82 cases. Int J Oral Maxillofac Surg. 2005; 34(5):528-532. 24. Poulopoulos AK, Andreades D, Epivatianos A, et al. Basal cell adenocarcinoma of the minor salivary gland: case report and cell adhesion molecules immunocytochemical profile. Oral Oncol Extra. 2005;41:150-153. 25. Ito FA, Ito K, Vargas PA, de Almeida OP, Lopes MA. Salivary gland tumors in a Brazilian population: a retrospective study of 496 cases. Int J Oral Maxillofac Surg. 2005;34(5):533-536. 26. Yamagata K, Oka K, Yoshida H, et al. Basal cell adenocarcinoma arising from the minor salivary

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gland in the soft palate: a case report. Pathol Res Pract. 2006;202(6):475-480. 27. Parashar P, Baron E, Papadimitriou JC, Ord RA, Nikitakis NG. Basal cell adenocarcinoma of the oral minor salivary glands: review of the literature and presentation of two cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2007;103(1):77-84. 28. Subhashraj K. Salivary gland tumors: a single institution experience in India. Br J Oral Maxillofac Surg. 2008;46(8):635-638.

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29. Ward BK, Seethala RR, Barnes EL, Lai SY. Basal cell adenocarcinoma of a hard palate minor salivary gland: case report and review of the literature. Head Neck Oncol. 2009;1(1):41.

35. Venkata V, Irulandy P. The frequency and distribution pattern of minor salivary gland tumors in a government dental teaching hospital, Chennai, India. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2011;111(1):e32-e39.

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2), Fascicle 10. Washington, DC: Armed Forces Institute of Pathology; 1974. 40. Ellis GL, Gnepp DR. Unusual salivary gland tumors: Basal cell adenocarcinoma. In: Gnepp DR, ed. Pathology of the Head and Neck. New York, NY: Churchill Livingstone; 1988:617-623. 41. Barnes L, Eveson JW, Reichart P, Sidransky D, eds. Pathology and genetics of tumors of the head and neck: World Health Organization Classification of Tumors. Vol 9. Lyon: IARC Press; 2005. 42. Farrell T, Chang YL. Basal cell adenocarcinoma of minor salivary glands. Arch Pathol Lab Med. 2007;131(10):1602-1604. 43. Williams SB, Ellis GL, Auclair PL. Immunohistochemical analysis of basal cell adenocarcinoma. Oral Surg Oral Med Oral Pathol. 1993;75(1):64-69. 44. Warrick PD, Irish JC, Mancer K, Dardick I, Pynn BR, Gullane P. Basal cell adenocarcinoma: a rare malignancy of the salivary glands. J Otolaryngol. 2000;29(2):102-109. 45. Tian Z, Li L, Wang L, Hu Y, Li J. Salivary gland neoplasms in oral and maxillofacial regions: a 23-year retrospective study of 6982 cases in an eastern Chinese population. Int J Oral Maxillofac Surg. 2010;39(3):235-242.

39. Thackray AC, Lucas RB. Tumors of the major salivary glands. In: Atlas of Tumor Pathology (Series

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Minor salivary gland basal cell adenocarcinoma: a systematic review and report of a new case.

Basal cell adenocarcinoma (BCAC) of the minor salivary gland is an extremely rare disease: the most recent substantive literature review reports only ...
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