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Case Reports in Dentistry

Volume 2021, Article ID 8853649, 6 pages
https://doi.org/10.1155/2021/8853649

Case Report
Polymorphous Adenocarcinoma: A Rare Case Report with Unique
Radiographic Appearance on CBCT

Jagadish Chandra,1 Junaid Ahmed ,2 K. M. Veena,3 M. Vijayakumar,4 Nandita Shenoy ,2

and Nanditha Sujir2
1
Dept. of Oral Surgery, Yenepoya Dental College, Yenepoya University, Deralakatte, India
2
Dept. of Oral Medicine and Radiology, Manipal College of Dental Sciences, Mangalore, Manipal Academy of Higher
Education (MAHE), Manipal, Karnataka 576104, India
3
Dept. of Oral Medicine and Radiology, Yenepoya Dental College, Yenepoya University, Deralakatte, Karnataka, India
4
Department of Surgical Oncology, Yenepoya Medical College, Yenepoya University, Deralakatte, Mangalore, India

Correspondence should be addressed to Junaid Ahmed; [email protected]

Received 29 May 2020; Revised 24 February 2021; Accepted 15 March 2021; Published 25 March 2021

Academic Editor: Sukumaran Anil

Copyright © 2021 Jagadish Chandra et al. This is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is
properly cited.

Background. Polymorphous low-grade adenocarcinoma (PLGA) is a slow growing malignant tumor of minor salivary glands and is
generally of indolent nature. However, according to the most recent WHO Classification of Salivary Gland Tumors (2017), the
cancer is classified as Polymorphous AdenoCarcinoma (PAC). PAC presents as a less aggressive tumor, though it could on rare
occasions demonstrate distant metastasis. Case Presentation. A 47-year-old man who was referred by a private practitioner for a
CBCT scan in reference to a proliferative soft-tissue growth in the hard palate. The growth was mild and tender and there was
Grade III mobility in relation to all the maxillary teeth. Panoramic radiograph taken previously had revealed evidence of
alveolar bone loss in relation to the maxillary teeth and was inconclusive of any other findings. The CBCT scan revealed
evidence of moth-eaten appearance of maxilla with destruction of medial and lateral walls and floor of maxillary sinus. There
was also evidence of involvement of right eustachian tube, ethmoidal wall, and nasopalatine canal. An intraosseous malignancy
of the palate was suspected, and a total maxillectomy was performed. The tissue sample was sent for histopathological
assessment wherein changes diagnostic for polymorphous low-grade adenocarcinoma of the palate were observed. Conclusion.
PAC is a distinct, yet commonly occurring, minor salivary gland tumor with varied clinical and histologic appearance. This case
report highlights the importance of CBCT in diagnosing the intraosseous involvement of such tumors which can help shed
some light in enhancing our knowledge about the minor salivary gland malignancies like PAC.

1. Background minor salivary glands, before Evans and Batsakis [3] catego-
rized them as a separate histologic entity in 1984. The World
Polymorphous low-grade adenocarcinoma (PLGA) is a slow Health Organization later categorized PAC as a separate cat-
growing malignant tumor of minor salivary glands and is egory of tumors of minor salivary glands in 1990 [4].
generally of indolent nature. However, according to the most Due to its architecturally diverse structure, clinically
recent WHO Classification of Salivary Gland Tumors (2017), indolent behavior, and cytological uniformity, PAC is
the cancer is classified as Polymorphous AdenoCarcinoma thought to be a unique entity [5, 6]. Being asymptomatic with
(PAC) [1]. Among all the tumors of oral salivary glands, oral a low metastatic potential, it is commonly seen in elder
salivary gland carcinomas comprise only 20% of the malig- women, with about 24% of primary tumors associated with
nant neoplasms [2]. Lobular carcinoma, terminal duct carci- major salivary glands (i.e., parotid, submandibular, or sublin-
noma, and low-grade papillary adenocarcinoma were the gual gland) and few in locations other than the head and
various names that were used for adenocarcinomas of the neck, such as the breast and vagina [7, 8].
2 Case Reports in Dentistry

Although the third most commonly presenting malig-

nant salivary gland tumor, our knowledge about PAC
remains restricted to published case reports. Also, the archi-
tectural diversity associated with the name can be confusing
for histopathological evaluation and can potentially be a
diagnostic dilemma. Salivary gland neoplasms can be aggres-
sive, and most of their clinical features often overlap; hence, a
detailed examination and clinical investigation should be car-
ried out in order to reach a definitive conclusion. Radiology is
an important diagnostic tool for salivary gland pathology. It
helps to determine the extent of involvement of bony struc-
tures, which can provide foresight into the treatment plan- Figure 1: Evidence of diffuse, proliferative soft tissue swelling in the
ning and provide a valuable tool in assessing the clinical region of the hard palate.
picture. Cone Beam Computed Tomography (CBCT) is use-
ful in the assessment of osseous structures and can help the
clinician to determine the aggressive nature of the tumor as
well as bony involvement due to a clear image of structures
that are highly contrasted, and in comparison to most 2-D
imaging methods, CBCT can provide a three-dimensional
image of structures of the head and neck [9]. The following
case report describes a unique case where a patient reported
with the complaint of a soft-tissue growth in relation to the
maxilla. This case report is aimed at highlighting the impor-
tance of CBCT in the detection of malignancies of salivary
gland tissues, especially those cases with extensive bony
involvement.

2. Case Presentation Figure 2: Evidence of erythema of the attached gingiva in relation to

maxillary posterior teeth. The clinical features resembled a
A 47-year-old man was referred to the Department of Oral periodontal pathology.
Medicine & Radiology with a history of soft tissue growth
in the palate for the past two months. The growth was sudden
in onset, growing rapidly in size over the next two months ate was suspected (Figures 4–9). The patient was then
and localized to the palate. The patient reported no pain dur- scheduled for surgery with excision biopsy and total maxil-
ing the onset of growth. However, for the past one week, the lectomy up to the floor of orbit.
patient reported mild pain. On clinical examination, there The surgical procedure consisted of visualization of hard
was a diffuse, proliferative soft tissue growth of the maxilla and soft palate and incision extending laterally around the
extending from the marginal gingiva in the palatal aspect of maxillary tuberosity exposing the posterior border. Follow-
the anterior tooth and involving the entire surface of the hard ing exposure of the maxillary tuberosity, attachment to the
palate up to the level of the hard and soft palate junction soft palate and hard palate was divided. After soft tissue dis-
along with erythema of attached gingiva in relation to poste- section up to the extension of the tumor, the extent of the
rior teeth palatally and buccally as well as Grade III mobility bone which was tailored to the primary tumor including
of all the anterior and posterior maxillary teeth (Figures 1 the lateral wall of the orbit and zygoma to be resected was
and 2). The cervical group of lymph nodes were nonpalpable marked. Following bone guttering, the bony resection was
clinically. carried out using a chisel and mallet through the frontal pro-
The referring private practitioner had advised a pano- cess of the maxilla and lacrimal bone. Bleeding encountered
ramic radiograph earlier which revealed severe bone loss in during this procedure from the greater palatine artery and
relation to maxillary teeth suggestive of generalized peri- the branches of internal maxillary artery was controlled using
odontitis and was inconclusive for any other relevant find- pressure packing followed by ligation to achieve haemostasis.
ings except for a root stump in the lower right quadrant After mobilizing the segment, final osteotomy was done to
(Figure 3). separate the maxillary tuberosity from the pterygoid plates.
The patient was advised for a CBCT scan which revealed Before removal, thorough clinical examination of the speci-
a moth-eaten appearance of the palate noticed bilaterally men was done to determine the adequacy of the tumor
with involvement of nasopalatine canal anteriorly and exter- (Figures 10(a) and 10(b) and 11(a) and 11(b)).
nal resorption of roots in relation to all the maxillary teeth The tissue sample was sent for histopathological exami-
present, with evidence of involvement of anterior and middle nation which revealed an unencapsulated tumor within the
ethmoidal sinus, eustachian tube, and nasopharynx on the underlying connective tissue approximating the surface epi-
right side. Based on CBCT and clinical findings, a prelimi- thelium, with a varied pattern of arrangement of tumor cells
nary diagnosis of intraosseous malignancy involving the pal- that included solid nests, strands, ducts, and tubular and
Case Reports in Dentistry 3

Figure 3: Panoramic radiograph of the patient revealing evidence of interdental bone loss in relation to all the maxillary teeth.

Figure 4: Coronal section of CBCT scan revealing destruction of

walls of maxillary sinus along with involvement of nasopalatine
canal.
Figure 7: Sagittal section of CBCT revealing destruction of hard
palate.

Figure 5: Axial view of CBCT scan revealing moth-eaten

appearance of maxilla involving the hard palate and maxillary bone.

Figure 8: Axial view of CBCT scan revealing involvement of

anterior and middle ethmoidal air sinus on the right side.

papillary patterns and was lined by 1-2 layers of oval/cuboi-

dal cells along with a peripheral layer of flat cells suggestive
of polymorphous low-grade adenocarcinoma of the palate
(Figures 12, 13, and 14). The patient is currently under
follow-up for the past six months and is asymptomatic.

3. Discussion and Conclusion

Figure 6: 3D reconstructed CBCT image revealing widespread Clinically, PAC presents as a firm, well-circumscribed, pain-
destruction of maxilla and resorption of all maxillary teeth root less, and slow growing mass mostly covered by nonulcerated
apices. mucosa resembling a benign neoplasm and could be fixed to
underlying structures, eroding and infiltrating the underlying
4 Case Reports in Dentistry

(a)

Figure 9: Axial view of CBCT scan revealing narrowing and

involvement of eustachian tube on the right side.

(b)

Figure 11: (a, b) Postsurgical maxillectomy specimen.

(a)

(b)
Figure 12: H and E section of tissue sample showing
Figure 10: (a, b) Maxillectomy procedure done under local
unencapsulated tumor close to surface epithelium with tumor cells
anesthesia.
arranged in solid nests.

bone, and is even present with perivascular and perineural

invasion. Metastasis when reported is mostly confined to
the regional nodes while spread to distant sites is a rarity
and so is the transformation of this low-grade entity into a
high-grade one [10].
Immunohistochemistry (IHC) has been utilized in a
number of case series to distinguish between PAC and
ACC, but there is a considerable overlap. Past studies con-
ducted regarding the expression of ki-67 have consistently
reported a very low labelling index (LI) in PAC which can
assist in differentiating it from adenoid cystic carcinoma
(ACC) [11–14]. In a few studies, p40 immunostaining has
been demonstrated as a new tool for distinguishing salivary Figure 13: H and E sections showing ductal pattern of arrangement
gland tumors with true myoepithelial differentiation from of tumor cells with evidence of vesicular nuclei.
those showing nonspecific p63 expression. When these
immunostains are performed in tandem, a discordant noma, which generally show p63/p40 concordance [15].
p63/p40-immunophenotype can reliably distinguish PAC Genetic abnormalities may be useful in differential diagnosis
from both adenoid cystic carcinoma and pleomorphic ade- of PAC. PACs harbor PRKD1 mutations in >70% cases, and
Case Reports in Dentistry 5

Adjuvant radiotherapy has been indicated in cases where

there is metastasis of cervical lymph nodes. A wide excision
has been shown to minimize the rate of recurrence in cases
of PAC, and radical excision has been suggested for the
recurrent cases [22].

3.1. Summary. Minor salivary gland malignancy is an

uncommon occurrence in daily practice. However, being
the third most common minor salivary gland malignancy, it
is necessary for the diagnostician to be well-versed with the
clinical, histopathological, and radiographic features of
PGLA. Although the knowledge available on this entity is
limited, it is necessary to be able to segregate it from the mis-
Figure 14: H and E sections showing arrangement of tumor cells in cellaneous tumors of minor salivary glands in order to pro-
tubular pattern in the given tissue specimen. vide appropriate treatment for the patient. Due to its
tendency for recurrence, it is important to regularly follow-
up the patient posttreatment. The importance of radiography
PRKD1 mutations appear to be pathognomonic for this especially 3D imaging in the form of CBCT in dental practice
entity. In contrast, a vast majority of adenoid cystic carcino- is highlighted in this case report as we have noticed that even
mas harbor MYB or MYBL1 gene rearrangements. While still though salivary gland malignancies usually limit themselves
not used widely in the clinic, use of these genetic markers to soft tissue involvement, there are cases wherein extensive
may greatly facilitate the correct diagnosis [16]. involvement of bone with a varied radiological presentation
The differential diagnosis for PAC includes pleomorphic can be observed.
adenoma and adenoid cystic carcinoma. PAC presents with a PAC is a distinct, yet commonly occurring, minor sali-
mucoid to the hyalinized matrix in comparison to pleomor- vary gland tumor with varied clinical and histologic appear-
phic adenoma which presents with a chondromyxoid matrix. ance. This case report highlights the importance of CBCT
Also, when compared to pleomorphic adenoma, PAC pre- in diagnosing the intraosseous involvement of such tumors
sents with perineural invasion. Another differentiating fea- which can help shed some light in enhancing our knowledge
ture between the two is that PAC stains positive for S-100 about the minor salivary gland malignancies like PAC.
and epithelial membrane antigen (EMA) whereas pleomor-
phic adenoma stains positive for glial fibrillary acidic protein
(GFAP). When compared to adenoid cystic carcinoma, stain- Abbreviations
ing is positive for p-53, ki-67, bcl-2, and CD117. Perineural
PAC: Polymorphous low-grade adenocarcinoma
invasion is a feature of both PAC and adenoid cystic carci-
CT: Computed tomography
noma, but the presence of the targetoid arrangement of peri-
CBCT: Cone beam computed tomography
neural invasion is characteristic of PAC [17]. Also, in
ACC: Adenoid cystic carcinoma
comparison to PAC, the cells of adenoid cystic carcinoma
PA: Pleomorphic adenoma
are smaller with hyperchromatic and angulated nuclei, less
IHC: Immunohistochemistry
cytoplasm, and a coarser nuclear chromatin [18].
LI: Labelling index
In the published literature, radiographic imaging of PAC
EMA: Epithelial membrane antigen
has been mainly restricted to CT imaging since they provide
GFAP: Glial fibrillary acidic protein.
a better soft-tissue contrast when compared to CBCT [19].
However, according to our knowledge, only a few case
reports have highlighted the importance of CBCT in diagno-
Data Availability
sis of PGLA which describes it as presenting with no evidence
of bone scalloping/erosion [20]. The radiographic appear- The datasets generated and/or analysed during the current
ance of PAC in our study resembled that of osteomyelitis study are not publicly available for the protection of privacy
and intraosseous malignancy. However, osteomyelitis of jaws of the patient but are available from the corresponding
is more common in the mandible when compared to the author on request.
maxilla. Also, osteomyelitis of jaws is preceded most com-
monly with an identifiable cause of odontogenic infection
such as a decayed tooth which was absent in our case. Ethical Approval
Intraosseous malignancies also present with a poorly defined,
moth-eaten appearance. However, they occur more fre- Ethics approval for the case report is not applicable as this is
quently in the mandibular posterior sections compared to not a study but a report of a single case. The patient’s confi-
the maxilla [21]. Also, these tumors reveal changes suggestive dentiality has been maintained in the manuscript. Our uni-
of carcinoma in the microscopic sections as defined by Suei versity rules do not mandate or require ethics approval for
et al. for a definitive diagnosis of intraoral malignancy. case reports.
6 Case Reports in Dentistry

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The authors declare that there are no competing interests
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