CASE REPORT

Adenoid cystic carcinoma of the maxillary tuberosity and palate:

A case report and a review of the literature.

Arjay Nino S. Dulay

Department of Dentistry, University of the Philippines – Philippine General Hospital, Manila Philippines
Univeristy of the East, College of Dentistry, Manila Philippines

KEYWORDS Summary Adenoid cystic carcinoma of the salivary gland is a

Carcinoma; slow growing malignant tumor characterized by wide local
Adenoid cystic; infiltration perinueral spread and a propensity for local recurrence.
Maxillary Approximately 50 to 70% of reported cases occur in the minor
Tuberosity; salivary glands. The most common location is the palate, generally
the area of the greater palatine foramen. Adenoid cystic carcinoma
tends to have a protracted clinical course with wide infiltration and
late distant metastases. In this paper, the proponent present 57-
year-old female patient complained of a slowly growing mass in the
left maxillary and palatal region of 3 year duration.

Introduction gland including lacrimal glands, mucous

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.2 In 1928 it was
renamed adenoid cystic carcinoma (ACC) and
since then has been the generally accepted
term for this neoplasm.3
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.14,18 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.18 ACC has previously been
shown to occur at a variety of anatomic sites
in addition to the major and minor salivary
glands of the aerodigestive tract, skin, and
breast. The nose and paranasal sinuses
represent the most common sites for minor
gland ACCs.12 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.4 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 of pain and numbness and tingling sensation
parotid may exhibit symptoms of facial nerve on the area.
paralysis. The extraoral examination was within
The three recognized histopathologic normal limits with no evidence of
patterns of adenoid cystic carcinoma are lymphadenopathy. Intraoral examination
cribiform, tubular, and solid with cribiform revealed swelling on the left maxillary ridge
being the most common and easily recognized which extends from the area of the 2nd
pattern and solid the least common premolar to the maxillary tuberosity;
histopathologic subtype. The cribiform involving the palatal area but not crossing the
subtype of ACC is thought to have the best midline. Swelling is fim with color same as the
prognosis and the solid subtype the worst sorrounding; with ulceration and
with the tubular form possessing an telangiectasia of the overlying mucosa (Figure
intermediate prognosis.4,8,9,10,16,17,21 Often, 1). The patient was partially dentate but was
more than one histopathologic pattern is not wearing dentures. The remainder of the
observed in a single neoplasm. All three intraoral examination was in normal limits.
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 Figure 1 Clinical image of the mass in
tubular patterns. In addition, occasional the left maxillary tuberosity and palatal region
comedonecrosis may also be observed which with prominent telangiectatic vessels and
is uncommonly observed in the other ulceration.
subtypes.
The patient subsequently underwent
an incisional biopsy (Figure 2). At gross
Case report examination, the specimen consisted of an
irregular pink-tan fragment of mucosal tissue
A 57-year-old female patient presented to the measuring 0.5 · 0.5 · 0.3 cm in greatest
dental infirmary for evaluation of a mass in dimension.
the left maxillary tuberosity and palatal
region. According to the patient 3 years prior
to consult she experienced intermittent pains
on the area, previously dentate with maxillary
molars. The molars were extracted secondary
to the complaint of pain. 2 years PTC, patient
complained pain and noticed a swelling on the
same region. Patient consulted another
dentist and was advised to have it biopsied;
clinical impression was residual cyst and
scheduled a biopsy to R/O TMD and Maxillary
Sinus Infection secondary to Dental infection.
Upon consultation patient revealed that she Figure 2 Incisional biopsy was
noticed that the swelling has slowly increased performed; tissue samples were gathered
in size, accompanied by occasional symptoms from two areas of the swelling.
 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). Tumor Island is
punctuated with multiple microcytic spaces
that divide the lobules into numerous
cylinders yeilding a “Swiss Cheese” or
Honeycomb appearance; the cylindrical
spaces contain mucin staining (Fig. 4). The
diagnosis was confirmed to be adenoid cystic
carcinoma. Patient was ultimately referred to
Department of Surgery for definitive
management. Further work up of the patient
revealed radiographic evidence of tumor
invasion involving of the maxilla. In addition,
there was neither clinical evidence of regional
nodal involvement nor metastatic disease.
Treatment consisted of wide surgical excision
and hemimaxillectomy of the left maxillary
region with post radiotherapy.
Figure 3 Low power photomicrograph
demonstrating tumor island punctuated with
multiple microcytic spaces that divide the
lobules into numerous cylinders yeilding a
“Swiss Cheese” or Honeycomb appearance.
Figure 4 High magnification
photomicrograph showing cylindrical spaces
containing mucin.
Discussion
Adenoid cystic carcinoma present a special
diagnostic and treatment challenge because of
its extraordinary nature. Most scientific
findings about ACC are still based on studies
with a small number of patients and there is
still lack of available information about the
clinical and behaviour as well as treatment
concepts and their results.10
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
corresponds to this case. Adenoid cystic
carcinomas of the minor glands have been
reported to have a worse prognosis that those
of the major salivary glands.15,5 This may be
explained by the fact that tumors of the minor
salivary glands can more readily infiltrate
extra glandular soft tissues and bone thereby
allowing for increased dissemination of the
tumor rendering complete excision more
problematic.6
In a study conducted by Kokemueller
et al. with 74 diseased patients, revealed an
overall survival rate at 5, 10, 15 years were at
71%, 54%, and 37% with mean overall
survival rate of 11.2 years. Distant failures
also occurred most frequently with 5 years
after treatment. In addition the study also
stated through experience, that tumors of the
nasal cavity and the maxillary sinuses are
usually detected with higher stages at the time
of diagnosis.
Several factors influence the survival
rates in patients suffering from ACC.
Histological patterns of the lesion such as the
glandular and tubular types are linked with
higher survival rates since they are more
differentiated than that of the solid type
counterpart.8 Consequently, Mastuba et al.
analysed 36 cases of ACC also reported that
the prognosis of tubular type was better than
that of the glandular type, while the solid type
was the worst with high metastatic rate.
Spiro et al. reported on 264 cases of
ACC of the salivary gland. ACC in the maxillary
antrum and submandibular gland tumors had
the worst prognosis. They explained the poor
prognosis by assuming that ACC often
extensively invaded the nerves, bones and
muscles and surgery could hardly secure a
radical cure.
Huang et al. reported that the rate of
involvement in the advanced clinical stages
was higher than in early clinical stages. The
study also demonstrated that perinueral
invasion was highest in the submandibular
and sublingual gland involvement. Vrielinck et
al. reported lower survival rate with nerve
invasion than those with out in a 5-year recall.
According to Huang et al. A degree of
invasion is not only expressed by nerve
involvement, but also of vessels, muscles and
bones. Tumor cells spread through narrow
spaces of the cancellous bone, nerves, vessels
or collagenous fibers but did not destroy the
bony trabeculae. This implies radiological
examination might not reveal invasion of the
bone. Thus radiographs cannot exclude the
involvement of bone.
Owing to the high degree of invasion,
wide resection remains to be the best
treatment. Huang et al. stated when ACC of the
palate has destroyed the posterior wall of the
maxillary sinus; radical resection of the tumor
should be carried out with inclusion of the
pterygoid plate, pterygopalatine canal and
greater palatine foramen.
Combined surgery and radiotherapy is
the management of choice. Ampil & Misra et al.
also found out that combined therapy reduced
the risk of recurrences as well as the rate of
metastasis.
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