Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 28 (2016) 6165

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Journal of Oral and Maxillofacial Surgery,

Medicine, and Pathology
journal homepage: www.elsevier.com/locate/jomsmp

Case Report

Central mucoepidermoid carcinoma in a young patient:

A case report and review of the literature
G. Del Corso a, , A. Pizzigallo b , C. Marchetti b , A. Tarsitano b
a

Department of Biomedical and Neuromotor Sciences, Section of Oral Science, University of Bologna, Italy
Department of Biomedical and Neuromotor Sciences, University of Bologna, Section of Maxillo-facial Surgery at Policlinico S. Orsola-Malpighi, Bologna,
Italy
b

a r t i c l e

i n f o

Article history:
Received 27 January 2015
Received in revised form 14 June 2015
Accepted 22 July 2015
Available online 28 August 2015
Keywords:
Mucoepidermoid carcinoma
Central oral lesions
Salivary glands neoplasm

a b s t r a c t
Background: Central mucoepidermoid carcinoma is a rare malignant salivary neoplasm arising inside
the bone of the jaws. We report a rare case of central mucoepidermoid carcinoma of a 16-year-old girl
arising in the maxilla. Clinical and radiological analyses were shown and discussed, as well as the surgical
treatment.
Methods: A left maxillectomy from the last molar to the rst premolar was performed, and the site was
reconstructed using a bula free ap stabilized with titanium plates.
Results: The young patient was followed up for more than 6 years, and to date no recurrence was observed.
A literature review of the 11 rare previous cases was provided to guide the clinician in the diagnosis and
management of this unusual glandular tumor.
Conclusion: It is of immense importance to differentiate the central mucoepidermoid from other osteolytic
lesion and odontogenic cysts because of his malignancy and local aggressiveness. The treatment option
is the radical excision, with the evaluation of neck nodes, and radiotherapy is only recommended in the
most aggressive of cases.
2015 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved.

1. Introduction
Mucoepidermoid carcinoma is the most frequent malignant
neoplasm of the salivary glands, effecting those with a mean age
of 45 years old [1]. It occurs frequently in major salivary glands,
especially the parotid, and in salivary glands of the palate and
buccal mucosa [1]. The jaws cortical bone is sometimes involved
ab estrinseco by mucoepidermoid carcinoma where the tumor
makes contact with the bone. On rare occasions, mucoepidermoid
carcinoma can be identied as a total intra-bony lesion without soft tissue involvement. This unusual pathological entity is
called central mucoepidermoid carcinoma (CMC). It is a very rare
variant of mucoepidermoid carcinoma representing 24% of all
cases/occurrences [2,3], and occurs in the fourth and fth decade,

especially in the maxilla of women [4]. However, CMC has also been
reported in young patients (less than 18 years old). The pathogenesis, treatment and prognosis of CMC are still controversial due to
its rarity. This case report describes a CMC arising in the maxilla
of a 16-year-old female patient diagnosed in December 2007 and
surgically treated in January 2008 at the Maxillo-Facial Unit of the
S. Orsola-Malpighi Hospital of the University of Bologna, Italy. The
patient was followed up for 6 years. The 11 previous cases of CMC
arising in young patients (less than 18 years old) reported in the
literature from 1952 to 2014 are described, summarizing the main
characteristics of this rare malignant variant of mucoepidermoid
carcinoma [514].

2. Case report

AsianAOMS: Asian Association of Oral and Maxillofacial Surgeons; ASOMP: Asian

Society of Oral and Maxillofacial Pathology; JSOP: Japanese Society of Oral Pathology; JSOMS: Japanese Society of Oral and Maxillofacial Surgeons; JSOM: Japanese
Society of Oral Medicine; JAMI: Japanese Academy of Maxillofacial Implants.
Corresponding author at: Department of Biomedical and Neuromotor Sciences,
University of Bologna, via S Vitale 59, Bologna, Italy.
E-mail address: [email protected] (G. Del Corso).

On December 2007, a 16-year-old white female patient came to

our attention due to an occasional radiological nding of left maxilla lesion. The young patient did not report any symptoms. There
was neither precedent toothache nor trauma. Clinical examination
revealed the presence of a swelling in the palate that was painless
and the overlying buccal mucosa was intact (Fig. 1). The expansive
central lesion caused the swelling of the mucosa. The mucosa was

http://dx.doi.org/10.1016/j.ajoms.2015.07.008
2212-5558/ 2015 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved.

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G. Del Corso et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 28 (2016) 6165

intact with blue-red color mimic a mucocele or a vascular lesion.

Neck evaluation was negative for lymph-node involvement. The
panoramic radiograph revealed a well-dened radiolucent lesion
close to the premolars and rst molar roots (Fig. 2).

Fig. 3. Computed-tomography scan showing a multilocular radiolucency area of the

left maxilla.

Fig. 1. Intraoral swelling of the palate caused by the expansion of the central lesion.

The computed-tomography (CT) scan revealed an expansive

multilocular intra-bony radiolucency area of the left maxilla, without extra-bony spread (Figs. 3 and 4). Based on clinical and
radiological features the differential diagnosis could be an odontogenic cyst, brosseous lesions or a keratocystic odontogenic tumor.
An incisional biopsy was performed resulting in the diagnosis
of a mucoepidermoid carcinoma.
A left maxillectomy from the last molar to the rst premolar
was performed (Fig. 5). The specimen consisted of a rm and solid
mass, with cystic spaces and no necrotic areas. The radical excision
of the tumor was conrmed by the pathologist. The surgical gap was
simultaneously reconstructed using an osteomuscolar vascularized
bula free ap stabilized with titanium plates (Fig. 6). The muscular
component of the ap was used to reconstruct the oral mucosal
layer. Gradual and complete mucosal metaplasia was recorded in
the intra-oral component of the ap during the follow-up. Fig. 7
shows the photomicrographs stained with hematoxylin and eosin
at low magnication. Microscopically, the specimen was composed
of mucous and epidermoid cells in a brous connective stroma and
bone. Cells show positive mucin staining, and it was excluded a
histopathologically similar primary lesion of salivary glands.
The denitive diagnosis was central mucoepidermoid carcinoma at intermediate grade of malignancy. Two years after

Fig. 4. Coronal CT scan revealing the tumor inside the maxillary bone without extracortical spread.

Fig. 2. Panoramic radiograph showing a well-dened radiolucent area in the left maxilla.

G. Del Corso et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 28 (2016) 6165

63

the surgical treatment, rehabilitation with two dental implants

inserted into the bula ap was performed and an implant-xed
prosthesis was carried out (Fig. 8).
The patient was followed up every six months, and no sign
of recurrence was identied until July 2014. The protocol was
reviewed and approved by the Institutional Review Board and
informed consent was obtained.

Fig. 7. Histological features of intermediate-grade central mucoepidermoid carcinoma (H&E, original magnication).

Fig. 5. The specimen of the tumor with cystic areas.

3. Discussion
CMC represents a rare variant of the mucoepidermoid carcinoma, and to date more than 130 cases have been reported in
literature. However, the pathogenesis of the tumor is not yet well
dened. Some articles suppose that CMC develops from remnant of
the dental lamina, or from an entrapment of minor salivary glands,
others suggest a neoplastic transformation of the epithelial lining of
an odontogenic cyst or the neoplastic transformation of the epithelium of the maxillary sinus [3,4,15]. We suppose an origin from an
ectopic salivary gland tissue because the epithelial lining of the
maxillary sinus is well-circumscribed by the maxillary bone [16].

CMC occurs on average in the fourth and fth decades of life;

it is more common in female patients and occurs especially in the
mandible [14]. The clinical aspect is not representative. In fact, the
mucosa can be intact and normal in color, or with blue-red color
mimicking a mucocele or a vascular lesion. The mucosa can appear,
sometimes, ulcerated [4]. The main symptom is the swelling that in
some cases is associated with pain, but CMC is frequently asymptomatic or accidentally discovered with a panoramic radiograph
[17], as in our case. Radiographically the tumor shows a unilocular
or multilocular radiolucency with intact cortical bones, with welldened or ill-dened margins [14]. A radiopaque aspect is very rare
for this tumor. Few cases show cortical destruction. In these cases
the correct diagnosis of CMC should be accurately assessed.
Pathologic features of CMC are very similar to the mucoepidermoid carcinoma of the salivary gland and may have cystic lesion,
or can be solid. Cystic lesions have a squamous epithelium mixed
with cells that produce mucus, and areas of intramural solid tumors
[18]. The histological features of CMC are not different from the
mucoepidermoid carcinoma. Thus, it is important to make a correct
diagnosis based on clinical and radiological data, and furthermore
with the following criteria [7,8]:

Fig. 6. Panoramic post-operative radiograph showing the left maxillectomy and the reconstruction performed using a single segment of bula free ap.

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G. Del Corso et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 28 (2016) 6165

Fig. 8. Final implant-prosthetic rehabilitation. The image shows: the surgical insert of the implants into the bula ap (A); panoramic radiograph (B); clinical and radiological
control after implant xed prosthesis rehabilitation (C, D).

preservation of cortical bone;

presence of a radiographic bone lesion;
exclusion of origin from salivary gland or odontogenic tumor;
the mucous membrane over the tumor is not involved;
histopathological conrmation.

Based on these criteria, a clinical staging system is described to

guide the clinician. Stage I of CMC presents an intact cortical bone
without signs of cortical expansion; Stage II are lesions that surround the cortical bone with some degree of expansion; the Stage III
is characterized by a perforation of cortical bone and of the periosteum or metastatic nodal spread [7]. In consideration of this staging
system, our case corresponds to the stage II.
The treatment choices for CMC are difcult to establish due to
the rarity of the tumor and the inadequacy of staging. However, the
treatment of choice should be radical excision of the tumor comprehensive of bone and surrounding mucosa removal with clear
margins. The neck should be dissected only if positive. According to data published we believe that surgical curettage does not
represent the correct treatment choice due to the high possibility of local recurrence. Indeed, the recurrence rate is about 13% in

the tumors treated with radical excision, while the curettage presented a higher percentage of recurrence ranging from 40 to 45%
[7,19]. However, in young patients no recurrence is reported in
a median follow-up of 6 years. Mucosa adjacent to the involved
bone should be removed. Distant metastasis of CMC is seen in
few cases representing no the 6% of them [19,20]. Only one case
shows distant metastases to the brain and lungs without lymph
node metastases [16]. The histological grade does not correlate with
prognosis; in fact low-grade CMC should be treated with radical
surgical resection. The neck dissection and adjuvant treatment are
still controversial [14].
Table 1 summarizes the 12 cases of CMC of the jaws described
in literature from 1952 to 2014 in young patients. The 42% of CMC
occurs in males and the 58% in females. The age ranges from 1 to
16 years, with a median age of 13.75 years. CMC occurs in the same
percentage in maxilla and in the mandible. The palate and the body
of the mandible were the most involved area. Few articles reported
the state of the radiological margins. Only two authors described
the margins of radiolucency. The radiological aspect diagnosed at
the CT scan reveals a radiolucent aspect with unilocular or multilocular areas. Only one case was treated with radiotherapy and

Table 1
List of the 12 cases and features of CMC in young patients (less than 18 years old) reported in the literature until 2014 [514].
Case

Gender/age

Location

Radiological appearance

Treatment

Follow-up

Author

Year

1
2
3
4
5
6
7

F/15
F/16
F/1
M/15
M/10
M/11
M/15

Hard palate, maxilla

Palate, maxilla
Mandible body
Maxilla
Mandible body
Mandible angle
Mandible body

Curettage
Maxillectomy
Marsupialization
Maxillectomy
Radiotherapy
Mandibulectomy
Mandibulectomy

Died 16 years later

3 years no recurrence
N/A
20 years no recurrence
N/A
4 years no recurrence
2 years no recurrence

Hertz
Chaundhry et al.
Bhaskar
Browand et al.
Brookstone et al.
Ezsias et al.
Baj et al.

1952
1961
1963
1975
1992
1994
2002

8
9

M/14
F/11

Mandible body
Maxilla

Cohen-Kerem et al.
Namin et al.

2004
2005

F/16

Mandible body

2.5 years recurrence

Zhou et al.

2012

11
12

F/15
F/16

Palate, maxilla
Hard palate, maxilla

Mandibulectomy
Hemimaxillectomy
Partial
mandibulectomy/radiotherapy
Curettage
Maxillectomy

<1 year
N/A

10

Unilocular, radiolucent
Multilocular, radiolucent
N/A
Radiolucent
Unilocular, radiolucent
Multilocular, radiolucent
Unilocular, radiolucent,
well-dened margins
Unilocular, radiolucent
Radiolucent, unilocular,
well-dened margins
N/A

5 years recurrence
6 years no recurrence

Zhou et al.
Present case

2012
2014

N/A
Multilocular, radiolucent,
well-dened margins

G. Del Corso et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 28 (2016) 6165

one with surgery and radiotherapy, while two cases were treated
with curettage and one with marsupialization. The main follow-up
period without recurrence is 7 years, and only two cases present a
recurrence, after 2.5 and 5 years of follow-up.
CMC of the adult does not differ from CMC of the young patient
in terms of histological diagnosis and radiographic appearance.
However, in the adult the CMC is four times more common in
the mandible compared to the maxilla, while in young patients
mandible and maxilla are equally involved. The exact pathogenesis remains still unclear in both types of patients. The criteria for
diagnosis are the same as well as the best treatment of choice is the
surgical resection. However, in older patients 7 cases of regional
metastasis to the neck are reported, while no one is reported in
young patients [19].
4. Conclusions
CMC is considered a low malignant carcinoma with a local
aggressiveness. It is a very rare variant of the mucoepidermoid
carcinoma representing the 24% of cases. The criteria for diagnosis are clinical and radiological, in addition to the histological
conrmation. CMC is usually radiolucent, while mixed radiolucentradiopaque are more rare. CMC mimics other osteolytic lesion and
odontogenic cysts, thus it is important to know the correct diagnostic criteria. CMC in young patients is rare and it has a median
age of about 14 years, with a same predilection for both jaws. The
treatment option is the radical excision, with the evaluation of neck
nodes, and radiotherapy is only recommended in the most aggressive of cases. The tumor rarely presents recurrence with a median
follow-up period of 6 years.
Compliance with ethical standards
No conict of interest and funding are declared.
Informed consent was obtained from all individual participants
included in the study.
All procedures performed in studies involving human participants were in accordance with the ethical standards of the
institutional and/or national research committee and with the 1964
Helsinki declaration and its later amendments or comparable ethical standards.
Acknowledgements
The authors thank Ban Camilleri from the International Vocational College of Malta for English revision on the manuscript.

65

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