Diagnosis and treatment for Central Mucoepidermoid Carcinoma in mandible:

report of a clinical case in a young patient.

Abstract

A clinical case of a 13-year-old male patient with a 2-year malignant tumor in the

mandibular body area and associated pain is reported. It is initially diagnosed as

low-grade central mucoepidermoid carcinoma using Hematoxylin and Eosin (H-E)

staining in multiple biopsies. Considering the clinical and radiographic

characteristics of the lesion, it was necessary to confirm the diagnosis using

periodic acid–Schiff (PAS). The treatment involved hemimandibulectomy, neck

emptying and complementary radiotherapy. The aim of this article is to present a

rare occurrence of this type of intraosseous malignant tumor of glandular origin in a

young patient.

Introduction

The World Health Organization defines Mucoepidermoid Carcinoma (MEC) as a

distinctive malignancy of mucinous, intermediate (clear cells) and squamous tumor

glands leading to cystic and solid patterns (1). It was first described in the mandible

of a 66-year-old woman (2). Later, two cases discussing the criteria of its origin,

histological composition and possible explanations for tumor pathogenesis were

published (3). Later on, in 1991 (4), the term mucoepidermoid carcinoma was

suggested. In 2008, MEC was included in the category of intraosseous lesions and

its differential diagnosis was established under clinical, histological and radiological

characteristics (5).
The most common occurrence of MEC is in the parotid gland, followed by the

palate, the submandibular gland, and other intraoral areas such as the minor

salivary glands. Primary intraosseous (central) MECs are rare in young people (1).

A recent review presented 147 cases and only five of them involved patients under

15 years of age (6). The aim of this article is to show how this pathology can

develop in young people and in a low-prevalence area, which makes the clinical

and histopathological diagnosis difficult.

Case

13-year-old male patient with a 2-year clinical case related to enlargement of the left

hemimandibula, mild pain, edema and induration; signs and symptoms are

associated with delayed eruption of the ipsilateral posterior molar.

During the extraoral physical examination, facial asymmetry was observed as a

result of swelling in the left mandibular body and angle, presence of bilateral,

mobile, submandibular adenopathies of less than 1 cm (figure 1a). Intraorally, a

cortical expansion in the left retromolar area with mild pain upon palpation of the

lingual plate and a stony consistency in an area covered by healthy mucosa was

observed (figure 1b).

In the initial panoramic radiograph, a multiloculated radiolucent lesion in the left

hemimandibula compromising the entire mandibular ramus and extending to the

mandibular symphysis without cortical erosion or root resorption was observed.

Additionally, the third molar was found to be in Nolla’s stage 6, displaced, and

retained in the coronoid process. The lesion extended from the canine area (tooth
33), the mandibular body and ramus to the coronoid process and condyle (figure

1c).

Three presumptive diagnoses were established according to clinical and

radiographic characteristics, age, anatomical location, associated structures and

evolution of the lesion: ameloblastoma, keratocyst and dentigerous cyst. For the

initial treatment of the patient, computerized axial tomography (CT) scan of the face

with 3D reconstruction, fine-needle aspiration biopsy, incisional biopsy,

decompression and histopathological study were prescribed.

In the 3D CT scan report, the pediatric radiologist reported a bone lesion with cystic

appearance and epicenter in the left hemimandibula compromising the condylar

neck, ramus, angle and hemibody, associated with cortical thinning and some areas

of hemimandibular disruption in the inner region (figure 1d); thus confirming

differential diagnoses: ameloblastoma, aneurysmal bone cyst and dentigerous cyst.

In the first intervention under local anesthesia, fine-needle aspiration of 3 cc of

citrine fluid was performed, followed by hematic fluid flow. Additionally, an incision

with a scalpel blade No. 15 was performed in the area of tooth 37. After periosteal

stripping, a thinning of the vestibular plate was observed; capsule and bone

samples were taken for histopathological study and the surgical area is left to drain

for a week.

The histopathological study reported proliferation of neoplastic epithelial cells with a

biphasic pattern: cells with broad clear cytoplasm and others with eosinophilic

cytoplasm and squamous appearance, arranged in cohesive layers, in a

vascularized stroma. There was little nuclear pleomorphism and very scarce

mitosis. No differentiation to odontogenic tissue was observed, leading to the

diagnosis of low-grade central mucoepidermoid carcinoma.

However, since the diagnosis did not coincide with the clinical characteristics and

evolution of the pathology, a second incisional biopsy of the vestibular plate, the

bone of the mandibular ramus and the intermediate bone was performed. The

histopathological study of these three samples reported the same diagnosis. The

pathologist suggested performing immunohistochemistry for CK 5/6, CK7, P63,

Ki 67.

In view of the above, a medical-surgical board was held at the maxillofacial surgery

service of Hospital Universitario San Vicente de Paul; the pathologist suggested

performing a periodic acid–Schiff (PAS) procedure in order to determine mucin

production, since it is a reliable confirmatory test and it is more affordable than

immunohistochemistry. The PAS staining results were positive (figure 1e), so in the

view of this broadly studied, low-grade central mucoepidermoid carcinoma

diagnosis, surgical management of the patient was recommended along with

general surgery; hemimandibulectomy, bilateral suprahomoid draining of lymph

nodes and mandibular and condylar reconstruction (figure 2 a,b,c).

Given that there was compromised medullary bone in the edge of the sectioned

mandibular block, treatment was complemented with radiotherapy. The patient

underwent clinical and radiographic monitoring for one year, without signs of new

lesions (figures 2d and 2e).

Discussion

Central or intraosseous mucoepidermoid carcinoma is an uncommon but

well-known neoplasm that affects the mandible and may be confused with other

cysts and tumors (7). The pathogenesis of central MEC remains unclear; however,

several theories have been reported (8-12).

In this case, a 13-year-old boy was diagnosed with a low-grade central MEC.

However, a high prevalence of this type of tumor was recently reported in people

between their fifties and sixties (with an average of 46.5 years). Then, it rarely

occurrs in teenagers; only three cases have been documented in patients younger

than 13 years (6).

Six criteria must be considered for the diagnosis of central MEC (13, 14). Although

cortical integrity is one of them, the central origin within the bone may be conditional

in the absence of prominent peripheral soft tissue despite cortical perforation (15).

Additionally, this report follows recently proposed guidelines for reporting of MEC

cases (10).

Regarding its clinical characteristics, unlike other malignant jaw tumors, central

MEC originates mainly as a cyst or as a benign tumor. The most frequent sign is

painless edema, which usually takes months or even years to evolve and may

involve facial asymmetry; clinical and radiographic diagnosis is therefore difficult. It

may also be associated with teeth displacement and malocclusion, which coincides

with this presentation. Paresthesia of the inferior alveolar nerve and dissemination

to lymph nodes have also been described. Its location is twice as frequent in the
mandible as it is in the maxilla (usually in the region of the premolars and molars);

few cases have been reported in the anterior region (14, 15).

Radiographically, it originates as a unilocular or multilocular mass with delimited

and well corticated margins. Cases reporting cortical disruption are rare; however,

its expansion is distinctive. The teeth are usually not affected by radicular

resorption, but the adjacent plate may indeed be modified (15).

Histologically, MEC is a malignant epithelial tumor formed by a variable proportion

of mucosal, epidermoid, intermediate, columnar and clear cells frequently

associated with a cystic component (6). On the other hand, the overall 10-year

survival rates for low, intermediate and high grade MEC are 90 %, 70 % and 25 %

respectively (6).

The basis of central MEC treatment is surgery with safety margins. Radical

treatment, such as segmental resection with and without adjuvant therapy, reports a

recurrence rate of 4 % (6). Considering these recurrence rates, this case was

managed with radical treatment involving hemimandibulectomy with disarticulation,

emptying of ipsilateral omohyoid lymph nodes, reconstruction with titanium plate

and articular prosthesis, which helped preserve functionality and symmetry in the

patient. Additionally, the patient underwent 30 radiotherapy sessions, as

recommended (6).

Central MEC metastasis occurs in 2 % of cases. It metastasizes mainly to regional

lymph nodes, the ipsilateral clavicle, the lungs and the brain (6).

Conclusion
MEC is a rare condition in teenagers; nevertheless, an early diagnosis is very

important to initiate treatment as soon as possible. Treatment involving radical

resection of the lesion offers a good scenario.

Acknowledgements

To doctor Leonor Gonzalez, specialist in oral pathology, for her collaboration

performing the PAS staining and taking the microscopic photographs.

This research received no specific grant from any funding agency in the public,

commercial, or non-profit sectors.

Conflicts of interest

The authors note that there are no conflicts of interest.

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Figures

Figure 1a. Facial asymmetry

Figure 1b. Expansion of bone plates

Figure 1c. Multilocular radiolucent lesion from mandibular parasinphysis to condylar

neck and left coronoids

Figure 1d. Bone cortical expansion with disruption zones

Figure 1e. PAS staining positive for mucin 40X.

Figure 2a. Jaw block removed

Figure2b. Mandibular reconstruction plate

Figure 2c. Immediate post-operative

Figure 2d One-year postoperative

Figure 2e One-year postoperative