giant cystic primary mucoepidermoid carcinoma of mandible: a rare case and literature review

OTPOL-130; No. of Pages 5

Case report/Kazuistyka

Giant cystic primary mucoepidermoid carcinoma ofmandible: A rare case and literature review

Roshan Kumar Verma 1,*, Satheesh Kumar Sunku 1, Amanjeet Bal 2,Naresh K. Panda 1

1Department of Otolaryngology and Head & Neck Surgery, Postgraduate Institute of Medical Education and Research,Chandigarh, India2Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

o t o l a r y n g o l o g i a p o l s k a x x x ( 2 0 1 3 ) x x x – x x x

a r t i c l e i n f o

Article history:

Received: 14.11.2013

Accepted: 28.11.2013

Available online: xxx

Keywords:� Primary mucoepidermoid

carcinoma� Giant cystic� Embryonic cell rest

a b s t r a c t

Primary intra-osseous mucoepidermoid carcinoma arising from jaw is an extremely rare

condition accounting to less than 2% of all mucoepidermoid carcinomas. In the jaw, it

occurs more commonly in mandible than maxilla. They are low-grade cancers and affect

jaw as uni- or multi-locular radiographic lesions. Here we discuss a rare case of giant

cystic primary intra-cystic mucoepidermoid carcinoma of the mandible which was exci-

sed in toto. Here we discuss the clinical features, radiological and histological characte-

ristics of this rare lesion, and review the literature.

© 2013 Polish Otorhinolaryngology - Head and Neck Surgery Society. Published by

Elsevier Urban & Partner Sp. z o.o. All rights reserved.

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Introduction

Mucoepidermoid carcinoma (MEC) was first described byAlexander et al. [1], as salivary gland tumor which accountsto 5–10% of all salivary gland tumors, involving mainlyparotid gland (86%), followed by submandibular gland (8%)and sublingual glands (0.4%) [2]. Minor salivary glands ofpalate are the next most common site for these tumors.

Mucoepidermoid carcinoma arising from aberrant salivarytissue within the jaw bones is extremely rare, accounting to2–4% of all mucoepidermoid carcinomas [3, 4]. These tumorspresent as Primary Intra Osseous Carcinomas affecting

* Corresponding author at: Department of Otolaryngology and Head &Tel.: +91 172 2747586–94x6764; fax: +91 172 2744401.

E-mail address: [email protected] (R.K. Verma).

Please cite this article in press as: Verma RK, et al. Giant cystic primaryreview. Otolaryngol Pol. (2013), http://dx.doi.org/10.1016/j.otpol.2013.11.00

0030-6657/$ – see front matter © 2013 Polish Otorhinolaryngology - Head and Neck Su

http://dx.doi.org/10.1016/j.otpol.2013.11.004

mandible more commonly than maxilla [5]. Lepp (1939) firstreported an intra-osseous mucoepidermoid carcinoma of themandible in a 66-year-old woman [6]. Bhaskar (1963) [7]reported two cases discussing the criteria for their origin,histological composition and possible explanations for tumorpathogenesis. Review of the English literature showed notmore than 110 cases of mucoepidermoid carcinoma of thejaws reported till date. Cystic mucoepidermoid carcinomas ofjaws are low-grade tumors and have favorable prognosis withen bloc resection.

Here we report a giant cystic variant of mucoepidermoidcarcinoma of the mandible in a 35-year-old male patientwhich caused a diagnostic confusion and was excised en bloc.

Neck Surgery, PGIMER, Chandigarh 160012, India.

mucoepidermoid carcinoma of mandible: A rare case and literature4

rgery Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.


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Table I – Waldron and Mustoe modification of WHOclassification of PIOC

Type 1 PIOC ex odontogenic cystType 2a Malignant ameloblastomaType 2b Ameloblastic carcinoma arising de novo, ex

ameloblastoma or ex odontogenic cystType 3 PIOC arising de novo(a) Keratinizing type(b) Non-keratinizing typeType 4 Intraosseous mucoepidermoid carcinoma

Fig. 1 – The preoperative extent of lesion

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Case report

A 35-year-old male presented to the outpatient clinic withcomplaints of slowly progressive painless swelling in leftmandibular region for 3 years. There was no history ofdifficulty in swallowing or breathing and no history ofaddictions was present. There was no history of trauma tothe mandible, or history of dental extraction or loosening ofteeth. On examination, a solitary, irregular, 15/12 cm, non-tender, stony hard swelling fixed to mandible extendingfrom left ear lobule superiorly till thyroid cartilage inferiorly,from angle of mandible till midline was seen in Fig. 1. Oralcavity examination showed a mild bulge of the left tonsilmedially. Dentition was normal and there was no evidenceof loosening of teeth. The mucosa overlying the swellingwas intact.

Computed tomography showed a homogenously enhan-cing, multi-loculated, expansile, dumbbell-shaped soft tissuelesion involving left mandible from the coronoid process ofmandible till midline. Intra-oral bulge of the swelling wasseen into the oropharynx and the swelling was extendinginto the para-pharyngeal space. The submandibular glandwas pushed laterally (Fig. 2). A presumptive diagnosis ofameloblastoma of mandible was made.

Standard trans-cervical approach with lip splitting alongwith lateral mandibulotomy was done to dissect the tumorand get better exposure. Surprisingly the tumor was not at allinvolving the mandible except for its attachment to theperiosteum near upper part of the ascending ramus. Thetumor was resected in toto along with stripping of a smallportion of the periosteum, and minimal irregularity of theunderlying bone was seen which was smoothened by drillinghowever there was no frank erosion or destruction of thebone. Submandibular gland was normal and pushed laterally.The tumor was a large, irregular, well encapsulated, grayishpink mass with variable consistency of predominantly cysticto stony hard at place where it was adherent to the mandible


(Fig. 3). Mandible was re-fixed with small plates and screws,and the wound closed primarily.

Post-operative histopathological examination was done:grossly the tumor was in the form of a globular mass. Oncut section it was dominantly cystic with solid nodularareas. On microscopic examination the cystic areas showedfibro-collagenous wall lined by intermediate cells admixedwith mucous cells (Fig. 4). Solid areas also showed mixtureof intermediate and mucous cells. The tumor was infiltra-ting the underlying bone. There was absence of normalsalivary gland tissue component within the tumor. Overallfeatures were of muco-epidermoid carcinoma (MEC) cysticvariant of the jaw was made.

Post-operatively the patient was subjected to a radicaldose of external beam radiotherapy. At 3 months follow-upthere was no evidence of loco regional recurrence.

Discussion

Though rare, intra-osseous carcinoma arising in the jawbones is a well-known clinical entity and was first describedas a central epidermoid carcinoma by Loos in 1913 [8]. LaterPindborg, in the first edition of WHO classification of theodontogenic tumors based on histopathological differentia-tion, coined the name ‘‘Primary Intra Osseous Carcinoma’’[9]. The WHO defines Primary Intra Osseous Carcinoma(PIOC) of the jaw as a ‘‘squamous cell carcinoma arisingwithin the jaw’’, having no initial connection with the oralmucosa and presumably developing from residues of theodontogenic epithelium [9]. Later Waldron and Mustoe [10]suggested that intra-osseous mucoepidermoid carcinoma beincluded in the classification of PIOCs as type 4 (Table I).This was based on the fact that mucoepidermoid carcinomaof jaws was similar to salivary mucoepidermoid carcinomahistologically and it was thought to arise from epithelialremnants of the odontogenic cyst.

Being a rare entity Kochaji and colleagues [4] publishedguidelines proposed for the future generation of the diag-nosticians, surgical pathologists and radiologists for thediagnosis of primary mucoepidermoid carcinoma of jaws(Table II). The most important criteria for diagnosis ofprimary mucoepidermoid carcinoma of jaws are to excludeprimary lesion in the salivary glands. In our case the parotidand sub mandibular glands were normal on CT scan neckand histology showed no evidence of normal salivary glandmorphology, so a diagnosis of primary intra-osseous muco-pidermoid carcinoma was entertained.



Fig. 2 – CT scan showing homogenously enhancing, multi loculated, expansile, dumbbell-shaped soft tissue lesion involvingleft mandible

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Possible theories of pathogenesis and origin of mucoepi-dermoid carcinoma in jaw bones include: entrapment ofretromolar mucous glands within the mandible, which subse-quently undergo neoplastic transformation; developmentallyincluded embryonic remnants of the submandibular and

Fig. 4 – Photomicrograph of cystic area showing cyst wallcomprised of fibrocollagenous tissue and lined byintermediate tumor cells (H&E + 200). Inset:Photomicrograph from solid areas showing dominantlyintermediate cells admixed with few mucus cells (H&E+ 200)

Fig. 3 – Intraoperative photograph with mandibulotomy andexcised specimen removed in toto

Please cite this article in press as: Verma RK, et al. Giant cystic primary mucoepidermoid carcinoma of mandible: A rare case and literaturereview. Otolaryngol Pol. (2013), http://dx.doi.org/10.1016/j.otpol.2013.11.004


Table II – Diagnostic criteria proposed by Kochaji andcolleagues

1. Intact cortical plates (however, cortical perforation does notexclude PIOC type 4)

2. Radiographic evidence of bony destruction3. Exclusion of another primary tumor that in its metastasis

could histologically mimic the central tumor4. Exclusion of an odontogenic tumor5. Histopathological confirmation6. Detectable intracellular mucin

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sublingual gland within the mandible; neoplastic transforma-tion of the mucous secreting cells commonly found in thepluripotential epithelial lining of dentigerous cysts associatedwith impacted third molars; and neoplastic transformationand invasion from the lining of the maxillary sinus [11, 12]. Inour case the probable source is embryonic cell rests ofmucous salivary epithelium present in the periosteum ofthe mandible.

Primary mucoepidermoid carcinoma affecting the jawsoccurs most commonly in the 4th and 5th decades of lifethough it has been reported to occur in all age groups [13]. Itaffects females twice more frequently than males and invol-ves the mandible twice more often than the maxilla [14]. Inchildren the incidence is almost equal in both maxilla andmandible. The most common site of occurrence is thepremolar–molar–angle region of the mandible [15]. It isassociated with mandibular cysts in approximately 50% cases[2, 11]. As the tumor shows a tendency to crop up at puberty,hormonal influence of salivary glands was suggested as anetiological factor [16]. Painless swelling of the jaw is the mostcommon presentation, and occasionally presents with pain,paraesthesia, numbness and loosening of tooth [11].

Though radiological findings are not specific, it is neces-sary to know the extent of the lesion, and its relation to thevital structures as optic nerve and orbit (in case of maxillarylesions). Panoramic radiography is a simple and cost-effectiveimaging modality for all jaw lesions. CT scan is useful andgives exact extent of lesion and bony destruction and nodalstatus. Based on radiology a three grade classification hasbeen given by Brookstone and Huvos [11]: grade 1, withoutexpansion and rupture of cortical plate; grade 2, with expan-sion but without rupture of cortical plate; grade 3, withrupture of cortical plates or presence of regional metastasis.

Surgery is the treatment of choice in primary intra-osseous mucoepidermoid carcinoma and includes: curet-tage, wide resection, and hemi mandibulectomy or hemimaxillectomy with or without neck dissection dependingupon cervical nodal status. In our case, en bloc resection oftumor with drilling of inner cortical plate of mandible wasdone. Radiotherapy is usually given post operatively in caseof high grade tumors [3]. Prolonged follow up is required inview of risk of distant metastasis and local recurrence,though less evidence is available in the literature [16].

Conclusion

Primary mucoepidermoid carcinoma of mandible is a rareclinical entity with various possible theories of origin. This


diagnosis should be considered only when primary lesionsin the salivary glands have been excluded. The posteriorend of mandible is the most common site of involvement.CT scan is useful for diagnosis and to determine the extentof lesion. Surgery is the treatment of choice in such cases.Long follow-up is a must in such cases as late recurrencesare known to occur.

Authors' contributions/Wkład autorów

According to order.

Conflict of interest/Konflikt interesu

None declared.

Financial support/Finansowanie

None declared.

Ethics/Etyka

The work described in this article has been carried out inaccordance with The Code of Ethics of the World MedicalAssociation (Declaration of Helsinki) for experiments invol-ving humans; EU Directive 2010/63/EU for animal experi-ments; Uniform Requirements for manuscripts submitted toBiomedical journals.

r e f e r e n c e s / p i �s m i e n n i c t w o

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[4] Kochaji N, Goossens A, Bottenberg P. Centralmucoepidermoid carcinoma: case report, literature reviewfor missing and available guideline proposal for comingcase reports. Oral Oncol (EXTRA) 2004;40:95–105.

[5] Namin AK, Moshref M, Shahoon H, Mashhadi A, Khojasteh A.Intraosseous mucoepidermoid carcinoma of the maxilla in ateenager: a case report and review of literature. Oral SurgOral Med Oral Pathol Oral Radiol Endod 2005;100:E93–E96.

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[7] Bhaskar SN. Central mucoepidermoid tumors of themandible. Report of 2 cases. Cancer 1963;16:721–726.

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giant cystic primary mucoepidermoid carcinoma of mandible: a rare case and literature review

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