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Open Access Highly Accessed Case report

Central Mucoepidermoid carcinoma of mandible – A case report and review of the literature

Deepti Simon, Thara Somanathan, K Ramdas and Manoj Pandey*

Author Affiliations

Departments of Surgical Oncology, Pathology and Radiation Oncology, Regional Cancer Centre, Trivandrum, Kerala, India

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World Journal of Surgical Oncology 2003, 1:1  doi:10.1186/1477-7819-1-1


The electronic version of this article is the complete one and can be found online at: http://www.wjso.com/content/1/1/1


Received:9 February 2003
Accepted:25 February 2003
Published:25 February 2003

© 2003 Simon et al; licensee BioMed Central Ltd. This is an Open Access article: verbatim copying and redistribution of this article are permitted in all media for any purpose, provided this notice is preserved along with the article's original URL.

Abstract

Background

Primary central mucoepidermoid carcinoma of jaws is a rare lesion comprising 2–3% of all mucoepidermoid carcinomas reported in literature.

Case presentation

The case presented here illustrates the hypothesis that its specific pathogenesis is unknown.

Conclusions

Mucoepidermoid carcinoma of the jaw is a rare tumour of unknown aetiology. Although about a 100 cases has been reported in literature, the speculation on its aetiopathogenesis has mainly centred on the pluripotential capabilities of the epithelial lining of odontogenic cysts.

Keywords:
Jaw tumours; intraosseous carcinoma; mucoepidermoid; odontogenic cyst

Background

Mucoepidermoid carcinoma is usually associated with salivary glands and comprises 5–10% of all salivary gland tumours [1-3]. In 1945, Stewart and associates described its mucous-secreting and epidermal cellular elements thus establishing it as a distinct pathologic entity [4]. Eversole reviewed 815 cases and found that of the major salivary gland tumours, 89.6% involved the parotid, 8.4% submandibular and 0.4% sublingual gland [1]. The palate was the most common site for minor salivary gland involvement, accounting for 41.1% of intraoral lesions [1].

Aberrant salivary gland neoplasms arising within the jaws as primary central bony lesions are extremely rare comprising 2–3% of all mucoepidermoid carcinomas reported [2]. Lepp in 1939 first reported an intraosseous mucoepidermoid carcinoma of the mandible in a 66-year-old woman [5] and Bhaskar [6] in 1963 reported two cases discussing the criteria for their origin, histological composition and possible explanations for tumour pathogenesis. In 1991, after a systematic review of its histology and degree of differentiation the WHO classification recommended that the term "mucoepidermoid tumour" be changed to "mucoepidermoid carcinoma"[7]. Waldron and Mustoe [8] suggested that intraosseous mucoepidermoid carcinoma be included in primary intraosseous carcinoma of jaw as type 4 (Table 1) [9]. A thorough review of the English literature revealed about 100 reported cases of mucoepidermoid carcinoma arising in mandible [2,3,10,11]. We report here another case of central mucoepidermoid carcinoma within the bony mandible with no initial connection to alveolus.

Table 1. Classification of Primary Intraosseous Carcinoma

Case presentation

A 67-year-old hypertensive woman sought treatment at a local hospital for a painful swelling in the right posterior mandible of three weeks duration. 9-years previously she had undergone tooth extraction and cyst enucleation in the same region. The swelling was diffuse and bony hard involving the ramus and body from the region of the 2nd molar onwards. There was expansion of buccal and lingual cortices and paraesthesia of ipsilateral tongue. Cervical lymphadenopathy was absent. Panoramic radiograph of the mandible revealed an irregular, lobulated, expansile osteolytic lesion 5 cm in size, extending from the mesial aspect of the 2nd molar to the ramus and coronoid process posteriorly (Figure 1).

thumbnailFigure 1. OPG of the mandible showing the lytic lesion in the body and ramus of the mandible.

With a provisional diagnosis of ameloblastoma a hemimandibulectomy was performed. The gross examination of resected specimen revealed a brownish black soft tissue mass expanding both cortices and involving the ramus, coronoid process and body till the region of 2nd molar.

Microscopic examination revealed a neoplasm composed predominantly of cystic spaces and an epidermoid component in a fibrous stroma (Figure 2). The cystic spaces were of varying sizes, lined by mucous secreting cells and cells of intermediate type (figure 3). Nests and strands of squamous cells formed the epidermoid component. Mucinous material was seen within the cystic spaces and extravasating into the stroma (Figure 4).

thumbnailFigure 2. Mucoepidermoid carcinoma, mandible. Cystic spaces (C) and nests of neoplastic squamous (N) cells along with bone spicules at the periphery (B). (H&E × 50)

thumbnailFigure 3. Mucoepidermoid carcinoma. Cystic spaces lined by mucous secreting cells (M) and intermediate cells (I). (H&E × 400).

thumbnailFigure 4. Mucoepidermoid carcinoma. Nest of neoplastic squamous cells (N) along with cystic spaces. (H&E × 400).

With a diagnosis of mucoepidermoid carcinoma with predominant mucinous component she was referred to Regional Cancer Centre, Thiruvananthapuram for further management. A histopathological review reconfirmed the diagnosis and patient was treated with adjuvant external beam radiotherapy at a dose of 45 Gy over 20 fractions. She is on regular follow-up and is disease free after 2-years.

Discussion

Central mucoepidermoid carcinoma affects females twice more frequently than males and involves the mandible twice more often than the maxilla [3]. The most common site of occurrence is the premolar-molar-angle region of the mandible [2,3]. It has been reported in all ages ranging from 1 to 78-years, with the overwhelming majority occurring in 4th and 5th decades of life [3]. Eversole et al., [1] found approximately 50% of the mandibular tumours associated with dental cysts and/or impacted teeth, while Brookstone and Huvos [10] reported a rate of 32%.

In the children the sex ratio is similar to that in adults although the mandible to maxilla ratio is 1:1 [3]. As its occurrence in children is rare, it is unlikely to be a developmental disturbance or a teratoma [3]. The tumour shows a tendency to crop up at puberty, which might suggest a hormonal influence on salivary glands [3].

The main symptoms are swelling and pain with trismus, paraesthesia and tooth mobility being noted occasionally [10]. Previous history of a cyst or impacted tooth gives credence to the theory that odontogenic epithelium is capable of giving rise to mucous secretory cells which may undergo neoplastic transformation to mucoepidermoid carcinoma [12]. The radiographic features are usually a well circumscribed unilocular / multilocular radiolucency [13].

The authenticity regarding its central origin is greater when the tumour is in the mandible, since such lesions in maxilla may actually arise from the submucosal mucous secreting glands in the antrum, or represent intraosseous extension of minor salivary gland tumours of the sinus mucosa [14].

Speculation abounds regarding its pathogenesis and four possible origins have been described [4]. 1) entrapment of retromolar mucous glands within the mandible, which subsequently undergo neoplastic transformation; 2) developmentally included embryonic remnants of the submaxillary gland within the mandible; 3) neoplastic transformation of the mucous secreting cells commonly found in the pluripotential epithelial lining of dentigerous cysts associated with impacted third molars; and 4) neoplastic transformation and invasion from the lining of the maxillary sinus. Our patient gave a prior history of cyst enucleation in the same region as the tumour, which may indicate the possibility of neoplastic transformation of the cyst wall into a malignant nonodontogenic tumour.

The most commonly accepted criteria for diagnosis proposed by Alexander et al., [15] and modified by Browand and Waldron [16] is detailed in table 2. Our case had expanded, yet intact cortices without any radiographic evidence of bone destruction. A thorough search made for a primary tumour elsewhere by detailed clinical and other diagnostic methods proved futile. The histopathological picture was typical of the lesion and intracellular mucin could be detected.

Table 2. Diagnostic Criteria

Brookstone and Huvos [10] had put forward a staging system based on condition of the overlying bone. Lesions with intact cortical plates with no evidence of bony expansion offer the best prognosis and indicate stage I disease. Stage II disease is surrounded by intact cortical bone that has undergone some degree of expansion. Any instance of cortical perforation, breakdown of the overlying periosteum or nodal spread is best categorized clinically as stage III disease. The case presented here showed expanded, but intact buccal and lingual cortices and could be imputed as stage II disease. Metastases are reported in 9% of central mucoepidermoid carcinomas mainly to the regional lymph nodes [10,13] and occasionally to the ipsilateral clavicle, [17] lung and brain.

Surgery is the mainstay of treatment. In a review of 64 patients, Brookstone and Huvos observed 40% recurrences after conservative surgical modalities like curettage, enucleation, marsupialization and marginal resection with or without adjuvant therapy, whereas in the group treated by radical methods such as segmental resection with / without treatment of associated neck and / or adjuvant therapy only 4% recurred [10]. Adjuvant radiotherapy is recommended for high-grade tumours [13].

The clinical significance of malignant tumours arising from odontogenic cysts or de novo should never be underestimated as illustrated by the present case. This re-emphasises the importance of radical surgery, adjuvant treatment and a careful histopathological evaluation of all excised tissue so that such neoplastic transformation may be identified and treated effectively.

Competing interests

None declared.

Author contributions

DS participated in the preparation of the manuscript and literature search. TS carried out the histopathological studies, participated in the preparation of manuscript and approval of final version; MP carried out the literature search, edited the manuscript for its scientific content and prepared the final version of the manuscript; RD participated in the editing of the manuscript and preparation of final version of manuscript.

References

  1. Eversole LR: Mucoepidermoid Carcinoma: Review of 815 reported cases.

    Oral Surg Oral Med Oral Pathol 1970, 28:490-495. PubMed Abstract OpenURL

  2. Gingell JC, Beckerman T, Levy BA, Snider LA: Central mucoepidermoid carcinoma. Review of literature and report of a case associated with an apical periodontal cyst.

    Oral Med Oral Surg and Oral Pathol 1984, 57:436-440. PubMed Abstract OpenURL

  3. Ezsias A, Sugar AW, Milling MAP, Ashley KF: Central mucoepidermoid carcinoma in a child.

    J Oral Maxillofac Surg 1994, 52:512-515. PubMed Abstract OpenURL

  4. Shafer WG, Hine MK, Levy BM: Text book of Oral Pathology.

    Philadelphia W B Saunders 3rd edition. 1974, 225-231. OpenURL

  5. Lepp H: Zur Kenntnis des papillar wachsenden schleimigen cystadenokarzinoms der mundhohle.

    Zieglers Beitrage Z Pathol Anat 1939, 102:164-166. OpenURL

  6. Bhaskar SN: Central mucoepidermoid tumors of the mandible. Report of 2 cases.

    Cancer 1963, 16:721-726. PubMed Abstract OpenURL

  7. Seifert G, Sobin LH: Histological typing of salivary gland tumours. In In International Classification of tumours. 2nd edition. New York Springer-Verlag; 1991. OpenURL

  8. Waldron CA, Mustoe TA: Primary intraosseous carcinoma of the mandible with probable origin in an odontogenic cyst.

    Oral Surg Oral Med Oral Pathol 1989, 67:716. PubMed Abstract OpenURL

  9. Thomas G, Mathew A, Pandey M, Abraham EK, Francis A, Somanathan T, et al.: Primary intraosseous carcinoma of the jaw. Report of two new cases and pooled analysis of world literature.

    Int J Oral Maxillofac Surg 2001, 30:349-55. PubMed Abstract | Publisher Full Text OpenURL

  10. Brookstone MS, Huvos AG: Central salivary gland tumors of the maxilla and mandible: a clinicopathologic study of 11 cases with an analysis of the literature.

    J Oral Maxillofac Surg 1992, 50:229-236. PubMed Abstract | Publisher Full Text OpenURL

  11. Maremonti P, Califano L, Mangone GM, Zupi A, Guida C: Intraosseous mucoepidermoid carcinoma. Report of a long-term evolution case.

    Oral Oncol 2001, 37:110-113. PubMed Abstract | Publisher Full Text OpenURL

  12. Eversole LR, Sabes WR, Rovin S: Aggressive growth and neoplastic potential of odontogenic cysts.

    Cancer 1975, 35:270-282. PubMed Abstract OpenURL

  13. Freje JE, Campbell BH, Yousif WJ, Clowry LJ: Central mucoepidermoid carcinoma of the mandible.

    Otolaryngol Head Neck Surg 1995, 112:453-456. PubMed Abstract | Publisher Full Text OpenURL

  14. Waldron CA, Koh ML: Central mucoepidermoid carcinoma of the jaws: report of four cases with analysis of the literature and discussion of the relationship to mucoepidermoid, sialodontogenic, and glandular odontogenic cysts.

    J Oral Maxillofac Surg 1990, 48:871-877. PubMed Abstract OpenURL

  15. Alexander RW, Dupuis RH, Holton H: Central mucoepidermoid tumor (carcinoma) of the mandible.

    J Oral Surg 1974, 32:541-547. PubMed Abstract OpenURL

  16. Browand BC, Waldron CA: central mucoepidermoid tumors of the jaws.

    Oral Surg Oral Med Oral Pathol 1975, 40:631-643. PubMed Abstract OpenURL

  17. Lebsack JP, Marrogi AJ, Martin SA: Central mucoepidermoid carcinoma of the jaw with distant metastasis. A case report and review of the literature.

    J Oral Maxillofac Surg 1990, 48:518-522. PubMed Abstract OpenURL