Mucoepidermoid carcinoma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Badria Munir M.B.B.S.[2] , Maria Fernanda Villarreal, M.D. [3]

Overview

Mucoepidermoid carcinomas arise from mucous cells, which are normally involved in the secretion of mucous and the protection of the surrounding tissue. The pathogenesis of mucoepidermoid carcinoma consists of abnormal production of mucin from mucous cells, associated with the aberrant overgrowth of squamous and epidermoid cells. Genes involved in the pathogenesis of mucoepidermoid carcinoma include the MECT1 and MAML2 fusion genes. On gross pathology, mucoepidermoid carcinomas have a cystic, solid or mixed appearance, are normally located on the parotid or submandibular gland, and range in size from 1 to 8 cm.

Pathogenesis

Genetics

  • Development of mucoepidermoid carcinoma may be the result of multiple genetic mutations.
  • Mucoepidermoid carcinoma is specifically associated with t(11;19)(q21;p13) translocation.[4][5]

Associated Conditions

Human cytomegalovirus (hCMV) infection is commonly seen associated with development of mucoepidermoid carcinoma.

  • As HCMV is commonly resides in salivary gland ductal epithelium, It is hypothesized that HCMV could be integral part of pathogenesis of mucopidermoid carcinoma.
  • Following results were noted when they tested implication of HCMV in development

Gross Pathology

  • On gross pathology, mucoepidermoid carcinoma has a cystic, solid, or mixed appearance.
  • Mucoepidermoid carcinoma usually occurs in the parotid or submandibular gland.
  • Low grade mucoepidermoid carcinoma are small and partially encapsulated.
  • It appears as fluctuant light blue or purplish submucosal lumps. [12]
  • Other findings on gross pathology include:
  • Tumor size ranging from 1 to 8 cm
  • Gray or white in color, with mucin filled cysts

Microscopic Pathology

The characteristic findings of mucoepidermoid carcinoma on microscopic histopathological analysis are listed below.[13][9][14][1][15][16]

  • Mucus secreting cells
  • Composed of three cell types:
    • Epidermoid
    • Intermediate
    • Columnar
    • Clear cells
  • They all demonstrates prominent cystic growth. [9]
  • Mucin vacuoles may be rare; however they are integral part of histological features of mucoepidermoid carcinoma.
  • Hallmark of these tumors are:
    • Prominent cystic structures lined by mature mucous, intermediate, or epidermoid cells
    • Solid areas are not evident
    • Prominent fibrous stroma
    • It grows in a well-circumscribed manner
  • Mucoepidermoid tumors are graded histologically into:
    • Low grade
    • Intermediate grade
    • High grade.
  • Low grade is characterized by well-differentiated cells with little cellular atypia, high proportion of mucous cells, and prominent cyst formation.
  • Intermediate grade is characterized by intermediate features
  • High grade is characterized by poorly differentiated with cellular pleomorphism, high proportion of squamous cells, and solid with few if any cysts

Gallery

References

  1. 1.0 1.1 Devaraju R, Gantala R, Aitha H, Gotoor SG (August 2014). "Mucoepidermoid carcinoma". BMJ Case Rep. 2014: bcr–2013–202776. doi:10.1136/bcr-2013-202776. PMC 4127757. PMID 25085946.
  2. Ali SA, Memon AS, Shaik NA, Soomro AG (2008). "Mucoepidermoid carcinoma of parotid presenting as unilocular cyst". J Ayub Med Coll Abbottabad. 20 (2): 141–2. PMID 19385480.
  3. Pires FR, Chen SY, da Cruz Perez DE, de Almeida OP, Kowalski LP (May 2004). "Cytokeratin expression in central mucoepidermoid carcinoma and glandular odontogenic cyst". Oral Oncol. 40 (5): 545–51. doi:10.1016/j.oraloncology.2003.11.007. PMID 15006629.
  4. Chen Z, Lin S, Li JL, Ni W, Guo R, Lu J, Kaye FJ, Wu L (April 2018). "CRTC1-MAML2 fusion-induced lncRNA LINC00473 expression maintains the growth and survival of human mucoepidermoid carcinoma cells". Oncogene. 37 (14): 1885–1895. doi:10.1038/s41388-017-0104-0. PMC 5889358. PMID 29353885.
  5. Yan K, Yesensky J, Hasina R, Agrawal N (February 2018). "Genomics of mucoepidermoid and adenoid cystic carcinomas". Laryngoscope Investig Otolaryngol. 3 (1): 56–61. doi:10.1002/lio2.139. PMC 5824110. PMID 29492469.
  6. Seethala RR, Dacic S, Cieply K, Kelly LM, Nikiforova MN (August 2010). "A reappraisal of the MECT1/MAML2 translocation in salivary mucoepidermoid carcinomas". Am. J. Surg. Pathol. 34 (8): 1106–21. doi:10.1097/PAS.0b013e3181de3021. PMID 20588178.
  7. Okabe M, Miyabe S, Nagatsuka H, Terada A, Hanai N, Yokoi M, Shimozato K, Eimoto T, Nakamura S, Nagai N, Hasegawa Y, Inagaki H (July 2006). "MECT1-MAML2 fusion transcript defines a favorable subset of mucoepidermoid carcinoma". Clin. Cancer Res. 12 (13): 3902–7. doi:10.1158/1078-0432.CCR-05-2376. PMID 16818685.
  8. Cheuk W, Chan JK (July 2007). "Advances in salivary gland pathology". Histopathology. 51 (1): 1–20. doi:10.1111/j.1365-2559.2007.02719.x. PMID 17539914.
  9. 9.0 9.1 9.2 Luna MA (November 2006). "Salivary mucoepidermoid carcinoma: revisited". Adv Anat Pathol. 13 (6): 293–307. doi:10.1097/01.pap.0000213058.74509.d3. PMID 17075295.
  10. Alamri AM, Liu X, Blancato JK, Haddad BR, Wang W, Zhong X, Choudhary S, Krawczyk E, Kallakury BV, Davidson BJ, Furth PA (January 2018). "Expanding primary cells from mucoepidermoid and other salivary gland neoplasms for genetic and chemosensitivity testing". Dis Model Mech. 11 (1). doi:10.1242/dmm.031716. PMC 5818080. PMID 29419396.
  11. Melnick, Michael; Sedghizadeh, Parish P.; Allen, Carl M.; Jaskoll, Tina (2012). "Human cytomegalovirus and mucoepidermoid carcinoma of salivary glands: Cell-specific localization of active viral and oncogenic signaling proteins is confirmatory of a causal relationship". Experimental and Molecular Pathology. 92 (1): 118–125. doi:10.1016/j.yexmp.2011.10.011. ISSN 0014-4800.
  12. Flaitz CM (2000). "Mucoepidermoid carcinoma of the palate in a child". Pediatr Dent. 22 (4): 292–3. PMID 10969433.
  13. Vulpe H, Giuliani M, Goldstein D, Perez-Ordonez B, Dawson LA, Hope A (October 2013). "Long term control of a maxillary sinus mucoepidermoid carcinoma with low dose radiation therapy: a case report". Radiat Oncol. 8: 251. doi:10.1186/1748-717X-8-251. PMC 3829377. PMID 24165756.
  14. Maloth AK, Nandan SR, Kulkarni PG, Dorankula SP, Muddana K (December 2015). "Mucoepidermoid Carcinoma of Floor of the Mouth - A Rarity". J Clin Diagn Res. 9 (12): ZD03–4. doi:10.7860/JCDR/2015/15595.6912. PMID 26813873.
  15. Ritwik P, Cordell KG, Brannon RB (July 2012). "Minor salivary gland mucoepidermoid carcinoma in children and adolescents: a case series and review of the literature". J Med Case Rep. 6: 182. doi:10.1186/1752-1947-6-182. PMID 22759529.
  16. Joseph TP, Joseph CP, Jayalakshmy PS, Poothiode U (2015). "Diagnostic challenges in cytology of mucoepidermoid carcinoma: Report of 6 cases with histopathological correlation". J Cytol. 32 (1): 21–4. doi:10.4103/0970-9371.155226. PMC 4408672. PMID 25948939.
  17. 17.0 17.1 17.2 17.3 Mucoepidermoid carcincoma Libre Pathology. http://librepathology.org/wiki/index.php/Mucoepidermoid carcincoma Accessed on February 17, 2015

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