Mucoepidermoid carcinoma overview

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Mucoepidermoid Carcinoma from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Staging

History and Symptoms

Physical Examination

Laboratory Findings

X Ray

CT

MRI

Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

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

Overview

Mucoepidermoid carcinoma (also known as "Salivary gland mucoepidermoid carcinoma") is a malignant salivary gland tumor that consists of epidermoid and mucin producing cells. Mucoepidermoid carcinomas arise from mucous cells, which is normally involved in the secretion of mucous and the protection of surrounding tissue.[1] Mucoepidermoid carcinoma is the most common malignant salivary gland tumor, and usually occurs in the parotid and submandibular salivary glands. Mucoepidermoid carcinomas may be classified according to location into 2 subtypes: salivary gland confined carcinomas and other organ mucoepidermoid tumors.[2] The majority of these tumors present as parotid gland mucoepidermoid carcincoma lesions (65%). Development of mucoepidermoid carcinoma is the result of an abnormal production of mucin by mucus-secreting cells.[3] Genes involved in the pathogenesis of mucoepidermoid carcinoma include MECT1 and MAML2 fusion genes.[1] Mucoepidermoid carcinomas are usually found in middle aged adults between the second and sixth decade of life. Males are slightly more affected with mucoepidermoid carcinoma than females. The incidence of mucoepidermoid carcinoma is approximately 2.5 to 3.0 cases per 100,000 individuals a year.[4] If left untreated, progression occurs slow and is then followed by sentinel lymph node metastasis. The treatment choice for mucoepidermoid carcinoma is surgical removal.[1]

Historical Perspective

Mucoepidermoid carcinoma was first described by Masson and Berger, two American pathologists, in 1924.[5]

Classification

Mucoepidermoid carcinoma may be classified according to location into 2 subtypes: salivary gland-confined carcinomas and other organ mucoepidermoid carcinomas.

Pathophysiology

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.[6] On microscopic histopathological analysis, mucoepidermoid carcinomas are characterized by mucous cells with abundant fluffy cytoplasm and large mucin vacuoles.[6]

Causes

There are no established direct causes for mucoepidermoid carcinoma. The development of mucoepidermoid carcinoma has been associated with the result of multiple genetic mutations in the MECT1 and MAML2 fusion genes.

Differentiating Mucoepidermoid Carcinoma from other Diseases

Mucoepidermoid carcinoma must be differentiated from other diseases that cause painless swelling, facial deformity, and facial numbness, such as Warthin tumour, adenoid cystic carcinoma, benign mixed tumor, and metastasis.

Epidemiology and Demographics

Mucoepidermoid carcinoma is the most common malignant salivary gland tumor among general population. The incidence of mucoepidermoid carcinoma is approximately 2.5 to 3.0 cases per 100,000 individuals a year.[2] The incidence of mucoepidermoid carcinoma increases with age. The median age at diagnosis is between 20 to 50 years.[7] Males are slightly more affected with mucoepidermoid carcinoma than females. There is no racial predilection for mucoepidermoid carcinoma.[4]

Risk Factors

Common risk factors in the development of salivary gland tumors include: ionizing radiation and occupations associated with an increased risk such as rubber products manufacturing, asbestos mining, plumbing, and some types of woodworking.[8]

Screening

According to the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for mucoepidermoid carcinoma.[8]

Natural History, Complications and Prognosis

If left untreated, patients with mucoepidermoid carcinoma may progress to develop sentinel metastasis to adjacent lymph nodes. Common complications of mucoepidermoid carcinoma include facial deformity, difficulty swallowing, and local lymph node metastasis. Prognosis will generally depend on the clinical stage, tumor size, and histological grade. The overall recurrence rate will depend on the stage.[9] Low grade tumors have a 90-98% survival rate and a low rate of local recurrence.[10]

Diagnosis

Staging

According to the American Joint Committee on Cancer (AJCC) 7th edition, there are 6 stages of mucoepidermoid carcinoma based on the tumor size and node invasion. Each stage is assigned a letter and a number that designate the tumoral size and node invasion.[11]

History and Symptoms

The hallmark symptom of mucoepidermoid carcinoma is painless swelling. A positive history of cancer may be suggestive of mucoepidermoid carcinoma. Symptoms related with mucoepidermoid carcinoma will vary depending on the size and location of the tumor. Common symptoms of mucoepidermoid carcinoma may include facial numbness, adjacent muscle soreness, mandible claudication, otorrhea, dysphagia, and trismus.[12]

Physical Examination

Physical examination findings of mucoepidermoid carcinoma depend on the size and location of the tumor. Physical examination of patients with mucoepidermoid carcinoma is usually remarkable for tenderness and intraoral bluish-red mucosa.[7]

Laboratory Findings

There are no diagnostic laboratory findings associated with mucoepidermoid carcinoma.

X Ray

On conventional radiography, there are no characteristic findings of mucoepidermoid carcinoma.[2]

CT

On CT scan, characteristic findings of mucoepidermoid carcinoma include: well-circumscribed masses, usually with cystic components (low-grade tumors), enhancements of solid components, and calcification. High-grade tumors have poorly defined margins, infiltrate locally, and appear solid.[2]

MRI

On MRI, characteristic findings of mucoepidermoid carcinoma will depend on the stage. Common findings include: low signal cystic spaces in T1 and high signal in cystic areas on T2.[2]

Ultrasound

On ultrasound, characteristic findings of mucoepidermoid carcinoma include: a well-circumscribed hypoechoic lesion with a partial or completely cystic appearance, in contrast to the relatively hyperechoeic normal parotid gland.[2]

Other Imaging Findings

There are no other imaging findings associated with mucoepidermoid carcinoma.[2]

Other Diagnostic Studies

Other diagnostic studies associated with mucoepidermoid carcinoma may include: fine needle aspiration biopsy and incisional biopsy.[2]

Treatment

Medical Therapy

There is no medical treatment for mucoepidermoid carcinoma. Radiotherapy can be neoadyuvant treatment in some lesions (dependent on the tumor stage and size).

Surgery

Surgery is the mainstay of therapy for mucoepidermoid carcinoma.[11]

Primary Prevention

There is no primary prevention for mucoepidermoid carcinoma.

Secondary Prevention

There is no secondary prevention for mucoepidermoid carcinoma.

References

  1. 1.0 1.1 1.2 ealey, W. V., Perzin, K. H. and Smith, L. (1970), Mucoepidermoid carcinoma of salivary gland origin. Classification, clinical-pathologic correlation, and results of treatment. Cancer, 26: 368–388. doi: 10.1002/1097-0142(197008)26:2<368::AID-CNCR2820260219>3.0.CO;2-K
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 Mucoepidermoid carcinoma. Radiopedia. Dr Frank Gailliard. http://radiopaedia.org/articles/mucoepidermoid-carcinoma-of-salivary-glands Accessed on February 17, 2016
  3. Mucoepidermoid Carcinoma. Wikipedia. https://en.wikipedia.org/wiki/Mucoepidermoid_carcinoma Accessed on February 17,2016
  4. 4.0 4.1 Evans HL (1984). "Mucoepidermoid carcinoma of salivary glands: a study of 69 cases with special attention to histologic grading". Am. J. Clin. Pathol. 81 (6): 696–701. PMID 6731349.
  5. Foote FWJ, Frazell EL. Tumors of the major salivary glands. Atlas of Tumor Pathology, first series, Fascicle 11. Washington, DC: Armed Forces Institute of Pathology, 1954
  6. 6.0 6.1 Mucoepidermoid carcinoma. Libre Pathology. http://librepathology.org/wiki/index.php/Mucoepidermoid carcinoma Accessed on February 17, 2015
  7. 7.0 7.1 Eneroth, C.-M. (1971), Salivary gland tumors in the parotid gland, submandibular gland, and the palate region. Cancer, 27: 1415–1418. doi: 10.1002/1097-0142(197106)27:6<1415::AID-CNCR2820270622>3.0.CO;2-X
  8. 8.0 8.1 Mucoepidermoid carcinoma. Salivary Gland Cancer Treatment–for health professionals (PDQ®).http://www.cancer.gov/types/head-and-neck/hp/salivary-gland-treatment-pdq#link/_403_toc Accessed on February 17, 2016
  9. Plambeck K, Friedrich RE, Schmelzle R (1996). "Mucoepidermoid carcinoma of salivary gland origin: classification, clinical-pathological correlation, treatment results and long-term follow-up in 55 patients". J Craniomaxillofac Surg. 24 (3): 133–9. PMID 8842902.
  10. Armstrong JG, Harrison LB, Spiro RH, Fass DE, Strong EW, Fuks ZY (1990). "Malignant tumors of major salivary gland origin. A matched-pair analysis of the role of combined surgery and postoperative radiotherapy". Arch. Otolaryngol. Head Neck Surg. 116 (3): 290–3. PMID 2306346.
  11. 11.0 11.1 https://en.wikibooks.org/wiki/Radiation_Oncology/Head_%26_Neck/Salivary_gland Accessed on February 17, 2016
  12. Eversole LR (1970). "Mucoepidermoid carcinoma: review of 815 reported cases". J Oral Surg. 28 (7): 490–4. PMID 5269211.

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