Revision 46 for 'Mucoepidermoid carcinoma of salivary glands'All Revisions
Mucoepidermoid carcinoma of salivary glands
A mucoepidermoid carcinoma is a tumor that usually occurs in the salivary glands. It can mimic most other tumors of the glands, and therefore is often considered in the differential.
Mucoepidermoid carcinomas are seen throughout all adult age groups, but are most common in middle age (35-65 years of age) 5. However, it is the most common malignant salivary gland tumor of childhood 4-5. Overall, mucoepidermoid carcinomas account for 1,5:
- 2.8-15.5% of all salivary gland tumors
In the parotid gland they are the most common malignant primary neoplasm. A slight female predilection has been described, and radiation has been implicated as a risk factor 5.
Mucoepidermoid carcinomas most frequently arise in the parotid gland, and presents as a painless swelling, with or without facial nerve involvement. These tumors can however be found anywhere there are salivary glands. Overall distribution across various glands is as follows 5:
- major salivary glands : ~ 50% *
- parotid gland : ~ 40%
- submandibular gland : ~ 7%
- sublingual gland : ~ 3%
- minor salivary glands : ~ 50%
- palate : most common
- retromolar area
- floor of the mouth
- buccal mucosa
- other : anywhere in the proximal aerodigestive tract, the lacrimal glands and even in the bronchi 2,5
As such, presentation will depend on the anatomic location.
The tumors are composed of a mixture of :
- mucus secreting cells (muco- )
- squamous cells (-epidermoid)
- lymphoid infiltrate often also present 3
Mucoepidermoid tumors are graded histologically into :
- low grade
- well differentiated cells with little cellular atypia
- high proportion of mucous cells
- prominent cyst formation
- intermediate grade : intermediate features
- high grade
- poorly differentiated with cellular pleomorphism
- high proportion of squamous cells
- solid with few if any cysts
Histology will often show clear mucin containing cells which stain reddish pink with the musicarmine stain.
Radiographic appearances largely depend on grade, making preoperative imaging important in planning and counseling.
Typically a well circumscribed hypoechoeic, with an either part of completely cystic appearance. The lesion stands out against the relatively hyperechoeic normal parotid gland.
Low grade tumors appear as well circumscribed masses, usually with cystic components. The solid components enhance, and calcification is sometimes seen. They have appearances similar to benign mixed tumors.
High grade tumors on the other hand, have poorly defined margins, infiltrate locally and appear solid.
Again, imaging is dependent on grade.
Low grade tumors have similar appearances to benign mixed tumors :
- T1 - low to intermediate signal ; low signal cystic spaces
- T2 - intermediate to high signal ; cystic areas will be high signal
- T1 C+ (Gd) - heterogeneous enhancement of solid components
High grade tumors on the other hand have lower signal on T2 and poorly defined margins and infrequent cystic areas.
- T1 - low to intermediate signal
- T2 - intermediate to low signal
It is essential to image the cranial nerves with fat saturated post contrast T1 sequences to assess for perineural spread, and as such the base of skull should be imaged up to and including the cavernous sinus and inner ear.
Treatment and prognosis
Treatment is dependent on grade and location.
- low grade (well circumscribed) can usually be treated with wide local excision and preservation of the facial nerve, without the need for neck dissection or adjuvant radiotherapy
- high grade (poorly circumscribed) usually requires complete parotidectomy, often with sacrifice of the facial nerve, neck dissection (as nodal metastases are common) and adjuvant radiotherapy
Prognosis is also very dependent on grade, with low grade tumors having a 90 - 98% survival and a low local recurrence rate, compared to 30 - 54% surviving, and a very high local recurrence rate for high grade tumors 1,5.
Additionally this tumor has a predilection for perineural spread, and careful and long term follow-up is therefore required.
The differential is therefore different according to appearance.
For well circumscribed lesions consider :
For infiltrative lesions consider :