carcinoma ex pleomorphic adenoma

Carcinoma ex pleomorphic adenoma is the most common of three malignant mixed tumors of salivary glands, and are thought to arise from pre-existing pleomorphic adenomas (or benign mixed tumors) .

Epidemiology

These tumors usually occur in older patients (6 to 8 decade), who have had a pleomorphic adenoma in situ for many years (typically 10-15 years) .

Clinical presentation

Patients will describe having had a stable parotid mass for many years, before experiencing rapid growth, often associated with painful facial nerve palsy due to tumor infiltration .

Radiographic features

As these tumors are aggressive and infiltrating, they present as irregular masses extending into the gland parenchyma and beyond.

MRI
  • T1: heterogeneous signal due to hemorrhage, necrosis and calcification
  • T2: heterogeneous but generally low signal
  • ADC: low values (compared to pleomorphic adenomas which typically have elevated values)

For staging see malignant salivary tumor staging.

Treatment and prognosis

These tumors have a high rate of metastasis at the time of diagnosis (25-76%) typically to brain, bone, lungs, and of course local lymph nodes .

Differential diagnosis

The differential is essentially that of other malignant salivary gland tumors including:

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