PMC6015682_01
Preoperative clinical photograph showing a right parotid swelling.
CRIOT2018-8768472.001.jpg
PMC6015682_01_CRIOT2018-8768472.001.jpg
fig1
Physical examination revealed a tender right parotid swelling below the ear lobule, which extended inferiorly to the angle of the mandible (Figure 1).
Axial CT with contrast shows a mass in the superficial lobe of the right parotid gland with post-contrast enhancement.
CRIOT2018-8768472.002.jpg
PMC6015682_01_CRIOT2018-8768472.002.jpg
fig2
The neck CT scan revealed a well-defined altered signal enhancing mass measuring 3.5 x 2.2 x 2.0 cm at the posterior aspect of the superficial part of the right parotid gland (Figure 2).
Clinical photograph showing intraoperative view of the parotid swelling.
CRIOT2018-8768472.003.jpg
PMC6015682_01_CRIOT2018-8768472.003.jpg
fig3
The superficial parotidectomy was done in piecemeal as the mass was unexpectedly adherent to the skin and underlying fascia (Figure 3). The facial nerve was intact and was examined branch by branch.
Histologically, diffuse-type neurofibroma arising in the parotid region with diffuse infiltration between salivary gland lobules and surrounding adipose tissue (arrows).
CRIOT2018-8768472.004.jpg
PMC6015682_01_CRIOT2018-8768472.004.jpg
fig4
Histopathologic examination showed a widely infiltrative tumor involving the parotid parenchyma and extending into the surrounding adipose tissue and skeletal muscles (Figure 4).
Diffuse-type neurofibroma comprises tumor cells with round to fusiform nuclei in a fine fibrillary collagenous matrix.
CRIOT2018-8768472.005.jpg
PMC6015682_01_CRIOT2018-8768472.005.jpg
fig5
The tumor was predominantly composed of bland spindle cells with wavy nuclear contours, embedded within a fibrillary, pale pink matrix, which resembled a neurofibroma (Figure 5).
Areas of increased cellularity with epithelioid cells showing nuclear atypia and mitotic activity indicate transition to MPNST.
CRIOT2018-8768472.006.jpg
PMC6015682_01_CRIOT2018-8768472.006.jpg
fig6
Of concern, there were evidently distinct scattered more cellular foci of epithelioid tumor cells with nuclear atypia and increased mitotic activity (Figure 6).
Clinical photograph showing multiple cafe au lait macules on the patient torso.
CRIOT2018-8768472.007.jpg
PMC6015682_01_CRIOT2018-8768472.007.jpg
fig7
Multiple cafe au lait macules were subsequently discovered on his trunk (Figure 7).