An unusual case of pilomatrix carcinoma of the salivary glands in a young man

We describe here a rare case of pilomatrix carcinoma in the right parotid region, arising in a young patient.

Pilomatrix carcinoma is a rare, locally aggressive tumor with a tendency to recur. Pilomatrixoma is a superficial benign skin tumor arising from the matrix cells of the hair follicle generally located in the scalp, face, and upper limbs. Clinically, it usually presents as a solitary subcutaneous blue-black nodule which grows slowly.

An 18-year-old male, non-smoker, in good health, was referred to our Unit after the onset of a swelling in the right parotid region. The lesion had been present for 1 year but the patient did not seek medical attention in that time. Clinical examination showed an exophytic mass in the right parotid region measuring 20 × 20 mm. An ultrasound of the major salivary glands showed an oval lesion of the right parotid gland; contrast-enhanced MRI appeared to show an oval lesion in the right intraparotid region, approximately 20 × 25 mm, which was hypointense in T1 images, and hyperintense in T2 images, with inhomogeneous contrast-enhancement; Fine Needle Aspiration Cytology (FNAC) was indicative of a lesion with high suspicion of malignancy.

We elected to carry out a facial nerve sparing total parotidectomy, under general anesthesia, and facial reconstruction of the remaining gap with a sternocleidomastoid muscle flap. Histopathological analysis demonstrated basaloid cell proliferation beneath granulation tissue, hemorrhage, and large number of “ghost cells” with high mitotic activity. This confirmed the diagnosis of pilomatrix carcinoma. Postoperative outcome was uneventful.