Rare parotid tumor


Authors: János Huszka MD and Gábor Katona MD, PhD

Institutions: Szent István Hospital, Dept.of ORL, Head & Neck Surg. Budapest/ Hungary

Heim Pál Pediatric Hospital, Dept. ORL & Bronchology  Budapest/Hungary


Salivary gland neoplasm represent rare lesions, accounting for fewer then 5% of tumors head and neck. In the literature only a few cases of malignant parotid tumors in childhood under the age of 5 have been reported. The most frequent malignant neoplasm is mucoepiermoid carcinoma which is followed by other types of less frequently occurring malignant tumors but, because of their rarity they are at risk of misdiagnosis and subsequent mistreatment.

During the last 4-year period (1999-2003) 144 parotid operations were performed. Among the operated patients there were only 2 children. A 16-year-old girl had acinic cell carcinoma and a 2-year-old boy had a very rare parotid malignant tumor. The last mentioned case caused serious diagnostic problem to the pathologists. We will introduce this case to you in the followings.

A 2-year-old boy was referred to our department with an asymptomatic 3 cm slightly tender mass located in the right pre-and sub-auricular region, which had been discovered 6 weeks before and had had a progressive growth. Clinical examination showed a slightly mobile, painless node without any fixation to the overlying skin. We observed some palpable lymph nodes in close connection to the tumor on the neck. Facial movements were normal. The remainders of the physical examination and laboratory investigations were unremarkable. After MRI fine needle aspiration biopsy (FNA) was performed under ultrasound scan. FNA result was a rare, specific malignance tumor. Immunohistochemical reactions precluded the possibilities of lymphoma malign and ganglio-neuroblastoma and rhabdomyosarcoma, but probably it is a rhabdoid tumor.

Total parotidectomy with preservation of facial nerve and upper modified neck dissection was performed. The tumor was located in the deep lob, under the main trunk and branches of the facial nerve. The styloid-muscles near the styloid process were involved by the neoplasm and it reached to the parapharyngeal space. The involved muscles were resected. In the upper modified neck dissection we removed the upper and mid-jugular nodes, the subdigastric nodes and the upper accessory nodes. The submandibular gland, the accessory nerve, the sternocleidomastoid muscle and jugular vein were preserved. Intraoperative frozen section showed the same result as FNA, and the investigated lymph nodes did not contain any signs of malignancies.  The child had facial movement disturbance after the operation.

Before the end of our presentation we would like to show a short video about the operation.

The histological diagnosis has been difficult; we are still waiting for it. Hopefully we can discuss this interesting case with you in the congress, already knowing the exact histological diagnosis.

The child is receiving chemotherapy at present.