RARE PAROTID TUMOUR IN A 2-YEAR-OLD CHILD

János HUSZKA & Gábor KATONA

Budapest

Hungary

In the literature only a few cases of parotid tumours in childhood under the age of 5 have been reported.

• S.O.Krolls et al.: Salivary gland lesions in children. A survey of 430 cases. (Cancer, 1972)

• D.L.Callender et al.: Salivary gland neoplasms in children. (Arch.Otolaryngol.Head Neck Surg.1992)

• P.D.Bull: Salivary gland neoplasia in childhood. (Int.J.Pediatr.Otorhinolaringol.1999)

• B.G.Bentz et al.: Masses of the salivary gland region in children. (Arch.Otolaryngol. Head Neck Surg. 2000)

• D.O.Walterhause et al.:Rhabdomyosarcoma of the parotid region occuring in

childhood and adolescence. (Cancer, 2001)• P.Claros et al.:Parotid gland mucoepidermoid carcinoma in a 4-year-old

child.(Int.J.Pediatric.Otorhinolaryngol.2002) • G.Y.Yu et al.: Diagnosis and treatment of epithelial salivary gland tumours in

children and adolescents. (British J.Oral Maxillofac.Surg. 2002)• S.Moriniere et al.: Massive infra-clinic invasion of the facial nerve by a

myoepthelial carcimona of the parotid.(Int.J.Pediatric.Otorhinolaringol.2003)

During the last 4-year period 114 parotid operations were performed

• Total parotidectomy 14• Subtotal parotidectomy 91• Subtotal parotidectomy+ neck dissection 1• Total parotidectomy+ neck dissection 2• Total parotidectomy+ neck dissection

and resection+reconstruction n.VII. 2• Enucleation 4

Histopathological diagnosis

Benign (n=94)

pleomorphic adenoma 28 Warthin’s tumour 41 chronic sialadenitis 8

myeloepithelioma 4 cysta simplex 4 lipoma 3 lipomatosis 2 lymphangioma 2 benign lymphoepithelial lesion 2

Histopathological diagnosis

Malignant (n=20)

mucoepidermoid ca. 4 mixed tumour mal. 3 squamocell ca. 3 adenoid cystic ca 3 lymphoma mal. 2 adenoca. 2 acinic cell ca. 1 oncocytoma mal. 1

synovial sarcoma/PNET/ rhabdoid tumour (?) 1

A 2-year-old boy was referred to the ENT department with an asymptomatic 3cm slightly tender mass located in the right pre-and

sub-auricular region, which had been discovered 6 weeks before and had had progressive growth.

Clinical examination showed a slightly mobile, painless node without any fixation. We observed some palpable lymph nodes in close connection with the

tumour on the neck. Facial movement was normal.The reminders of physical examination and laboratory tests were

unremarkable.

CT

After CT investigation fine needle aspiration biopsy (FNA) was performed under ultrasound scan. The result of FNA was a rare, specific malignance

tumour. Immunohistochemical reactions precluded the possibilities of lymphoma malign and ganglio-neuroblastoma and rhabdomyosarcoma, but

it is probably a rhabdoid tumour.

Total parotidectomy with preservation of facial nerveand upper modified neck dissection was performed.The tumour was located in the deep lobe, under the

main trunk and the branches of the n.VII.

The styloid-muscles near to the styloid process wereinvolved 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 lymph nodes, thesubdigastric and the upper accessory nodes.

The submandibular gland, the accessory nerve, thesternocleidomastoid muscle and the jugular vein were

preserved.

The incision line

The opened field of operation

The preparation for the intraopertive frozen section The investigated lymph nodes did not contain any signs of malignancies.

n.auric. magn.

The main trunk of the n.VII. was explored.

Dissection of the lateral lobe from the facial nerve.

After separation of the superficial lobe it was clear that the tumour was located under the main trunk and cervicofacial branches.

The lateral lobe was removed.

Delicate separation of the nerve from the tumour.

Before removing the dissected block.

Histological diagnosis seemed to be very difficult. The tumour was very cellular consisting of primitive small round blue cells growing

in diffuse sheets. There were no specialised architectural arrangements. The cytoplasm exhibited distinct features only on

keratin immunostains.

Diagnostically challenging case

• Semmelweis Medical University

II. Pathological Clinic (Budapest)• The University of Chicago

Department of Pathology, Section of Anatomic Pathology

In conjunction with immunohistochemical results the differential diagnosis is narrowed down to three entities:

rhabdoid tumour, PNET/Ewing’s and the poorly differentiated variant of monophasic synovial sarcoma..

He received chemotherapy treatment one week later.

(CWS96 soft tissue protocoll)(Ifosfamid, Vincristin, Actinomycin-D)

The child had facial movement disturbance at the branches of his eye and the field of r. marginalisafter the operation.

According to the oncologist, he received nine doses of this treatment altogether.

He lost his hair, whoever, he otherwise

remained in a good condition troughout the treatment. His hair has

grown back and his face movement disturbance has

complitely disappered.

Thank you for your kind attention