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RARE PAROTID TUMOUR IN A 2-YEAR -OLD CHILD

RARE PAROTID TUMOUR IN A 2-YEAR -OLD CHILD. J ános 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.

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RARE PAROTID TUMOUR IN A 2-YEAR -OLD CHILD

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  1. RARE PAROTID TUMOUR IN A 2-YEAR-OLD CHILD János HUSZKA & Gábor KATONA Budapest Hungary

  2. 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)

  3. 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

  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 lesion2

  5. 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

  6. 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.

  7. 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

  8. 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.

  9. Total parotidectomy with preservation of facial nerve and 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 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 lymph nodes, the subdigastric and the upper accessory nodes. The submandibular gland, the accessory nerve, the sternocleidomastoid muscle and the jugular vein were preserved.

  10. The incision line

  11. The opened field of operation

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

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

  14. Dissection of the lateral lobe from the facial nerve.

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

  16. The lateral lobe was removed.

  17. Delicate separation of the nerve from the tumour.

  18. Before removing the dissected block.

  19. 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.

  20. 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..

  21. The child had facial movement disturbance at the branches of his eye and the field of r. marginalis after the operation. He received chemotherapy treatment one week later. (CWS96 soft tissue protocoll) (Ifosfamid, Vincristin, Actinomycin-D)

  22. 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.

  23. Thank you for your kind attention

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