A Rare Case of Extraosseous Osteosarcoma (EOS) of Parotid Gland

Extraosseous osteosarcoma is a rare malignant soft tissue neoplasm, and extraosseous osteosarcoma of the parotid gland is very rare. It has a very aggressive course, and there are no standardized treatment guidelines. We report the case of a 20 year old male patient who presented with history of right neck swelling since 6 years for which he had undergone right parotid surgery 5 years ago. The final histopathological report indicated that the mass was a pleomorphic adenoma. One year after the first surgery, the patient experienced recurrence of swelling over the operated site, and the size of the swelling has been increasing gradually since then. He was evaluated clinically, and a large mass was noted over the upper aspect of the right upper neck, extending to the occipital and parotid regions. An MRI scan was done which showed a 12 × 10 × 8 cm lesion centred in the right parotid gland, involving paraspinal muscles, C1-C2 vertebrae and extending into the parapharyngeal space. FNAC of the lesion showed features of pleomorphic adenoma. The patient underwent a complete excision of the tumour. The patient's post-operative period was uneventful. The final histopathological report of the patient was extraosseous osteosarcoma of the parotid gland. The patient was referred for adjuvant radiotherapy. He has been on regular follow-up for the past 6 months and has shown no sign of recurrence. EOS is an extremely rare tumour of the head and neck region which often requires extensive surgical resection with or without adjuvant radiotherapy. It has a high rate of local recurrence and a very low disease free survival. Such patients should be kept on a close follow-up.