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+A 13 year-old boy presented with a 1 month history of intermittent episodes of small volume epistaxis.
+There was no significant past medical, surgical or family history.
+Physical examination revealed a left palatal fleshy mass centered at left posterior molars.
+A nasoendoscopic examination demonstrated a large swelling on the floor of left anterior nasal space, involving the left inferior meatus and abutting the inferior turbinate.
+Computed Tomography (CT) of the paranasal sinuses showed a lobulated, expansile lytic lesion of left maxilla [Fig 1] with a lucent center and peripheral calcifications.
+The lesion was associated with a large soft tissue component that demonstrated mild heterogeneous contrast enhancement.
+The lesion was centered at the left maxillary alveolus, involving the left hard palate, inferior part of the anterior left maxillary wall and medial left maxillary wall.
+The lesion also involved the roots of the left maxillary molars and pre-molars and was abutting the left orbital floor, with no gross orbital invasion.
+The patient subsequently underwent 18-FDG PET-CT study.
+The mass centered in left maxilla was hypermetabolic, with index SUVmax 9.7 [Fig 2].
+There was no FDG-avid metastatic disease.
+A trans-oral incisional biopsy of the left palatal mass was performed and was suggestive of a preliminary diagnosis of mesenchymal chondrosarcoma, as evidenced by a biphasic pattern featuring sheets of small round cells with hemangiopericytoma-like vascular pattern and mature cartilaginous islands.
+The patient underwent left subtotal maxillectomy via lateral rhinotomy and translabial approach.
+The final histology was conclusive of a 5cm high-grade (Grade 3) mesenchymal chondrosarcoma.
+One of the resection margins was positive for tumor involvement and the patient was planned for adjuvant chemotherapy.