Primitive neuroectodermal tumor is a member of malignant small round cell tumors. These tumors especially originate from the central and autonomous nervous system. However, these tumors may be originated from peripheral tissues and are called peripheral primitive neuroectodermal tumor. A 14-year-old girl attended to the Ear Nose Throat Clinic with the complaint of progressive painless swelling mass for 2 months on the right side of the face. Neck magnetic resonance imaging showed 3.5 × 2.5 × 2-cm isointense mass on T1 and hyperintense on T2 sequences. There was no pathological lymphadenopathy on computed tomographic scan. As a result of mandibular cortical invasion seen on computed tomographic scan, radical surgical excision was decided as surgical treatment. Total parotidectomy with preserving facial nerve and partial mandibulectomy with a 2-cm margin of safety were done, and reconstruction plaque applied to the mandible. Two lymph nodes were seen at the submandibular region. For this reason, prophylactic supraomohyoid neck dissection had also been performed. Pathological assessment proved the diagnosis of PNET, and chemoradiotherapy was planned for the patient.To our knowledge, this is the second reported case in literature. In this present case, peripheral neuroectodermal tumor in the masseter muscle and its diagnosis and treatment process were reported with literature review.
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