Peripheral primitive neuroectodermal tumor of the maxillary gingivae with metastasis to cervical lymph nodes: Report of a case

Abstract

Primitive neuroectodermal tumor (PNET) is a rare malignant, small, round cell tumor of neuroectodermal origin. The tumor affects both genders and occurs in all age groups, with peak occurrence during adolescence. Most PNETs arise within the chest and limbs; only 3% of these tumors have been reported within the skull and jaws. Central PNETs (cPNETs), which arise within the central nervous system, differ from their counterparts (pPNET) genetically. Also, cPNETs much less often arise within bone and soft tissue. 1 Cohen BH Packer RJ Chemotherapy for medulloblastoma and primitive neuroectodermal tumors. J Neurol Oncol. 1996; 29: 55 Crossref PubMed Scopus (33) Google Scholar , 2 Jones JE McGill T Peripheral primitive neuroectodermal tumors of the head and neck. Arch Otol Head Neck Surg. 1995; 121: 1392 Crossref PubMed Scopus (75) Google Scholar , 3 Batsakis JG Mackay B El-Nagar AK Ewing's sarcoma and peripheral primitive neuroectodermal tumor: An interim report. Ann Otol Rhinol Laryngol. 1996; 105: 838 PubMed Google Scholar , 4 Dehner LP Peripheral and central primitive neuroectodermal tumors. Arch Pathol Lab Med. 1986; 110: 997 PubMed Google Scholar We present the case of a patient with rapidly growing pPNET of the right maxillary gingivae, with neck metastases. There was an attempt to control both the primary and metastatic lesions with chemotherapy. The tumor was studied with conventional microscopy, immunohistochemistry (IHC), and transmission electron microscopy (TEM). Clinicopathologic features and differential diagnosis of PNET are reviewed.