title: Malignant peripheral primitive neuroectodermal tumor-peripheral neuroepithelioma of the head and neck: a clinicopathologic study of five cases and review of the literature.
abstract: BACKGROUND
  The term primitive neuroectodermal tumor (PNET) encompasses a number of neoplasms of common neuroectodermal origin, but of variable clinical, histopathologic, ultrastructural, and molecular characteristics. Here, we focus on one particular member of the PNET family, the malignant peripheral PNET (pPNET) or peripheral neuroepithelioma of head and neck.
  
  
  METHODS
  Five patients diagnosed with malignant pPNET-peripheral neuroepithelioma of head and neck were included in the study. All existing literature was reviewed.
  
  
  RESULTS
  The diagnosis of malignant pPNET-peripheral neuroepithelioma of the head and neck was confirmed in all five cases. All patients are alive after treatment with a combination of therapeutic modalities; only one patient had metastatic disease develop. A literature review revealed another 38 reported cases.
  
  
  CONCLUSIONS
  Malignant pPNET-peripheral neuroepithelioma should be included in the differential diagnosis of small, round, blue cell tumors of head and neck. A combination of multiple diagnostic modalities is essential for correct diagnosis, and multimodality treatment offers the best outcome.
authors.affiliation: Department of Diagnostic Sciences and Pathology, Dental School, University of Maryland, Baltimore, 666 W Baltimore Street, Room 4-C-02, Baltimore, Maryland 21201-1586, USA. nin001@dental.umaryland.edu
authors.forename: Nikolaos G
authors.initials: NG
authors.lastname: Nikitakis
authors.forename: Andrew R
authors.initials: AR
authors.lastname: Salama
authors.forename: Bert W
authors.initials: BW
authors.lastname: O'Malley
authors.forename: Robert A
authors.initials: RA
authors.lastname: Ord
authors.forename: John C
authors.initials: JC
authors.lastname: Papadimitriou
mesh_terms: D000071443:12E7 Antigen; D000293:Adolescent; D000328:Adult; D015703:Antigens, CD / Q000032:analysis; D015815:Cell Adhesion Molecules / Q000032:analysis; D017024:Chemotherapy, Adjuvant; D005260:Female; D006258:Head and Neck Neoplasms / Q000175:diagnosis* / Q000628:therapy*; D006801:Humans; D008297:Male; D008875:Middle Aged; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000175:diagnosis* / Q000628:therapy*; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D000071443:12E7 Antigen; D015703:Antigens, CD; C061828:CD99 protein, human; D015815:Cell Adhesion Molecules