title: A peripheral primitive neuroectodermal tumor in the larynx: A case report and literature review.
abstract: Primitive neuroectodermal tumors (PNETs) are malignant tumors comprised of small round cells of neuroectodermal origin. Current evidence indicates that peripheral PNETs (pPNETs), which arise in the non-central nervous system, possess histological similarity to Ewing's sarcoma. Though the occurrence of pPNETs in the head and neck region is rare, these are aggressive malignant tumors, and long-term survival rates following diagnosis remain poor. The current report presents a case of pPNET and evaluates its significance with regard to previous studies. In the present case, a tumor was located in the larynx of the patient, and was diagnosed as pPNET. Immunohistochemical analysis indicated that tumor cells were positive for cluster of differentiation 99. The patient was treated with surgery, multiagent chemotherapy and radiotherapy. Five years subsequent to treatment, the patient had survived and demonstrated no evidence of disease recurrence. In existing literature concerning pPNET located outside the head and neck region, it is recommended that patients are treated with a combination of resection with a wide surgical margin, multiagent chemotherapy and radiotherapy. The present case report concluded that the combination of surgery, systematic chemotherapy and radiotherapy, offers an improved outcome for pPNET localized to the head and neck region, compared with any of these therapies alone.
authors.affiliation: Department of Otolaryngology - Head and Neck Surgery, Nagoya City University Graduate School of Medical Sciences, Nagoya, Aichi 467-8601, Japan.
authors.forename: Kei
authors.initials: K
authors.lastname: Ijichi
authors.affiliation: Department of Pathology, Nagoya Daini Red Cross Hospital, Nagoya, Aichi 466-8650, Japan.
authors.forename: Toyonori
authors.initials: T
authors.lastname: Tsuzuki
authors.affiliation: Department of Oral and Maxillofacial Surgery, Asahi University Murakami Memorial Hospital, Gifu 500-8856, Japan.
authors.forename: Makoto
authors.initials: M
authors.lastname: Adachi
authors.affiliation: Department of Otolaryngology - Head and Neck Surgery, Nagoya City University Graduate School of Medical Sciences, Nagoya, Aichi 467-8601, Japan.
authors.forename: Shingo
authors.initials: S
authors.lastname: Murakami
keywords: Ewing's sarcoma; head and neck; head and neck sarcoma; larynx; peripheral primitive neuroectodermal tumor; primitive neuroectodermal tumor