Primitive Neuroectodermal Tumor of the Head and Neck: Incidence, Diagnosis, and Management

• Published in: Annals of otology, rhinology & laryngology rhinology & laryngology, 2004-07, Vol.113 (7), p.533-543
• Main Author: Windfuhr, Jochen P.
• Format: Article
• Language: English
• Subjects: Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Biological and medical sciences; Brain - diagnostic imaging; Brain - pathology; Brain - surgery; Brain Neoplasms - diagnosis; Brain Neoplasms - genetics; Brain Neoplasms - therapy; Child; Chromosomes, Human, Pair 11 - genetics; Combined Modality Therapy; Diagnosis, Differential; Head and Neck Neoplasms - diagnosis; Head and Neck Neoplasms - epidemiology; Head and Neck Neoplasms - therapy; Humans; Immunohistochemistry; Incidence; Magnetic Resonance Imaging; Male; Medical sciences; Neuroectodermal Tumors, Primitive - diagnosis; Neuroectodermal Tumors, Primitive - epidemiology; Neuroectodermal Tumors, Primitive - therapy; Neurosurgical Procedures - methods; Otorhinolaryngology. Stomatology; Sarcoma, Ewing - diagnosis; Sarcoma, Ewing - genetics; Sarcoma, Ewing - therapy; Tomography, X-Ray Computed; Translocation, Genetic - genetics
• ISSN: 0003-4894; 1943-572X
• DOI: 10.1177/000348940411300705

Primitive neuroectodermal tumors are in the Ewing's sarcoma family of tumors and are composed of small round cells. Because of their rare occurrence, optimal therapy is challenging, particularly if they occur in the head and neck. Diagnosis is based on history, immunostaining with at least 2 neural markers, ultrastructural examination, and evidence of an abnormal t(11;22)(q24;q12) translocation as the hallmark for the Ewing's sarcoma family. The prognosis in general is poor because of overt metastasis at the time of diagnosis. Of 27 reported patients with primitive neuroectodermal tumors of the head and neck, 23 were less than 20 years of age. Most patients presented with a tumor in the nasal cavity, paranasal sinuses, or neck. Symptoms developed rapidly (3.6 months, on average), and a lethal outcome occurred in 9 patients. This highly malignant tumor requires an aggressive combination of radical resection, chemotherapy, and radiotherapy. A close follow-up with regular radiographic examination for at least 5 years is mandatory.