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Primitive neuroectodermal tumor of the head and neck: incidence, diagnosis, and management.

Abstract

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.

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  • Publisher Full Text
  • Authors+Show Affiliations

    Department of Otorhinolaryngology-Plastic Head and Neck Surgery, St Anna Hospital, Duisburg, Germany.

    Source

    MeSH

    Antineoplastic Combined Chemotherapy Protocols
    Brain
    Brain Neoplasms
    Child
    Chromosomes, Human, Pair 11
    Combined Modality Therapy
    Diagnosis, Differential
    Head and Neck Neoplasms
    Humans
    Immunohistochemistry
    Incidence
    Magnetic Resonance Imaging
    Male
    Neuroectodermal Tumors, Primitive
    Neurosurgical Procedures
    Sarcoma, Ewing
    Tomography, X-Ray Computed
    Translocation, Genetic

    Pub Type(s)

    Case Reports
    Journal Article
    Review

    Language

    eng

    PubMed ID

    15274413

    Citation

    Windfuhr, Jochen P.. "Primitive Neuroectodermal Tumor of the Head and Neck: Incidence, Diagnosis, and Management." The Annals of Otology, Rhinology, and Laryngology, vol. 113, no. 7, 2004, pp. 533-43.
    Windfuhr JP. Primitive neuroectodermal tumor of the head and neck: incidence, diagnosis, and management. Ann Otol Rhinol Laryngol. 2004;113(7):533-43.
    Windfuhr, J. P. (2004). Primitive neuroectodermal tumor of the head and neck: incidence, diagnosis, and management. The Annals of Otology, Rhinology, and Laryngology, 113(7), pp. 533-43.
    Windfuhr JP. Primitive Neuroectodermal Tumor of the Head and Neck: Incidence, Diagnosis, and Management. Ann Otol Rhinol Laryngol. 2004;113(7):533-43. PubMed PMID: 15274413.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Primitive neuroectodermal tumor of the head and neck: incidence, diagnosis, and management. A1 - Windfuhr,Jochen P, PY - 2004/7/28/pubmed PY - 2004/8/17/medline PY - 2004/7/28/entrez SP - 533 EP - 43 JF - The Annals of otology, rhinology, and laryngology JO - Ann. Otol. Rhinol. Laryngol. VL - 113 IS - 7 N2 - 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. SN - 0003-4894 UR - https://www.unboundmedicine.com/medline/citation/15274413/Primitive_neuroectodermal_tumor_of_the_head_and_neck:_incidence_diagnosis_and_management_ L2 - http://journals.sagepub.com/doi/full/10.1177/000348940411300705?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -