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Natale R, Thariat J, Vedrine PO, et al. Conservative multimodal management of a primitive neuroectodermal tumor of the thyroid. Rare Tumors. 2013;5(2):75-8doi: 10.4081/rt.2013.e20.
Natale, R., Thariat, J., Vedrine, P. O., Bozec, A., Peyrottes, I., Marcy, P. Y., Haudebourg, J., Pedeutour, F., Saâda, E., & Thyss, A. (2013). Conservative multimodal management of a primitive neuroectodermal tumor of the thyroid. Rare tumors, 5(2), 75-8. https://doi.org/10.4081/rt.2013.e20
Natale, Romain, et al. "Conservative multimodal management of a primitive neuroectodermal tumor of the thyroid." Rare tumors vol. 5,2 (2013): 75-8. doi: https://doi.org/10.4081/rt.2013.e20
Natale R, Thariat J, Vedrine PO, Bozec A, Peyrottes I, Marcy PY, Haudebourg J, Pedeutour F, Saâda E, Thyss A. Conservative multimodal management of a primitive neuroectodermal tumor of the thyroid. Rare Tumors. 2013 May 29;5(2):75-8. doi: 10.4081/rt.2013.e20. Print 2013 Apr 15. PMID: 23888220; PMCID: PMC3719115.
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Rare Tumors. 2013 May 29;5(2):75-8. doi: 10.4081/rt.2013.e20. Print 2013 Apr 15.

Conservative multimodal management of a primitive neuroectodermal tumor of the thyroid.

Rare tumors

Romain Natale, Juliette Thariat, Pierre Olivier Vedrine, Alex Bozec, Isabelle Peyrottes, Pierre Yves Marcy, Juliette Haudebourg, Florence Pedeutour, Esma Saâda, Antoine Thyss

Affiliations

  1. Centre Antoine Lacassagne, Institut Universitaire de la Face et du Cou , Nice.

PMID: 23888220 PMCID: PMC3719115 DOI: 10.4081/rt.2013.e20

Abstract

Primitive neuroectodermal tumors (PNET) represent 1% of sarcomas. Head and neck peripheral PNETs have an intermediate prognosis between abdominopelvic disease and extremities. We here report the case of a 40-year old male who presented with primitive neuroectodermal tumor of the thyroid and was treated by multimodal treatment, including surgery, chemotherapy and intermediate dose radiotherapy. The patient is alive and fit with a functional larynx at 27 months. Multimodal treatments yield five-year survival rates of about 60%. Major drug regimens use vincristine, doxorubicin, ifosfamide or cyclophosphamide, dactinomycin and/or etoposide. Complete surgical excision is undertaken whenever possible to improve long-term survival. However, the relative radiosensitivity of tumors of the Ewing family, suggest multimodal treatment including adjuvant conformal radiotherapy in case of positive margins or poor response to chemotherapy rather than resection with 2-3 cm margins, which would imply laryngeal sacrifice for thyroid tumors. The role of expert rare tumor networks is crucial for optimal decision-making and management of such rare tumors on a case by case basis.

Keywords: multimodal treatment; primitive neuroectodermal tumor; rare tumor; sarcoma; thyroid

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