title: Conservative multimodal management of a primitive neuroectodermal tumor of the thyroid.
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.
authors.affiliation: Centre Antoine Lacassagne, Institut Universitaire de la Face et du Cou , Nice.
authors.forename: Romain
authors.initials: R
authors.lastname: Natale
authors.forename: Juliette
authors.initials: J
authors.lastname: Thariat
authors.forename: Pierre Olivier
authors.initials: PO
authors.lastname: Vedrine
authors.forename: Alex
authors.initials: A
authors.lastname: Bozec
authors.forename: Isabelle
authors.initials: I
authors.lastname: Peyrottes
authors.forename: Pierre Yves
authors.initials: PY
authors.lastname: Marcy
authors.forename: Juliette
authors.initials: J
authors.lastname: Haudebourg
authors.forename: Florence
authors.initials: F
authors.lastname: Pedeutour
authors.forename: Esma
authors.initials: E
authors.lastname: Saâda
authors.forename: Antoine
authors.initials: A
authors.lastname: Thyss
keywords: multimodal treatment; primitive neuroectodermal tumor; rare tumor; sarcoma; thyroid