Article Dans Une Revue Rare tumors Année : 2013

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

Romain Natale
  • Fonction : Auteur
Pierre Olivier Vedrine
  • Fonction : Auteur
Alex Bozec
  • Fonction : Auteur
Isabelle Peyrottes
  • Fonction : Auteur
Pierre Yves Marcy
  • Fonction : Auteur
Juliette Haudebourg
  • Fonction : Auteur
Florence Pedeutour
Esma Saâda
  • Fonction : Auteur
Antoine Thyss
  • Fonction : Auteur

Résumé

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.

Dates et versions

hal-00868851 , version 1 (02-10-2013)

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Citer

Romain Natale, Juliette Thariat, Pierre Olivier Vedrine, Alex Bozec, Isabelle Peyrottes, et al.. Conservative multimodal management of a primitive neuroectodermal tumor of the thyroid.. Rare tumors, 2013, 5 (2), pp.75-8. ⟨10.4081/rt.2013.e20⟩. ⟨hal-00868851⟩
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