Primitive neuroectodermal tumor of thyroid: a rare presentation

Abstract

Extraskeletal Ewing’s sarcoma (EES) commonly arises in the soft tissues of trunk or extremities. EES is rare in the head and neck region; most having been documented in nasal cavity, paranasal sinuses and neck. Head and neck PNETs have an intermediate prognosis. We report the case of a 12 year old boy who presented with primitive neuroectodermal tumor of the thyroid and was treated with multimodal treatment including surgery, chemotherapy and radiotherapy. The patient is alive and fit with a functional larynx. 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 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.