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.

Peripheral primitive neuroectodermal tumor: Dynamic CT, MRI and clinicopathological characteristics - analysis of 36 cases and review of the literature

“…pPNET often occur in the thoracopulmonary region (Askin tumor), retroperitonium, and extremities [ 11 ]. Uncommon locations include the thyroid [ 12 ], kidney [ 13 ], breast [ 14 ], nose [ 15 ], adrenal gland [ 16 ], prostate [ 17 ], head and neck [ 18 ], abdomen, pelvic soft tissues [ 9 ], and bone [ 19 ]. Our patients had tumors in bone ( n = 19: limbs 11, vertebrae 4, craniofacial skeleton 4), soft tissue ( n = 10: pelvis 2, retroperitoneum 2, groin 1, nasopharynx 1, chest wall 2, mediastinum 2), and organs ( n = 7: parotid 1, pulmonary 4, spleen 1, liver 1).…”