Small cell neuroendocrine carcinoma of the sinonasal tract is an uncommon neoplasm with aggressive clinical behaviour. Recurrence is frequent and the prognosis is poor. However, the current treatment of these neuroendocrine neoplasms varies widely.
MEC is very rare in the pediatric age group. Treatment involves surgical removal of the tumor plus radiotherapy, according to histologic staging. MEC has a good prognosis in young patients. The survival rate does not differ in the subgroup of patients with MEC as a secondary tumor.
Early CT and MRI should be performed when facing aspecific, rhinological, or neuro-ophtalmological symptoms. Cranial neuropathies indicate a worse prognosis. Surgery, including debulking surgery, may be preferred to combined modality treatments without surgery. Its apparently favorable impact on prognosis would need to be tested in homogenous histological groups of patients, which is impossible because of the rarity of the disease. Highly conformal radiotherapy (adjuvant or definitive) should be encouraged and optimized with concurrent chemotherapy in advanced stages. Aggressive multidisciplinary management including surgery, chemotherapy, and radiotherapy should be encouraged and adapted on histology and tumor extensions. Progress is still warranted to improve outcomes.
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.
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